in Cardiovascular & Thoracic Surgeryezzeldinmostafa.com/Files/Ain Shams Lecture...

Part IV General Thoracic

Surgery (GTS)

Ain Shams Lecture Notes in

Cardiovascular & Thoracic Surgery

Ezzeldin A. Mostafa, MD, PhD, MBA Professor of Cardiovascular & Thoracic Surgery Hany H. Elsayed, MD, PhD, FRCS (CT) Associate Professor of Cardiovascular & Thoracic Surgery

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AinShamsLectureNotes

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SurgeryVolumeIV:GeneralThoracicSurgery(ThirdEdition)

EzzeldinA.Mostafa,MD,PhD,MBAProfessorofCardiovascular&ThoracicSurgeryDepartmentofCardiovascular&ThoracicSurgeryFacultyofMedicine,AinShamsUniversity,Cairo,Egypt.HanyH.Elsayed,MD,PhD,FRCS(CT)AssociateProfessorofCardiovascular&ThoracicSurgeryDepartmentofCardiovascular&ThoracicSurgeryFacultyofMedicine,AinShamsUniversity,Cairo,Egypt.

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Ain Shams Lecture Notes in Cardiovascular & Thoracic Surgery Part IV: General Thoracic Surgery

3

ToMyStudents

Youwillremember

someofwhatyouhear,

muchofwhatyouread,

moreofwhatyousee,

and

almostallofwhatyouexperienceandunderstandfully.

‘Happinesscomeswhenyoubelieveinwhatyouaredoing,knowwhatyouaredoing,

andlovewhatyouaredoing’’

EzzeldinA.Mostafa,MD,PhD,MBA

Tellme.....andIwillforgetShowme.....andImayremember

Involveme.....andIwillunderstand

Confucius,450BC

1

Symbols, Abbreviations & Acronyms This is the list of abbreviations and acronyms which occur frequently in the text. Those less commonly used are explained where they occur.

General Abbreviations & Acronyms

2

General Abbreviations & Acronyms (Cont’d)

Symbols

≈ approximately § cross-reference ° degrees ® Trade name + ve positive ± with/without < less than > more than − ve negative ≤equal or less than ≥ equal or more than DDx or DD differential diagnosis

ê decreased Dx Diagnosis Hx History é increased è leads to or results in M:F Male to Female ratio ò Female ô Male Tx Treatment α alpha β beta δ delta Δ triangle μ mean

III

Specific Abbreviations & AcronymsABG arterial blood gas ACE angiotensin-converting enzyme AChR acetyl cholinesterase receptor ADH antidiuretic hormone ADP adenosine diphosphate AF atrial fibrillation or AFib AMI acute myocardial infarction ANS autonomic nervous system AP anteroposterior APC antigen presenting cell APTT activated partial thromboplastin time ARDS acute respiratory distress syndrome ARF acute respiratory failure ARI acute renal insufficiency ATP adenosine triphosphate bid twice a day (bis in die) BiPAP biphasic positive pressure ventilation BP blood pressure BSA bovine serum albumin BUN blood urea nitrogen C/O complaint of Ca2+ calcium CAVH continuous arteriovenous hemofiltration CBC complete blood count CDC Centers for Disease Control and Prevention CHF congestive heart failure CLL chronic lymphocytic leukemia cm centimeter/s CMR cardiac magnetic resonance CMV cytomegalovirus or controlled mechanical ventilation CN cyanide CNS central nervous system CO cardiac output COPD chronic obstructive pulmonary disease CPAP continuous positive airway pressure CPB cardiopulmonary bypass CPR cardiopulmonary resuscitation CRI chronic renal insufficiency CRP C-reactive protein CSF cerebrospinal fluid CT computed tomography CTA computed tomography angiogram CVA cerebrovascular accident CVP continuous venous pressure CVVH continuous venovenous hemofiltration CXR chest X-ray DIC disseminated intravascular coagulation DLCO diffusing capacity of the lung for carbon monoxide DMSO dimethyl sulfoxide solution

DO2 oxygen delivery DSWI deep sternal wound infection DVT deep vein thrombosis E/M electron microscopy EBV Epstein–Barr virus ECF extracellular fluid ECMO extracorporeal membrane oxygenation EEG electroencephalogram EKG electrocardiogram EMG electromyogram EPO erythropoietin ERT emergency room thoracotomy ET endotracheal EtOH ethanol (alcohol) EUS endoscopic ultrasound EVLW extravascular lung water FB foreign body or feedback FBC full blood count Fe iron FEV1 forced expiratory volume in 1 second FFP fresh frozen plasma FFR fractional flow reserve FiO2 fraction of inspired oxygen FVC forced vital capacityg gram/s GFR glomerular filtration rate GI gastrointestinal HACEK Hemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella HAR hyperacute rejection HCV hepatitis C virus HIT heparin-induced thrombocytopenia HITT heparin-induced thrombocytopenia and thrombosis HLA human leukocyte antigen HR heart rate hr hour HTLV human T-lymphotrophic virus IABP intra-aortic balloon pump ICD implantable cardioverter-defibrillator or International Classification of Diseases ICU intensive care unit IM intramuscular INPV intermittent negative pressure ventilation INR international normalized ratio Int intermediate IPPV intermittent positive pressure ventilation ITA internal thoracic artery IV intravenous IVC inferior vena cava IVUS intravascular ultrasound JVP jugular venous pressure K potassium kg kilogram/sL liter/s L milliliter/sm

IV

L. left-sided LA left atrium/atrial LAO left anterior oblique LAP left atrial pressure LAP mean left atrial pressure LASER is an acronym for Light Amplification by Stimulated Emission Radiation. LFT liver function test LMS left main stem LMWH low molecular weight heparin LN lymph node LPA left pulmonary artery LSV long saphenous vein LSVC left superior vena cava LV left ventricle M/E microscopic examination MAO monoamine oxidase MAP mean arterial pressure MAPCA major aortopulmonary collateral artery MCV mean corpuscular volume MDT multidisciplinary team mg milligram/s MG myasthenia gravis MI myocardial infarction min minute/sm MMF mycophenolate mofetil mmol millimole/s MMV mandatory minute ventilation mPAP mean pulmonary artery pressure MRA magnetic resonance angiography MRI magnetic resonance imaging MRSA meticillin-resistant Staphylococcus aureus ms millisecond/s MVO2 mixed venous oxygen saturation MVV maximal voluntary ventilation N/E naked eye appearance Na sodium NBM nil by mouth NG nasogastric NIBP non-invasive blood pressure NIPPV non-invasive intermittent positive pressure ventilation NO nitric oxide nocte at night NPO withold food and drink by mouth (nil per os) NSAID non-steroidal anti-inflammatory drug NYHA New York Heart Association O/E on examination OCP oral contraceptive pill od once a day (omne in die) OR operating room or odds ratio pa per annum PA pulmonary artery or posteroanterior or physician assistant PAH pulmonary arterial hypertension PAN polyarteritis nodosa PAP pulmonary artery pressure

PAPVC partial anomalous pulmonary venous connection PAWP pulmonary artery wedge pressure PDA posterior descending artery or patent ductus arteriosus PDGF platelet-derived growth factor PEA pulseless electrical activity PEEP positive end-expiratory pressure PET positron emission tomography PFO patent foramen ovale PGE1 prostaglandin E1 PICU pediatric intensive care unit Plts platelets PNS peripheral nervous system po by mouth (per os) POBA plain old balloon angioplasty PPI proton pump inhibitor ppo predicted postoperative prn pro re nata (as required) PS pulmonary stenosis or pressure support PSV pressure support ventilation PT prothrombin time PTCA percutaneous transluminal coronary angioplasty PTE pulmonary thromboembolism PVC polyvinyl chloride PVR peripheral vascular resistance PVRI pulmonary vascular resistance index qid four times a day (quater in die) R. right-sided RA right atrium/atrial RAAS renin–angiotensin–aldosterone system RAO right anterior oblique RAP right atrial pressure RBB right bundle branch RBC red blood cell RCT randomized controlled trial RPA right pulmonary artery RSPV right superior pulmonary vein RSV respiratory syncytial virus RV right ventricle S. & S. symptoms and signs s/l sublingual SA sinoatrial SAM systolic anterior motion SaO2 oxygen saturation of arterial blood SBT spontaneous breathing trials SC subcutaneous SCBU special care baby unit SIMV synchronized intermittent mandatory ventilation SIRS systemic inflammatory response syndrome SLE systemic lupus erythematosus SNP sodium nitroprusside

V

SPECT single positron emission computed tomography SR survival rate SSEP somatosensory evoked potential SVC superior vena cava SvO2 mixed venous oxygen saturation SVR systemic vascular resistance SWI superficial wound infection TAPVC total anomalous pulmonary venous connection TEE transesophageal echocardiography TEG thrombelastography TIA transient ischemic attack tid three times a day (ter in die) TIVA total intravenous anesthesia TLC total lung capacity TOF tetralogy of Fallot or tet TOS thoracic outlet syndrome TRAM transverse rectus abdominis myocutaneous TTE transthoracic echocardiography

U unit/s U/S ultrasound U&E urea and electrolytes URTI upper respiratory tract infection VATS video-assisted thoracic surgery VAVD vacuum-assisted venous drainage VC vital capacity VF ventricular fibrillation vit vitamin VQ ventilation-perfusion VSD ventricular septal defect VT ventricular tachycardia, VTach WCC white cell count WL window level WW window width XM cross match yr(s) year (s)

1 Cardiothoracic Trauma

2 The Chest Wall

3 The Diaphragm

4 The Pleurae

5 The Tracheobronchial Tree

6 The Lungs

7 The Esophagus

8 The Mediastinum

ezzeldinmostafa.com

General Thoracic Surgery Dr. Ezzeldin Mostafa [email protected] www.ezzeldinmostafa.com

Spring 2016 1. Cardiothoracic Trauma

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1 Cardio-Thoracic Trauma

Table of Contents

1 Cardio-Thoracic Trauma ......................... 2 Blunt Thoracic Trauma .......................................... 5

Management (= The 5 General Principles of Management of Injured Person) ......................... 5 Initial Examination & Evaluation (emergency room care). ......................................................... 5 How To Read Plain Chest X-Ray ....................... 5 Chest Wall Injuries ............................................. 6 Pulmonary Injuries .............................................. 6 Tracheal/Bronchial Injuries ................................. 7 Cardiac/Great Vessel Injuries ............................. 7 Diaphragm Rupture ............................................ 7

Penetrating Thoracic Trauma ................................ 8 General ............................................................... 8 Primary Survey ................................................... 8 Secondary Survey .............................................. 8 Ancillary tests ..................................................... 9 Diagnosis ............................................................ 9

Treatment and Resuscitation ........................... 10 Blast Injuries ........................................................ 12

Complications of thoracic trauma ..................... 13 Chest Wall Injuries ............................................... 15 Thoracic Outlet Compression Syndrome ............. 15

Recurrent Thoracic Outlet Syndrome ............... 18 Traumatic Intrathoracic Collections ..................... 18 Pneumothorax ..................................................... 19 Pulmonary Injuries ............................................... 20 Traumatic Diaphragmatic Hernation (CDH) or Perforation ........................................................... 20 Tracheobronchial Injuries .................................... 21 Esophageal Injuries ............................................. 22

Esophagus - Caustic Burns .............................. 23 Traumatic Injuries of the Mediastinum ................. 23

Mediastinal Emphysema .................................. 23 Traumatic Asphyxia .......................................... 24

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Chapter Objective At the end of this chapter the resident understands types of cardio-thoracic traumas, including those leading to different pathologies, and uses operative and non-operative therapy

Learner Objectives: Upon completion of this chapter the resident: 1. Understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for blunt, penetrating and blast injuries; the dozen (12) lethal traumas, the 5-stage management of thoracic trauma

2. Knows the pathogenesis and management of blunt thoracic trauma trauma.

3. Knows the pathogenesis and management of blunt thoracic trauma trauma;

4. Knows the pathogenesis and management of blast injuries; 5. Understands the pathogenesis and management of traumatic

chest wall injuries in details; 6. Understands the pathogenesis and management of traumatic

intrathoracic collections (air, blood, chyle and others) in details; 7. Understands the pathogenesis and management of the 10

traumatic pulmonary injuries in details; 8. Understands the pathogenesis and management of R. & L.

traumatic diaphragmatic injuries in details; 9. Understands the pathogenesis and management of traumatic

tracheobronchial injuries in details; 10. Understands the pathogenesis and management of traumatic

esophageal injuries in details; 11. Understands the pathogenesis and management of traumatic

mediastinal injuries in details;

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Definition Trauma to the chest is usually divided into blunt and penetrating injury. Proper emergency care and resuscitation are integral parts of the management of these patients, who may have airway obstruction, life-threatening hemorrhage, and severe associated injuries. Classification of cardiothoracic trauma according to the mechanism of injury.

Types 1) Blunt (closed) 2) Open (penetrating, perforating) 3) Blast injuries

Delayed & missed diagnoses in chest trauma Delayed Diagnosis Missed Diagnosis

Types of Cardiothoracic Trauma

Classification of cardiothoracic trauma according to the mechanism of injury.

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Classification of cardiothoracic trauma according to life threatening The deadly dozen (12) chest injuries

The 5“Killers” in Thoracic Trauma 5 Immediate Life Threats Mechanism of Early Death after Thoracic Injury

5 Potential Life Threats

5 Other Frequent Injuries

1. Airway obstruction 2. Tension pneumothorax 3. Open pneumothorax • “sucking chest wound

4. Massive hemothorax 5. Cardiac tamponade •

1. Lung contusion 2. Heart contusion 3. Aorta rupture 4. Tracheobronchial tree injury

- larynx, trachea, bronchus 5. Esophagus trauma

1. Subcutaneous emphysema 2. Traumatic asphyxia 3. Simple pneumothorax 4. Hemothorax 5. Rib fractures •

6. Flail chest is # 6 6. Diaphragm rupture is # 6

6. Scapula fracture is # 6 Blunt Thoracic Trauma

Management (= The 5 General Principles of Management of Injured Person)

I. First-aid Measures (immediate measures at the scene of an accident).

II. Initial Examination & Evaluation (emergency room care). III. Resuscitation & Initial Therapy. IV. Definitive Treatment (& definitive diagnosis) (Principles

of management of specific types of injuries). V. Postinjury Care (& Complications).

Initial Examination & Evaluation (emergency room care). A. Clinical Evaluation B. Radiographic evaluation How To Read Plain Chest X-Ray ABC”s approach

How To Read Plain CXR “Some Bastard Pinched My Toy Dinosaur” “Some Body Pinched My Toy Dinosaur” S: Soft tissue B: Bones- clavicles, ribs P: Pleura, Pulmonary Parenchyma M: Mediastinum T: Thorax, Tubes, Lines D: Diaphragm Follow The Same Steps Of How To Read: Soft tissues Bone Pleura &

Parenchyma Mediastinum Tubes Diaphragm

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Chest Wall Injuries • Rib fracture is the most common thoracic injury • Significant intrathoracic injury may be present without rib fracture

in children due to rib cage elasticity • Narcotics and intercostal nerve blocks are sufficient for simple rib

fractures • Patients with flail chest should be supported with mechanical

ventilation for several days to regain chest wall stability • Consider tracheostomy for prolonged intubation to minimize

laryngeal injury and facilitate pulmonary care • First rib fracture indicates significant force, and aortography is

indicated if the patient also has brachial plexus deficit, absent radial pulse, pulsating supraclavicular mass, or widened mediastinum

Traumatic Intrathorathic collection

1. Gas collections Emphysema

Subcutaneous emphysema (15%) Mediastinal emphysema (3.5%) Retroperitoneal emphysema.

Paramediastinal peumatocele. Traumatic pneumothorax

Uncomplicated closed (30%) Open (small wound, large wound) Tension compressive, (25%).

2. Blood collections Hemothorax

Mild Moderate (25%) Severe (Massive).

Hemopneumothorax. Extrapleural hematoma. 3. Post-traumatic pachypleuritis (organised

hemothorax) organised hemothorax 4. Other traumatic effusions

Hydrothorax. Pyothorax.

- Chylotnorax. - Bilithorax.

5. Pericardia! effusions (see later)

10 pulmonary injuries With BAD Prognosis (3)

With GOOD Prognosis (7)

1. ARDS 2. Lung Contusion 3. Pulmonary blast

injuries

1. Uncomplicated lung contusion (lung concussion)

2. Fat embolism. 3. Intrapulmonary hemotoma 4. Pneumatocele. 5. Lung Laceration. 6. Lung Wound. 7. Lung barotrauma.

Pulmonary Injuries • Pulmonary contusion probably occurs to a varying degree in all

thoracic injuries and is a major component of flail chest • Significant hypoventilation and shunting from contusion requires

judicious fluid management and ventilatory support, if indicated • Partial, complete, and tension pneumothorax should all be managed

promptly with chest tube insertion • Subcutaneous emphysema should prompt investigation for

pneumothorax but is not in itself an indication for chest tube placement

• Hemothorax should be managed with early chest tube drainage to

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prevent clot formation and incomplete evacuation • Surgical exploration is recommended if initial output is more than

1000 ml or chest tube drainage is more than 100 ml/hr for 4 hours • A clotted hemothorax should be evacuated early by thoracotomy to

improve pulmonary function and prevent late fibrothorax

Tracheal/Bronchial Injuries • Most tracheal injuries are cervical and range from crush injuries to

compete tracheal separation • If endotracheal intubation is not possible, a surgical airway should

be obtained • Primary repair of tracheal lacerations or separation should be

performed, if possible • Blunt trauma typically causes a circumferential laceration of either

main bronchus with complete separation • Only 50% of patients will have a pneumothorax with this injury,

and hemothorax is uncommon • Only 1/3 of patients are diagnosed in the first 24 hours, and only

1/2 within the first month • Early repair is the preferred treatment if the diagnosis is made, and

requires thoracotomy with intubation of the uninjured bronchus • Late strictures from incomplete tears or parenchymal isolation from

complete tears can be repaired with bronchoplastic procedures, but may require pulmonary resection

Cardiac/Great Vessel Injuries • Myocardial contusion is the most common injury and is suspected

with EKG changes and serial enzyme elevations • Coronary artery injury can result in thrombosis and myocardial

infarction • Atrial or ventricular rupture is usually fatal, although the

pericardium may restrict bleeding enough to allow survival to the ER

• The patient should be monitored in the ICU and may require heparinization for coronary thrombosis and anti-arrhythmic therapy

• Echocardiography and angiography are indicated for tamponade and post-injury murmurs, which suggest valvular insufficiency or septal defect

• Aortic rupture is also usually fatal, but can result in formation of a false aneurysm, typically at the aortic isthmus

• Patients with a widened mediastinum on CXR should have prompt aortography, which will demonstrate an intimal tear

• Surgical repair should be done promptly, as fatal hemorrhage can occur at any time

• Techniques include LA-FA bypass, proximal aorta-distal aorta shunting, and cross-clamping without cardiopulmonary bypass

Diaphragm Rupture • Most lacerations occur on the L. hemidiaphragm and result from

automobile accidents • Usually, the stomach herniates and undergoes volvulus, massively

dilates, and causes L. lung collapse and mediastinal shift to the R. • Gastric distension can also result in perforation and should be

prevented by NG tube placement

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• Splenic and liver injury is also common in this setting • The diaphragm can be repaired either through the chest or

abdomen, and all tears should be closed in double-layer fashion Penetrating Thoracic Trauma

General In civilian practice, 85 % can be managed with simple procedures and

only 15 % will require definitive surgical intervention

• Chest trauma causes 25 % of trauma deaths in the U.S.

• ≈15 % of combat injuries involve the thorax.

• Penetrating injury accounts for 95 % of these casualties.

• About 10 % involve only the chest wall, 67 % involve the heart, great vessels, or pulmonary hilum, and ≈ 15-20 % involve the pulmonary parenchyma.

Primary Survey

Evaluate the patient for the presence of a patent airway, adequate breathing, and intact circulation (ABCs) per ATLS guidelines.

• Assess airway competency for injuries of the base of the neck and the thoracic outlet.

• Visually inspect and palpate the chest. Observe for enlarging hematomas, creptitis, hemoptysis, and dyspnea.

• Observe for cyanosis, respiratory movement/quality, intercostal or suprclavicular retractions, tachypnea, deviated trachea, and a change in respiratory pattern. Auscultate to ensure airway exchange in the lungs.

• Immediate life threatening injuries for thoracic trauma include airway obstruction, cardiac tamponade, flail chest, tension pneumothorax, open pneumothorax, and massive hemothorax.

Immobilize the head and neck, and maintain the cervical spine in a neutral position. Assume a cervical spine injury is present until proven otherwise by palpation and radiological examination. Disability: abbreviated neuro exam to include pupils and level of consciousness. Exposure: fully undress the patient Determine pre-hospital care already delivered Mechanism of injury. Pain - presence, absence, location. Examine the remainder of the patient for any other life threatening injuries.

Primary Survey: 3E E−Evaluate Immobilize Disability: E−Exposure Determine Mechanism Pain E−Examine

Secondary Survey • An "AMPLE" history per ATLS.

An "AMPLE" history per ATLS. A−Allergies M−Medications taken by the

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patient P−Past medical history L−Last meal E−Environment and events related to patients injury(s)

Ancillary tests • CXR • Hgb/Hct followed sequentially may point to occult bleeding. • Pulse oximetry, Cardiac monitor, and an ECG. • ABG's (if available) confirm adequacy of oxygenation, ventilation,

and shock resuscitation

CXR • Manditory after 1° survey • Mark entry and exit wounds

with a radiopaque marker • May be useful to exclude

injury to the thoracic viscera if wounds appear superficial.

• Essential after an intervention (i.e. chest tube) to document amelioration of injury.

• Serial CXRs at least every 6 hours with penetrating trauma until stable after 24 hours.

Diagnosis

Injuries due to penetrating trauma.

• Airway obstruction. • Tracheobronchial disruption - noisy labored breathing, hemoptysis,

subcutaneous emphysema, massive air leak out chest tube. • Esophageal rupture - particulate matter in chest tube, mediastinal

air, intermittent air leak. • Cardiac tamponade - narrowed pulse pressure, distended neck

veins, muffled heart sounds, pulsus paradoxus, but no tracheal deviation.

• Tension pneumothorax - respiratory distress, ipsilateral absence of breath sounds, ipsalateral hyperresonance with percussion, tracheal deviation to the opposite side (late finding), +/- neck vein distension (often absent), and cyanosis (late).

• Open pneuomothorax (sucking chest wound) – hypoxia results due to chest wall defect

• Massive hemothorax - shock, absent breath sounds with dullness to percussion, +/- neck vein distension (depending on intravascular volume status).

• Pulmonary contusion - seen on chest x-ray and may manifest by progressive hypoxia.

• Traumatic diaphragmatic laceration - suspect on chest x-ray if diaphragm not clear, NG tube curls up into chest, loculated subpulmonic pneumothorax.

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Indications of surgery in chest trauma. Potentially acutely lethal injuries of the chest and their management Injury Management Tension pneumothorax Tube thoracostomy Massive intrathoracic hemorrhage Tube thoracostomy, operative repair Cardiac tamponade Pericardiocentesis, operative repair Deceleration aortic injury Operative repair Massive flail chest with pulmonary contusion Intubation, pain control, fluid restriction Upper and lower airway obstruction Intubation, airway, bronchoscopy Tracheobronchial rupture Bronchoscopy, operative repair Diaphragmatic rupture with visceral herniation Operative repair Esophageal perforation Operative repair

Treatment and Resuscitation ABCs as per ATLS. Serious penetrating chest injuries • Ensure an airway, intubate if necessary. • Cover chest wall holes on 3 sides. Do not totally occlude seal

because this can lead to a tension pneumothorax. Don't remove impaled, embedded objects.

• Place a chest tube to remove air and/or blood from the pleural space. Don't insert through an injury site.

Comment: Knowledge of the type of weapon in gunshot wounds is useful, as unbalanced or hollow-point ammunition can cause extensive internal destruction despite small entrance wounds. In addition, such missiles can fragment and embolize. It is important to remember that any penetrating

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• Start two large bore IV's and administer Ringer's lactate solution and blood as necessary to maintain adequate blood pressure.

• ABG's if available should be assessed to ensure adequate oxygenation, ventilation, and resuscitation from shock.

• If cardiac tamponade suspected, perform pericardiocentesis. If tamponade found, leave a plastic catheter in place attached to a stopcock, so reaspiration may be performed prn.

• Evacuate as a litter patient for surgical evaluation. • Place a one-way (Heimlich) valve in line of every chest tube unless

a pleuovac or equivalent water seal system can be used. • Every such patient requires supplemental oxygen, NG or OG

intubation, and a bladder catheter. • Minor injuries. Superficial chest wall injuries may be debrided and

closed. If no evidence of hemo- or pneumothorax is seen on CXR in 6 hours, and the patient is otherwise asymptomatic, return to duty is indicated.

• After initial evaluation, emergency management, resuscitation, and stabilization of the casualty with a penetrating thoracic cavity injury, all such injured should be emergently evacuated to a facility with surgical capability.

injury to the fourth interspace or below may well have passed through the diaphragm, and attention given to possible intraabdominal injury.

1. Chest Wall Injuries • Laceration of intercostal or internal mammary arteries can be life-

threatening and operative intervention based on chest tube output • The pulmonary vessels are rarely the source of major bleeding

unless a hilar vessel is injured • High-velocity missiles and shotgun wounds can produce extensive

open wounds requiring immediate occlusion and intubation, followed by operative repair

2. Pulmonary Injuries • Most penetrating wounds only require chest tube insertion and

lung expansion • Parenchymal injuries requiring operation can usually be oversewn

without difficulty • Bronchial or pulmonary artery injury can require resection • A large vascular clamp placed across the lung hilum facilitates

exploration and vessel repair

3. Base of Neck Injuries • The close proximity of major structures make injury highly

probable • This can be assessed by angiography, contrast swallow,

endoscopy, or surgical exploration • The surgical approach will vary, but median sternotomy with

lateral or superior extension provides the widest exposure • Avoid prosthetic grafts for vascular repair if the trachea or

esophagus are also injured • Cardiopulmonary bypass may be required if the aorta must be

cross-clamped

4. Cardiac/Great Vessel Injuries • The R. ventricle is most commonly injured, followed by the L.

ventricle • Ventricular septal defect is the most commonly intracardiac injury • Most patients do not reach the hospital, as the injury to the

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pericardium leads to exsanguination instead of tamponade • Hypotension that does not respond to rapid volume replacement

suggests significant injury • CXR, EKG, and echocardiography have little diagnostic value in

these patients • Subxiphoid pericardiocentesis is useful for diagnosis; negative

deflection of the QRS complex indicates contact with the epicardium and a drain should be L. in place

• Subxiphoid pericardial window is preferred for tamponade, however, and should be performed in the operating room, as the patient may rapidly exsanguinate

• Emergency room thoracotomy is seldom indicated, being reserved for moribund patients or rapid deterioration without time to transfer to the OR

• Median sternotomy is the preferred approach • Repair ventricular lacerations with pledgetted nonabsorbable

horizontal mattress sutures • Oversew atrial or aortic injuries • Coronary artery division should be managed by ligation and

bypass grafting on cardiopulmonary bypass • Obvious septal defects or gross valvular insufficiency should be

repaired; otherwise, the injury should be more adequately studied with postoperative catheterization 5. Tracheal/Bronchial Injuries

• Tracheal injury is suggested by pneumothorax, pneumomediastinum, subcutaneous emphysema, hemoptysis, and airway obstruction

• Following intubation or a surgical airway, an anterior collar incision is the best approach

• Median sternotomy may be required for associated vascular injury or intrathoracic tracheal laceration

• Avoid tracheostomy if possible when a vascular repair is in proximity

6. Esophageal injuries • Blunt injury is rare; the most common cause is endoscopic

perforation, followed by penetrating injuries • Mediastinitis is a lethal complication and early surgical

intervention is recommended • Cervical esophageal injury should be approached through a lateral

neck incision and thoracic injuries via thoracotomy • If the tissue is not extensively damaged, primary repair with

drainage is appropriate; otherwise, the wound is L. open • Postemetic rupture (Boerhaave's syndrome) presents with pain,

fever, and shock; death can occur within 24-48 hours • The diagnosis is suggested by cervical and mediastinal air,

widened mediastinum, and pleural effusion • The esophagus should be closed in two layers, the mediastinum

widely opened, and the area drained into the pleural space via thoracotomy

Blast Injuries

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FIGURE 1-7. Algorithm for the management of blast injuris.

Complications of thoracic trauma 1. ARDS follows many types of injuries, but is particularly common in

thoracic trauma 2. It typically begins a few hours after injury and progresses rapidly 3. Ventilatory support with PEEP and high FIO2 is the standard of

care

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4. Failure of ARDS to improve after 4-6 days is associated with a high incidence of death

5. Arrhythmias are common in this patient population, particularly atrial fibrillation, which can be treated with standard measures

6. Ventricular arrhythmias suggest myocardial injury or infarction and should be investigated

7. Many patients require tracheostomy and attention should be given to proper care

8. Other complications include atelectasis, thromboembolism, infection, and air embolism

Complications Preventing Measures Treatment

• Shock ARF (ARDS) • Aspiration • Retained secretions • Fat embolism • Undiagnosed hemo-or pneumo-thorax • Fluid administration • Abdominal distension. • Gross obesity • Chest wall instability

Restoration of blood volume In hypovolemic shock Blood Filters in blood transfusion Fixation of fractures Respiratory physiotherapy Prevention of pulmonary infection

Monitoring. Ventilation. Medication. Anticoagulant. Steroids Antibiotics Correction of predisposing cause.

Atelectasis

Assisted coughing Intratracheal suction (2 tech) Haight's technique Transcutanoeus Installation of mucolytic agents Aspiration bronchoscopy

Aspiration Bronchoscopy

Infection Infectious pneumonitis Empyema

Prophylactic antibiotic therapy Cephalosporin Aminoglycoside (short-term ONLY 24-48 hrs).

Antibiotics Culture Ventilatory support

PTE

Ambulation, exercises Low-dose heparin Oral anticoagulants Perfusion lung scan and/or PA angio.

Heparin Vena cava filter Supportive Rx

Fat embolism

Examine urine for fat Prompt fixation of fracture

Stabilize fractures Supportive Rx Ventilate.

Tracheal njuries related to intubation

Terminating IPPV & PEEP as soon as possible Low-pressure cuffed tubes Avoid over-inflation Changing the length of the tube Maximum sterility No flap tracheostomy (simple incision Only)

Early diagnosis ê Surgical treatment

Arterial injury (TIAF)

Insertion of a longer tube Interposition of a flap of pectoral muscle & skin. If sudden massive Hge. Immediate over-inflation of the cuff Digital compression while the tube is changed By a long ET

Repair simple bypass

CHF

Avoid over-infusion Swan-Ganz catheter Rx pre-existing cardie disease

Cardiac-support Duiresis digitalis venisection

Dysrrhythmias

AFib, PVCs Rx pre-existing cardiac disease

Propranolol Cardioversion Lignocaine

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Chest Wall Injuries First Ribs (Protected Region) Middle Ribs (Exposed Region) Lower Ribs (Supple Region)

Rupture of the aorta Rupture of the vessels at the thoracic outlet. Rupture of the trachea. Rupture of the bronchi. Tear of the brachial plexus. Phrenic nerve injury. Fracture of the clavicle.

Flail chest with paradoxical motion*. Lung contusion. Peripheral avulsion of the diaphragm.

Rupture of the spleen. Rupture of the liver. Rupture of the kidney. Rupture of th diaphragm.

Thoracic Outlet Compression Syndrome Definition Compression of the subclavian vessels and brachial plexus at the superior aperture of the chest, most commonly against the first rib. Other terms for this syndrome include scalenus anticus syndrome, costoclavicular syndrome, hyperabduction syndrome, cervical rib syndrome, and first thoracic rib syndrome.

Anatomy A. Surgical Anatomy · The first rib divides the cervicoaxillary canal into a proximal space and a distal space (the axilla) · Most neurovascular compression occurs in the proximal section, which consists of the costoclavicular space and the scale triangle · Costoclavicular space boundaries: clavicle (superior), first rib (inferior), costoclavicular ligament (anteromedial), and scalenus medius/long thoracic nerve (posterolateral) · Scalene triangle boundaries: scalenus anticus (anterior), scalenus medius (posterior), and first rib (inferior) · The subclavian vein lies anteromedial to the scalenus anticus; the subclavian artery and brachial plexus run posterolateral to this muscle

B. Functional Anatomy · Certain movements and position of the arm and shoulder girdle, as well as anatomic variations, can narrow the costoclavicular space or scalene triangle · Arm abduction rotates the clavicle toward the first rib · Arm hyperabduction pulls the neurovascular bundle around the coracoid process and head of the humerus · Poor shoulder posture lessens the angle of the sternoclavicular joint as the distal end of the clavicle "droops" · Severe emphysema or excessive muscular development causes abnormal lifting of the first rib · Anatomic variations narrow either the superior angle or the base of the scalene triangle, producing upper and lower types of compression syndromes, respectively

Etiology There are many factors which can cause neurovascular compression

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at the thoracic outlet. Bony abnormalities are present in about 30% of patients, and some of these may be visualized on plain chest x-ray.

Etiology of The TOS I. Anatomic Factors

· Interscalene compression · Costoclavicular compression · Subcoracoid compression

II. Congenital Factors ·

. Cervical rib · Rudimentary first rib · Scalene muscle abnormalities · Fibrous bands · Bifid clavicle · First rib exostosis · Enlarged C7 transverse process · Omohyoid muscle abnormalities · Anomalous transverse cervical artery · Postfixed brachial plexus · Flat clavice

III. Traumatic Factors

· Fractured clavice · Humeral head dislocation · Upper thorax crush injury · Sudden effort of shoulder girdle muscles · C-spine injuries/cervical spondylosis

IV. Atherosclerosis

*adapted from Kirklin and Barratt-Boyes Clinical Presentation The character and pattern of symptoms will vary depending on the degree to which nerves, blood vessels, or both are compressed A. Neurogenic · More frequent than vascular compression · Pain and paresthesias present in 95% of patients · True motor weakness with atrophy of hypothenar/interosseus muscles found in 10% · Sensory fibers lie on the outside of the nerve bundles and are the first to be affected by compression · Symptoms usually have ulnar nerve distribution (medial arm and hand, 4th and 5th fingers) · Pain is insidious and involves neck, shoulder, arm and hand · Strenuous physical exercise preciptates the symptoms, with arm in abduction and neck hyperextended B. Vascular · Pain is usually diffuse and associated with coldness, weakness, and easy fatiguability of the hand and arm · Unilateral Raynaud's phenomonen in about 7.5% of patients, which can be precipitated by hyperabduction or carrying heavy objects · There may be signs of distal embolization, poststenotic dilation or aneurysm of the subclavian artery, or true arterial occlusion · Venous obstruction is much less common and is known as "effort thrombosis" or "Paget-Schroetter syndrome" · The affected arm is edematous, discolored, and aches

Urschel HC Jr and Razzuk MA. Upper plexus thoracic outlet syndrome: optimal therapy. . Annals of Thoracic Surgery 1997 63(4):935-9. MacKinnon SE, Patterson GA, Novak CB. Thoracic outlet syndrome: A current overview. Seminars in Thoracic and Cardiovascular Surgery 1996 8(2):176-82. Urschel HC Jr. Dorsal sympathectomy and management of thoracic outlet syndrome with VATS. Annals of Thoracic Surgery 1993 56(3):717-20. Urschel HC Jr and Razzuk MA. The failed operation for thoracic outlet syndrome: The difficulty of diagnosis and management. Annals of Thoracic Surgery 1986 42(5):523-8.

Diagnosis A. Clinical maneuvers · Positive findings for all tests include a decrease or loss of the radial

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pulse, or reproduction of symptoms · Adson/scalene test: patient holds a deep inspiration, fully extends neck, and turns head to the side · Costoclavicular test: shoulders drawn inferiorly and posteriorly · Hyperabduction test: arm is hyperabducted to 180 degrees B. Radiologic tests · CXR and C-spine films can detect cervical ribs and degenerative changes · Cervical CT should be performed if osteophytic changes and intervertebral space narrowing are present · Angiography is indicated for a pulsating paraclavicular mass, absent radial pulse, or paraclavicular bruit C. Ulnar nerve conduction velocity · Points of stimulation include the supraclavicular fossa, middle upper arm, below elbow, and wrist · Normal value across the thoracic outlet is 72 m/sec; any value less than 70 m/sec indicates compression

Grading of Compression Grading of Compression

Velocity Grade 66-69 m/sec Slight 60-65 m/sec Mild 55-59 m/sec Moderate less than 54 m/sec Severe

Differential Diagnosis · The differential diagnosis for thoracic outlet syndrome is quite broad and includes neurologic, vascular, pulmonary, cardiac, and esophageal disorders. · Some of the more common conditions include herniated cervical disk, cervical spondylosis, and peripheral neuropathies Treatment · Physical therapy should be initiated in all patients · Most patients with an UNCV above 60 m/sec will improve with conservative therapy · Surgical intervention should be considered if symptoms persist after physical therapy and the UNCV shows minimal or no improvement Operative Technique · Always document preoperative neurologic findings · Transaxillary first rib resection avoids division of major muscle groups, ensures complete removal of the first rib, and has the best cosmetic result · Position the patient in the lateral position with the affected arm abducted 90 degrees and loosely suspended (straight up to the ceiling) · Transverse incision in the axilla between pectoralis major and latissimus dorsi · Dissect along the external thoracic fascia to the first rib · Divide the scalenus anticus at its insertion on the rib · Remove middle and anterior portion of first rib after periosteal elevation · Divide costoclavicular ligament and remove posterior portion of first rib · Always protect the brachial plexus and vessels · Remove the entire first rib, as any residual portion may cause recurrence

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Results · Almost all patients will have relief with conservative therapy, with about 5% requiring surgery · Symptoms recur in about 10% of patients · Less than 2% will require reoperation · A recent study from the Annals of Thoracic Surgery of over 2200 patients showed excellent or good results after operation in over 90% of cases

Recurrent Thoracic Outlet Syndrome • About 1-2% of patients will have persistent or progressively more

severe symptoms after their operatio • Most have recurrence within 3 months of operation • Symptoms, physical examination, and UNCV findings should be

diagnostic before reoperation • Pseudorecurrence occurs in patients in whom a cervical rib or the

second rib was resected instead of the first rib, or the first rib was resected instead of the causative cervical rib

• True recurrence occurs in patients in whom the first rib was incompletely resected or there was excessive scar development around the brachial plexus

• The posterior thoracoplasty approach provides the best exposure • Persistent or recurrent bony remnants should be excised • Careful neurolysis of the nerve root and brachial plexus is

performed along with dorsal sympathectomy • One series of over 400 patients had improvement in symptoms in

about 80% of patients; 7% required a second reoperation

Sources for further reading Textbook Chapters Chapter 18: Thoracic Outlet Syndrome. Surgery of the Chest (Sabiston and Spencer), 5th ed., 536-53. Chapters 32: Thoracic Outlet Syndromes. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 567-80

Traumatic Intrathoracic Collections

Traumatic Intrathorathic collection 6. Gas collections Emphysema

Subcutaneous emphysema (15%) Mediastinal emphysema (3.5%) Retroperitoneal emphysema.

Paramediastinal peumatocele. Traumatic pneumothorax

Uncomplicated closed (30%) Open (small wound, large wound) Tension compressive, (25%).

7. Blood collections Hemothorax

Mild Moderate (25%) Severe (Massive).

Hemopneumothorax. Extrapleural hematoma. 8. Post-traumatic pachypleuritis (organised hemothorax) organised hemothorax 9. Other traumatic effusions

Hydrothorax. Pyothorax.

- Chylotnorax. - Bilithorax.

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10. Pericardia! effusions (see later)

Pneumothorax Classification of Pneumothorax Spontaneous

Primary No underlying pathology

Secondary Underlying pulmonary disorders

Catamenial Neonatal Traumatic Iatrogenic

Thoracentesis, mechanical ventilation, central vein cath., postoperative

Blunt, penetrating Diagnostic

Air-contrast study of pleuropulmonary pathology

Treatment options in pneumothorax • Observation • Needle aspiration • Percutaneous catheter to drainage • Water-seal or Pleur-evac type • Heimlich valve • Tube thoracostomy • Water-seal or Pleur-evac • Heimlich valve • Tube thoracostomy with instillation of pleural irritant • Video-assisted thoracic surgery (VATS) • Thoracotomy

Indications for Thoracotomy • Massive air leak that prevents re-expansion of the lung • Persistent air leak > 7-10 days • N.B.: 7 - 10 days (traditional) • 2 - 4 days (recent) • Second episode of spontaneous PTX • Complications of PTX • Hemothorax • Empyema • Chronic PTX

Specific surgical indications for conditions that cause secondary spontaneous PTX • Occupational indications after first episode • Airline pilots • Scuba divers • Individuals in remote areas • Previous contralateral PTX • Bilateral simultaneous PTX • Presence of large cysts visible on CXR

Caveats One can never be faulted for placing a chest tube (unless the chest tube was placed in the abdomen). The first thing into the chest is your finger.

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Usually, do not place a chest tube below the nipple line. Pulmonary Injuries

10 pulmonary injuries With Bad Prognosis (3) With Good Prognosis (7)

1. ARDS 2. Lung Contusion 3. Pulmonary blast injuries

1. Uncomplicated lung contusion (lung concussion)

2. Fat embolism. 3. Intrapulmonary hemotoma 4. Pneumatocele. 5. Lung Laceration. 6. Lung Wound. 7. Lung barotrauma.

Traumatic Diaphragmatic Hernation (CDH) or Perforation Acute Rupture Delayed Rupture The five criteria of Bowditch in TDH 1. Prominence and immobility of the L. thorax. 2. Displacement to R. of cardiac dullness. 3. Absent breath sounds. 4. Presence of bowel sounds in the chest. 5. Tympany to percussion in the L. chest.

Pericardial chest pain or epigastric discomfort, esp. when laying down after meals in cases of previous penetrating or blunt thoracic or ab-dominal trauma should be suggestive of TDH and had to further examination with barium meal or enema.

Penetrating perforation should be suspected with any thoracic injury below the level of the nipples (5th ICS) · Most blunt hernias are caused by automobile accidents, and about 90% occur in the L. hemidiaphragm · Blunt trauma defects are large, usually about 10-15 cm, and typically located in the posterior L. hemidiaphragm · Stomach is the most commonly herniated organ, followed by spleen, colon, small bowel, and liver · Respiratory insufficiency is common early, while intestinal obstruction predominates later · CXR and CT scan will diagnose most; barium contrast is contraindicated, as it can produce a total obstruction in this setting · Missed injury and delayed diagnosis commonly leads to bowel incarceration and obstruction

Symptoms Signs

R-ray

Abdominal pain

Abdominal tenderness

Bowel above hemidiaphragm

Nausea and vomiting

êbreath sounds

Density lung base

Dyspnea

êbowel sounds

Pleural effusion Shoulder pain

Fever

High diaphragm. Chest pain

Peritoneal signs

Mortality is relatively high (15-40%) due to high incidence of associated injuries

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· Repair should be undertaken promptly with full exploration for other injuries · L.-sided perforation should be repaired through the abdomen to allow correction of associated injuries · R.-sided perforations may require thoracotomy Tracheobronchial Injuries A. Penetrating injuries · Usually involve the cervical trachea · Fresh injuries can be closed primarily B. Blunt injuries · Closed trauma may lacerate or sever the trachea · A severed trachea should be re-approximated if possible · The distal trachea should be intubated for security · A tracheostomy tube should be inserted in the distal severed end for complex injuries and repair undertaken later · A vertical split at carina is usually diagnosed by a pneumothorax that fails to resolve with closed suction · Bronchoscopy, thoracotomy, and occasionally cardiopulmonary bypass are required to repair this lesion · Traumatic TEF should be promptly repaired, as inflammation makes repair difficult when the diagnosis is delayed · Always establish the function of the recurrent laryngeal nerves and larynx prior to surgery, if possible

Postintubation Injuries

These are currently the most frequent cause of tracheal stenosis A. Laryngeal level · Sealing cuffs may irritate vocal cords, causing granulomas and fusion of the posterior commissure · Mucosal erosion at the cricoid can cause subglottic stenosis · Most lesions are reversible with time

B. Stomal level · Principle factors include a stoma that is too large, loss of tissue from infection (rare), and pressure erosion from rigid connecting systems (most common) · Avoid injuring the first cartilaginous ring during original tracheostomy to prevent subglottic stenosis · Avoid low placement of the tracheostomy to prevent innominate artery erosion and supracarinal stenosis

C. Sealing cuff level · Most common cause is direct pressure necrosis by high-pressure cuff · This has essentially disappeared with use of low-pressure, high-volume cuffs · Children may develop granuloma on anterior tracheal wall when ventilated without a cuff

D. Tracheomalacia · Usually occurs in segment of trachea between stoma and the cuff · Secretions pool here and inflammation leads to cartilage thinning

E. Tracheoesophageal fistula · Manifested by sudden appearance of secretions in tracheobronchial

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tree · Pneumonitis, pneumonia, abscess, and gastric dilitation may develop · Methylene blue in tube feedings will appear promptly in the trachea F. Trachoinnominate fistula · Manifested by sudden exsanguinating hemorrhage into the tracheobronchial tree · Emergent tamponade with high-pressure cuff or digital pressure is necessary

Diagnosis · CXR · Fluoroscopy determines laryngeal function and presence of tracheomalacia · CT to evaluate extramural extension of tumors · Bronchoscopy and biopsy at the time of operation

Esophageal Injuries

Etiology

Instrumental 1. Endoscopy: rigid, flexible. 2. Dilatation: Bougies (rigid, flexible) 3. ET tubes. 4. Esophageal intubation (prosthesis). 5. EOA (Esophageal obsturator airway). 6. Segstaken-Blackmore tube. 7. Nd-YAG laser photocoagulation 8. Sclerotherapy of esophageal varicose 9. Intracavitary irradiation for esophageal carcinoma

Non-instromental A. Postemetic, with or without pre-existing esophageal disease. B. Other acute increases in intraesophageal pressure, as with compressed

air, Valsava maneuver, defection, labor, convulsions, blunt trauma to abdomen.

C. Ingestion of sharp F.B. or corrosive sub. D. External trauma to esophagus.

1. Accidental blunt or penetrating injury (chest, neck, abdomen) Postsurgical.(operation on esophagus or adjacent structures, notably esophageal incisions and resections, pneumonectomy, vagotomy, and diaphragmatic hernia repair): (a) Accidental injury to esophagus or its blood supply; (b) Esophageal perforation as consequence of adjacent postoperative infection; (c) Postoperative esophageal anastomotic or suture-line leakage. E. Miscellaneous: 2ry to disease of CNS, 2ry to erosion by cancer, specific infection, idiopathic.

Treatment Treatment consists of (1) repairing the laceration; (2) covering it; (3) draining the mediastinum and neighboring cavities; and (4) discharging the esophagus of any saliva or gastric residue. On occasion, it can be necessary also (5) to exclude; (6) resect; and (7) replace part of the esophagus. N.B. Steps 5, 6, and 7 are basically required in cases of corrosion, or if treatment is late. 1. Repair (in 2 layers): Cervical approach, thoracotomy, L. thoracotomy, and Laparotomy (possibly with high gastrostomy)with direct suturing or Thai patch procedure.

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N.B. Repair is sometimes fragile, if not impossible. 2. Cover with: adjacent organs, pleural racket, pleural, pericardia! or diaphragmatic racket, and Nissen fundoplication. 3. Drain: Cervical drain, R. thoracostomy drain, L. thoracostomy drain (Aspiration at -12 cm H2O), and Abd. drain. 4. Discharge: Levin tube, intermittent asp. For 10 to 20 days or gastrosting, Parentral hyperalimentation Only, Aspiration through Abbott T-tube, and 5. Exclude: Pharyngastomy, Esophagostomy, Closure of cardia, and Gastrostomy- which later can be used for feeding. 6. Resect 7. Replace (to re-establish continuity of GIT). N.B. Steps 6, and 7 can be done in reverse order. Esophagectomy or esophagogastrectomy as required; with stomach, after Sweet (L. or R. thoracotomy); by jejunal interposition, after Cesar-Roux; by interposition of L. or R. colon (subcutaneous or retrosternal).

Esophagus - Caustic Burns

• Acid or alkaline injury • Accidental - children < 5 yrs, household cleaners • Intentional - adults with psych disorders, suicide attempts • Severity related to strength / duration of exposure (prox < distal) • Laryngeal edema - respiratory distress

Phases • Inflammation, edema, necrosis - initial few days • Ulceration, sloughing, granulation - 3 – 4 weeks • Cicatrization, scar formation - weeks -months • Long-term

o Recurrent strictures o Respond to dilatation

• Pre-cancerous: may develop up to 40 yrs later = Increased cancer risk 1000 fold (debated)

Treatment • Appropriate dilution, no emesis • Fluids, antibiotics, (steroids controversial) • Fiberoptic laryngoscopy / intubation • Esophagoscopy - 48 hours, stop at first evidence of burn • Barium swallow 3 - 4 weeks, intermittently thereafter • Later dilatation for strictures • Surgery usually not necessary early Sepsis / full-thickness necrosis: cervical esophagostomy, gastrostomy with isolation, later reconstruction, (resection controversial)

Traumatic Injuries of the Mediastinum Mediastinal Emphysema · Introduction of air from esophagus, tracheobronchial tree, neck, or abdomen · Causes include penetrating or blunt trauma, or spontaneous mediastinal emphysema · Presents as substernal chest pain, crepitation, and pericardial crunching sound · May result in tamponade

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· Treat underlying cause; may require chest tube placement for pneumothorax Traumatic Asphyxia • 10% of crush injuries of the chest. • 0.5% of all severe chest injuries. • Acute venous hypertension from mechanical obstruction of SVC such as when the victum is pinned beneath an automobile, machinery • Treatment is supportive, with focus primarily on the associated injuries. Prompt establishment of the ABCs of trauma management is critical, with special attention paid to reestablishing oxygenation and perfusion to ensure a successful outcome. Head elevation should be maintained at 30°.

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Spring 2016 2. Chest Wall Anomalies & Tumors

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2 Chest Wall Anomalies and Tumors

Table of Contents

2 Chest Wall Anomalies and Tumors ........ 2 Contents of The Chest Wall ................................... 4 Congenital Deformities Of Chest Wall ................... 4

Pectus Excavatum .............................................. 4 Other Deformities ............................................... 6

Chest Wall Tumors ................................................ 6

Incidence ............................................................ 7 Clinical Presentation .......................................... 7 Principles of Treatment ...................................... 7 Principles of reconstruction ................................ 7 Results ............................................................... 7

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Unit Objective At the end of this unit the resident: • understands the anatomy, physiology, and embryology of the

chest wall and interprets diagnostic tests. • understands acquired abnormalities and neoplasms of the chest

wall and performs biopsy, incision, resection, reconstruction, and stabilization of the chest wall.

• understands abnormalities, including those leading to thoracic outlet syndrome, and uses operative and non-operative therapy.

Learner Objectives Upon completion of this chapter the resident: 1. Understands the anatomy and physiology of the cutaneous,

muscular, and bony components of the chest wall and their anatomic and physiologic relationships to adjacent structures and the surgical anatomy of the vascular, neural, muscular, and bony components of the thoracic outlet;

2. Recognizes pectus excavatum and pectus carinatum, understands possible physiologic disturbances, and interprets diagnostic tests to identify such physiologic disturbances;

3. Evaluates and diagnoses primary and metastatic chest wall tumors, and understands the indications for incisional versus excisional biopsy and knows the radiologic characteristics of tumors and the types of chemotherapy and radiotherapy (induction neo-adjuvant and adjuvant therapy) of chest wall tumors and the indications for preoperative and postoperative therapy;

4. Knows the indications for and methods of prosthetic chest wall reconstruction (e.g., methyl-methacrylate, Marlex®, Gortex®, Vicryl®, and Dacron® mesh);

5. Understands the etiology, evaluation, differential diagnosis, and diagnostic criteria for thoracic outlet syndrome and knows the operative and non-operative management of thoracic outlet syndrome

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Contents of The Chest Wall I-Chest wall anatomy

a- Skeletal b- Muscular c- Neural d- Vascular e- Relationships to adjacent structures

II-Diagnostic tests to define chest wall anatomy a- Chest x-ray b- CAT scans c- MRI scans d- Nuclear scans e- Pulmonary function tests

III-Major flaps of the chest wall and their vascular pedicles: Latissimus dorsi, Pectoralis major, Serratus anterior, Trapezius, Intercostal,Pleural, pericardial fat pad, Rectus abdominis, Omental, Vascularized rib graft.

Congenital Deformities Of Chest Wall

Pectus Excavatum

• Most common congenital sternal deformity, occurring in 1 in 400 children

• Excessive growth of lower costal cartilage results in sternal depression

• Usually causes a deeper depression on the R., pushing heart to the L.

• Congenital with progressive worsening over time • Scoliosis in the setting of pectus excavatum usually involves a

single curve between T4 and T9 • MVP has been observed in 40-65% of pectus excavatum patients.

There may be a mechanical etiology, as MVP often resolves after operation. As many as 2/3 of Marfan patients have pectus excavatum

• Rarely familial

Physiologic Manifestations • Usually asymptomatic • Subjective decrease in respiratory reserve with exercise • Scoliosis and mitral valve prolapse have been associated with

pectus excavatum • Decreased maximal voluntary ventilation and a mild restrictive

pattern on PFTs has been documented in some studies • Decreased SV and CO during upR. exercise has also been

demonstrated Physiologic Manifestations of Pectus Excavatum Subjective Asymptomatic Pain Decrease in respiratory reserve Objective

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Pulmonary function tests Decreased maximal voluntary ventilation Mild restrictive pattern

Hemodynamics Stroke volume and cardiac output reduced during upright exercise

Operative Indications • Cosmetic correction is the most common reason • Psycho-social factors, however, may be quite limiting, particularly

in older children • Respiratory insufficiency and recurrent pulmonary infections • Best results are obtained in patients between the ages of 3 and 5

Fonkalsrud EW, Salman T, Guo W, Gregg JP. Repair of pectus deformities with sternal support. Journal of Thoracic and Cardiovascular Surgery 1994 107(1):37-42.

Operative Technique A. Ravitch repair · Midline or transverse inframammary incision · Pectoralis reflected bilaterally to expose costal cartilages · Subperichondrial resection of all deformed costal segments · Elevate sternum from underlying structures and separate from cartilage · Transverse sternal osteotomy and fixation with pin or cartilage support

B. Sternal eversion · En bloc excision of sternum and associated deformed cartilages · Free graft everted and fixated · Alternatively, the graft can be mobilized on an internal mammary artery pedicle · New anterior surface of the sternum shaped to form proper contour

C. Prosthetic implants · Silastic or other prosthetic molds generally give poor results

Haller JA Jr, Scherer LR, Turner CS, Colombani PM. Evolving management of pectus excavatum based on a single institutional experience of 664 patients. Annals of Surgery 1989 209(5):578-82.

Results • Cosmetic results are good in 80-90% • Recurrence occurs in about 10-20% of patients • Surgical correction in a Marfan patient has a higher recurrence rate • Return of normal respiratory function • Improvement in cardiac output • Improvement in maximal oxygen consumption and voluntary

ventilation

Peterson RJ, Young WG Jr, Goldwin JD, Sabiston DC Jr, Jones RH. Noninvasive assessment of exercise cardiac function before and after pectus excavatum repair. Journal of Thoracic and Cardiovascular Surgery 1985 90(2):251-60.

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Other Deformities

Pectus Carinatum • More common in males and is associated with scoliosis • Usually presents as anterior sternal displacement with symmetric

costal cartilage concavity(90%) • Asymmetric deformity with unilateral concavity of the costal

cartilages (9%) • Chondromanubrial synostosis (Curranino-Silverman syndrome) (1%) • Treatment is cosmetic by cartilage shaving or resection. Costal

cartilage resection gives excellent results

Poland's syndrome • Unilateral absence of pectoralis major with hypoplasia or aplasia of

ipsilateral breast and ribs, and bradysyndactyly • More common in males, usually occurs on the R. side, and is most

often sporadic • Operative repair involves rib grafts and prosthetic patching of the

chest wall

Sternal fissure • Complete, upper, or distal varieties occur • Narrow cL.s can be closed primarily after mobilization by oblique

chrondotomies • Complete cleft • . Associated with anterior diaphragmatic defect and diastasis recti

. Requires extensive surgical correction • . Cantrell’s pentalogy • . Distal cleft, omphalocele, anterior diaphragm defect, pericardial

defect, and CHD • Cardiac anomaly is usually VSD, TOF, ASD, or an LV diverticulum • Broader cL.s may require a prosthesis to avoid compressing the heart

Cantrell's Pentalogy • Characterized by a distal cL., omphalocele, diaphragmatic cL.,

pericardial defect, and congenital heart defect (usually VSD or TOF • One-stage repair is usually possible

Poland’s Syndrome • Absence of the sternum • Variable absence of the pectoralis major and minor, serratus anterior,

rectus abdominus, and latissimus dorsi • Hypomastia or amastia • Ipsilateral abnormality of the hand • 3 X more common in males • 75% on the right side • Possibly caused by disruption of the subclavian or vertebral blood

flow in utero • There is little physical disability

Chest Wall Tumors

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Incidence · Comprise 7-8% of all bony tumors · Most primary chest wall tumors are malignant · 85-90% occur in the ribs (50% malignant) · 10-15% occur in the sternum (95% malignant) · Male:female = 2:1

Clinical Presentation · Slowly enlarging mass eventually causes pain and presence of mass · Pain is more common in malignant tumors, but 20-25% are asymptomatic · Tumors occur at any age and are more likely to be malignant in older patients · CXR with rib detail films and CT scan are usually adequate and can evaluate associated pulmonary nodules · MRI distinguishes nerve and vascular invasion

Anderson BO and Burt ME. Chest wall neoplasms and their management. Annals of Thoracic Surgery 1994 58(6):1774-81.

Etiology of The Chest Wall Tumors Malignant Benign Chondrosarcoma Myeloma Osteogenic sarcoma Ewing's sarcoma

Fibrous dysplasia (40%) Chondroma (30%) Osteochondroma Desmoid

Principles of Treatment · Excisional rather than incisional biopsy should be peformed if a primary chest wall tumor is suspected · Full thickness excision of the tumor with 1 rib margin is necessary; do not compromise resection to avoid large chest wall defect · Large tumors may warrant incisional biopsy · Needle biopsy is best for suspicious mets or myeloma · Sternal tumors should be treated by sternectomy

Principles of reconstruction · A defect less than 5 cm does not require reconstruction · Posterior defects do not require reconstruction due to scapula · Defects larger than 5 cm will require reconstruction · Skeletal stabilization can be accomplished with a mesh patch or methyl methacrylate · Soft tissue reconstruction can be done in a variety of ways, including myocutaneous flaps (latissimus dorsi, pectoralis major, rectus abdominus) and omental transposition

Sources for further reading Textbook Chapters Chapter 15: Disorders of the Sternum and the Thoracic Wall. Surgery of the Chest (Sabiston and Spencer), 6th ed., 494-521.

Chapters 33 and 34: Chest Wall Abnormalities and Chest Wall Tumors. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 581-608.

Results · Low operative mortality and good postoperative pulmonary function · Overall long term survival is about 50-70%, with best rates for chondrosarcoma and rhabdomyosarcoma, and worst rates for malignant fibrous histiocytoma · Survival is better with wide excision · Adjunctive therapy may improve survival

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Spring 2016 3. The Diaphragm

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3 The Diaphragm

Table of Contents

3 The Diaphragm ......................................... 2 Anatomical Considerations .................................... 4 Congenital Diaphragmatic Hernias ........................ 4

Bochdalek's Hernia ............................................. 4 Morgagni's Hernia .............................................. 4

Esophageal Hiatal Hernia .................................. 4 Traumatic Perforation ............................................ 5 Tumors of the Diaphragm ...................................... 5 Diaphragmatic Pacing ........................................... 5

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Chapter Objective

At the end of this chapter the resident understands the anatomic, embryologic, physiologic and the pathogenesis and management of diaphragmatic diseases.

Learner Objectives Upon completion of this chapter the resident: 1. Understands the anatomical and radiological compartments, as

well as the the lymphatic drainage; 2. Understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for congenital diaphragmatic hernias; 3. Traumatic perforations; 4. Knows the pathogenesis and management of the primary

diaphragmatic tumors; 5. 5) Knows the indications, mechanism, and techniques of the

diaphragmtic pacing

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Anatomical Considerations Anatomy · Composed of a central tendinous portion and a peripheral muscular portion · Muscular portion consists of sternal, costal, and lumbar components · Three major openings: aortic (aorta, azygos vein, thoracic duct), esophageal (esophagus, vagus nerves), caval (IVC) · R. and L. phrenic arteries arise from the abdominal aorta · Additional arterial supply from pericardiophrenic and musculophrenic arteries · Venous drainage is via R. and L. phrenic veins to the IVC; some drainage to the L. renal vein as well · R. and L. phrenic nerves supply both sensory and motor innervation

Sources for further reading Textbook Chapters

Chapter 29: Diaphragm and Diaphragmatic Pacing. Surgery of the Chest (Sabiston and Spencer), 5th ed., 957-73.

Chapters 35 and 36: The Diaphragm: Developmental, Traumatic, and Neoplastic Disorders; The Diaphragm: Dysfunction and Induced Pacing. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 609-42.

Congenital Diaphragmatic Hernias

Bochdalek's Hernia

· Occurs posterolateral in the area of the 10th and 11th ribs · 90% occur on the L. · 2:1 male to female incidence · Usually isolated and not associated with other congenital defects · Typically manifests as acute respiratory distress · CXR demonstrates intestine in the thorax and shift of mediastinal contents to the R. · Initial treatment includes NG decompression, positive-pressure ventilatory support, and surgical correction · Approach L.-sided defect through the abdomen in order to explore for malrotation and obstruction · R.-sided defects are repaired through a thoracotomy · Postoperative mortality can be as high as 50%, mostly attributed to increased pulmonary vascular resistance · ECMO is useful to reduce pulmonary vascular resistance and help resolve persistant fetal circulation

Heiss KF. Congenital diaphgramatic hernia in 1994: A hard look at the need for "emergency surgery". Seminars in Thoracic and Cardiovascular Surgery 1994 6(4):221-7.

Morgagni's Hernia

· Defect occurs in a subcostosternal location · Uncommon (less than 3% of diaphragmatic hernias) and usually asymptomatic · Well defined hernia sac becomes symptomatic typically after age 40, when obesity, pregnancy, or trauma increases intraabdominal pressure · The transverse colon is the most common organ to herniate, and can present as an acute colonic obstruction · Repair is usually performed through a upper midline incision

Esophageal Hiatal Hernia

· Congenital defects causing these hernias are uncommon in adults, but some neonates and infants may have reflux associated with an esophageal hiatal hernia · Typical symptoms are vomiting, respiratory complications, anemia, and failure to thrive · Diagnosis rests on esophagography, fluoroscopy, and pH monitoring

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· Treatment is primarily medical; surgery is indicated for medical failure Traumatic Perforation · Penetrating perforation should be suspected with any thoracic injury below the level of the nipples (5th ICS) · Most blunt hernias are caused by automobile accidents, and about 90% occur in the L. hemidiaphragm · Blunt trauma defects are large, usually about 10-15 cm, and typically located in the posterior L. hemidiaphragm · Stomach is the most commonly herniated organ, followed by spleen, colon, small bowel, and liver · Respiratory insufficiency is common early, while intestinal obstruction predominates later · CXR and CT scan will diagnose most; barium contrast is contraindicated, as it can produce a total obstruction in this setting · Missed injury and delayed diagnosis commonly leads to bowel incarceration and obstruction · Mortality is relatively high (15-40%) due to high incidence of associated injuries · Repair should be undertaken promptly with full exploration for other injuries · L.-sided perforation should be repaired through the abdomen to allow correction of associated injuries · R.-sided perforations may require thoracotomy

Shah R, Sabanathan S, Mearns AJ, Choudhury AK. Traumatic rupture of diaphragm. Ann Thorac Surg 1995;60:1444-9.

Tumors of the Diaphragm A. Primary · Rare tumors; cysts are more common than inflammatory masses, which are more common than neoplasms · Equal male:female incidence; L.-sided tumors are slightly more common than R.-sided tumors · Symptoms include pain, cough, dyspnea, and GI symptoms · CXR and CT scan will localize the tumor · The majority of neoplasms are benign (60%), which are usually cysts · Up to 40% are malignant, usually sarcomas · Treatment includes excision and closure of the diaphragmatic defect

Anderson LS and Forrest JV. Tumors of the diaphragm. American Journal of Roentgenology, Radium Therapy and Nuclear Medicine 1973 119(2):259-65.

B. Metastatic · Most neoplastic involvement of the diaphragm occurs from contiguous extension of nearby tumors · The most common lesions arise from lung, esophagus, stomach, liver, and the retroperitoneum · Treatment is based on the primary tumor

Diaphragmatic Pacing A. Indications · Sarnoff (1940's) and Glenn (1950's) were the primary developers

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of diaphragmatic pacers · Pacing is indicated in patients who have chronic ventilatory insufficiency with normal nerves, lungs and diaphragm · This includes some quadriplegic patients and central alveolar hypoventilation · Contraindications to pacing are lower motor neuron dysfunction, muscular dystrophy, and extensive lung disease B. Mechanism · There are four components to a diaphragmatic pacer: 1) Transmitter: sets respiratory rate and length of inspiration 2) Antennae: transfers signal across intact skin to the receiver 3) Receiver: obtains signal and energy from external portion by inductive coupling 4) Electrode: stimulates the phrenic nerve · The electrode portion is usually implanted on the phrenic nerve through the 2nd ICS anteriorly · The receiver is placed in a subcutaneous pocket

C. Central Alveolar Hypoventilation · Features of CAH include: hypoxemia and hypercapnia increasing with sleep, hypoventilation or apnea during sleep, and clinical findings of cyanosis, polycythemia, and cor pulmonale · These patients have near-normal ventilatory capacity tests, but have a reduced response to induced hypoxemia and hypercapnia · Absence of upper airway obstruction or persistence after relief must also be demonstrated · These patients should begin pacing within 3 weeks of operation

Stemmer ED, Crawford DW, List JW, Heber RE, Connolly JE. Diaphragm pacing in the treatment of hypoventilation syndrome. Journal of Thoracic and Cardiovascular Surgery 1967 54(5):649-57.

D. Quadriplegia · Patients with high cervical lesions (C1 or C2) are suitable candidates; injury to C3-C5 may injure the motor component of the phrenic nerves, preventing adequate pacing · Delay surgery for several months to allow for potential recovery after spinal cord injury · Pacing should be gradually introduced to avoid diaphragmatic fatigue and permanent damage · Patients should be selected who are good candidates for long-term rehabilitation

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Spring 2016 4. The Pleurae

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4 The Pleurae

Table of Contents

4 The Pleurae ............................................... 2 Pleural Space Anatomy ......................................... 4 Pleural Space Physiology ...................................... 4 Pleural Effusions .................................................... 5 Malignant Pleural Effusions ................................... 5 Pneumothorax ....................................................... 8 Thoracic Duct, Anatomy ...................................... 12 Chylothorax ......................................................... 12

Infections of The Pleura ....................................... 14 Postpneumonic Empyema ........................... 14

Pleural Tumors .................................................... 18 Pleural plaques ................................................ 19 Mesothelioma ................................................... 19

Operations for The Pleura ................................... 24 Decortication .................................................... 24 Thoracoplasty ................................................... 24

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Chapter Objective

At the end of this chapter the resident understands the anatomic, embryologic, physiologic and the pathogenesis and management of pleural diseases.

Learner Objectives Upon completion of this chapter the resident: 1. Understands the pleural space anatomy; 2. Understands the pleural space physiology; 3. Understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for non-malignant pleural effusions; 4. Understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for malignant pleural effusions; 5. Knows the anatomy of the thoracic duct and the pathogenesis and

management of the chylothorax; 6. Knows the operative management of infections of the pleura

(postpneumonic empyema and postresectional empyema ±BPF); 7. Knows the pathogenesis and management of the pleural tumors

(benign localized, benign diffuse and diffuse malignant mesotheliomas).

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Pleural Space Anatomy • -mesothelial cells • -parietal/visceral components • Parietal and visceral surfaces create a potential space in the chest cavity

Histology: single layer of mesothelial cells resting on an elastic membrane Function: fluid resorption, phagocytosis, etc

Blood supply • -Parietal—Systemic>>intercostal, bronchial, and subclavian arteries • -venous drainage into the peribronchial veins • -Visceral—Systemic and pulmonary circulations • -venous drainage into the pulmonary venous system • Parietal surface is supplied by the systemic circulation only (intercostal, mam mary and bronchial vessels) • Venous drainage is by the peribronchial veins that empty into the vena cava • Visceral surface has a dual blood supply (systemic and pulmonary) • Venous drainage is through the pulmonary veins.

Lymphatic system—two systems • -visceral—pulmonary system (lower lobes) -parietal—direct communications with the pleural space—stomata • Visceral lymphatics are subpleural and distributed more over the lower lobes • Parietal • Anteriorly to the mammary lymph nodes Posteriorly to the intercostal lymph nodes Diaphragmatic drainage is to the retrosternal, mediastinal and celiac nodes Transdiaphragmatic anastomoses allow for the passage of fluid and particles from the peritoneal cavity into the pleural space

Innervation: • Visceral pleura is devoid of somatic innervation • Parietal pleura is innervated with somatic, sympathetic and parasympa thetic fibers • Pain from the diaphragm is transmitted via the phrenic nerve

Pleural Space Physiology -pleural pressure is essentially negative during the breathing cycle—at FRC, pressure is –2 to –5 cm H2O; full inspiration –25 to –35 cm H2O -more negative at the apex than the base Fluid—200cc to 1L absorbed Q24 hours There is a near constant negative intrapleural pressure: At FRC (functional residual capacity) -2 to -5 cm H2O With inspiration the pressure drops to -25 to -35 cm H2O The lung and bony thorax represent two elastic structures coupled in series.

Pleural fliud dynamics (formation and reabsorption) is normally follow the Sarling equation, which depends on hydrostatic, colloid, and tissue pressures,

Fluid is continuously produced and reabsorbed Visceral surface absorbs fluid secreted from the parietal pleura

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Parietal pleural lymphatics absorb the excess fluid Inspiration Expiration Intra-Alveolar Pressure*

-3.0 mm Hg +3.0 mm Hg

Intrapleural Pressure*

-6.0 mm Hg -3.0 mm Hg

Difference in Pressures* (i.e., Pressure Gradient)*

+3.0 mm Hg [ -3 - (-6) = +3 ]

+6.0 mm Hg [ +3 - (-3) = +6 ]

Pleural Effusions • Lymphocytes predominate in advanced TB, lymphoma, or malignancy • Neutrophils predominate in pneumona, pulmonary infarction, pancreatitis, and early TB • Empyema can be differentiated from chylothorax by centrifugation, which gives a clear supernatant in empyema • Black fluid suggests Aspergillus • Grossly bloody effusions are due to trauma, pulmonary infarction, or malignancy • Glucose level below serum glucose is seen in Tb, rheumatoid arthritis, empyema, and malignancy • VATS can establish the diagnosis in 80-95% of effusions that remain undiagnosed after thoracentesis and needle pleural biopsy • The most common causes of malignant pleural effusions are lung cancer in men and breast cancer in women • Effusion develops in 50% of patients with Dressler syndrome, and is generally small in volume • Effusion develops in 50% of patients with a PE with or without a pulmonary infarction

A combination of effusion cytology and pleural biopsy can make a diagnosis of Tb in 70-95% and malignancy in 60-90%

Malignant Pleural Effusions -due to a disturbance in the equilibrium of production and absorption of fluid -lymphatic obstruction esp. with paraneoplastic effusions

Malignant Pleural Effusion (MPE) Accumulation of fluid in the pleural space with malignant cells (diagnosed in the pleural fluid or by pleural tissue by closed needle biopsy or through thoracoscopy or thoracotomy or at autopsy).

Paramalignant Pleural Effusion (P/MPE): A documented malignancy with pelural effusion and failure to demonstrate malignant cells in pleural fluid nor pleural tissue.

Mechanism of formation: Impaired pleural space lymph, drainage. Of all the pleural effusions 20-50% are malignant Most associated with advanced lung or breast cancers

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Occasionally secondary to pleural metastasis from ovarian, GU or GI malignancies Mesothelioma, metastatic sarcoma, or lymphoma are less common Often the first sign of and first diagnostic source for a malignancy Pathophysiology There is an increased capillary permeability associated with tumor implants that cause an increase in fluid transudation, while lymphatic obstruction impairs reabsorption of the effusion. Fluid: Nearly all malignant effusions are exudates. • Appearance - nonspecific, bloody or milky (chyle) • Biochemistry - exudate • Protein > 3 grams/dL • pH < 7.3 • Amylase > 160 U • LDH > 200 U • Glucose < 60 mg/dL • Pathology - [+] cytology and histology • Miscellaneous -

o Positive chromosomal examination o High CEA (> 20 mg/dL) content o Pathophysiology o Fluid characteristics o • Appearance Chemistry (exudate) o • Protein > 3 g/dl; LDH > 200 u pH < 7.3; glucose < 60

mg/dl o Amylase > 160 u o • Cytology, Histology

V.A.T.S.

Management • Management—palliation/relief of symptoms (usually dyspnea) is the goal—removal of effusion • -options—pleurectomy; mechanical pleurodesis; talc poudrage; pleuroperitonealshunt; tube thorocostomy and sclerosis • Repeated thoracentesis Chest tube drainage with intrapleural sclerosis • Empty pleural space

Expandable lung CT output < 150 - 200 cc/day

• Pleurectomy Miscellaneous • Shunts, XRT • Hormonal therapy, chemotherapy VATS VATS increases the diagnostic yield to 90-95% and is recommended when two aspirations have not given the diagnosis Procedure of choice for simultaneous diagnosis and treatment

Video Assisted Thoracotomy Indications - Diagnosis Undiagnosed pleural effusion Pleural based lesion Tumor staging and diagnosis (biopsy) Evaluation of thoracic injury 4. Indications - Therapeutic Pneumonolysis Foreign body extraction Pleurodesis Debridement of empyema cavity Control of hemorrhage Clot evacuation Sealing of pneumothorax

Video Assisted Thoracic Surgery (VATS) • VATS is useful in the management of many pleural diseases: • Diagnosis and treatment of pleural effusions (the diagnostic yield

is increased with biopsies, etc.) • Pleurodesis (for effusions or pneumothorax) • Evaluation and resection of pleural based lesions • Tumor staging and diagnosis (biopsy), determine resectability,

evaluate contralateral lesions • Evaluation of thoracic injury and hemorrhage, evaluate diaphragm

and intercostals, control of hemorrhage, clot evacuation • Foreign body extraction

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• Pneumonolysis • Debridement of empyema cavity N.B.: The surgeon should be prepared for an open thoracotomy (thoracic surgeon) Malignant Pleural Effusion - Management The goal is to empty the pleural space and expand the underlying lung in an effort to obtain apposition of the two pleural surfaces Chest tube drainage is the least invasive Sclerosing agents can be instilled through the chest tube, but this is slow and sometimes ineffective Sclerosis has been achieved with a variety of materials including: • Quinacrine • Mustard • Radioisotopes • Bleomycin • Tetracycline • Talc •

Agent % Success Range % Mean Quinacrine 64-100 86 Mustard 7-85 44 Radioisotopes 28-80 56 Bleomycin 60-85 71 Tetracycline 25-100 69 Talc 72-100 96

Baue AE, et al, Glenn's Thoracic & Cardiovascular Surgery. 1996,1:548.

Although some are very effective they can have undesirable side effects: • Radioisotopes - no longer available • Bleomycin - for sclerosing effects • Tetracycline - very good but no longer available • (can substitute doxycycline) • Talc - full circle, fibrosis possible Pleurectomy can be performed if sclerosis is unsuccessful (VATS) This is good with malignancies but very painful, most invasive and can cause significant bleeding Pleuroperitoneal shunts: The fluid can be reabsorbed in the peritoneal space. Potential spread of tumor and it requires repeated manual pumping. Low rate of patient compliance PleuRx catheter (Denver shunt) - outpatient management Chemotherapy with or without radiation therapy can be used, usually with lymphoma and small cell lung carcinoma. Hormonal treatment is possible for breast cancer. Diagnosis Pleural Tumors • Pleural plaques are discrete 1-5 mm areas of thickened parietal pleura of unknown cause • The relationship of pleural plaques to the development of mesothelioma is uncertain • Primary pleural neoplasms are rare compared to metastatic lesions • Lipomas are the most common benign pleural lesions and are usually well encapsulated, free from underlying tissue, and firmly fixed to the pleura • Many can be diagnosed and resected by VATS

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•

• • FIGURE 4− . Algorithm for the management of MPE

Pneumothorax

Causes of Secondary Spontaneous Pneumothorax Airway disease

Bullous disease COPD Asthma Cyst (congenital) Cystic fibrosis

Interstitial disease Idiopathic pulmonary fibrosis Eosinophilic granuloma Sarcoidosis Tuberous sclerosis Collagen vascular disease

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Causes of Secondary Spontaneous Pneumothorax

Infections Anaerobic pneumonia Staphylococcal pneumonia Gram-negative pneumonia Lung abscess Actinomycosis Nocardiosis Tuberculosis Atypical mycobacteria

Neoplasms Primary Metastatic

Other diseases Endometriosis Ehlers-Danlos syndrome Pulmonary embolism Marfan's syndrome

Classification of Pneumothorax Spontaneous Primary No underlying pathology Secondary Underlying pulmonary disorders Catamenial Neonatal Traumatic Iatrogenic Thoracentesis, mechanical ventilation, central vein cath., postoperative Blunt, penetrating Diagnostic Air-contrast study of pleuro-pulmonary pathology

Indications for Thoracotomy Massive air leak that prevents re-expansion of the lung Persistent air leak > 7-10 days N.B.: 7 - 10 days (traditional) 2 - 4 days (recent) Second episode of spontaneous PTX Complications of PTX

Hemothorax Empyema Chronic PTX

Bertrand PC, Regnard JF, Spaggiari L, et al. Immediate and long-term results after surgical treatment of primary spontaneous pneumothorax by VATS. Ann Thorac Surg. 1996 61 (6):1641-5.

Cardillo G, Facciolo F, Giunti R, et al. Videothoracoscopic treatment of primary spontaneous pneumothorax: a 6-year experience. Ann Thorac Surg. 2000 Feb;69(2):357-61; discussion 361-2.

Chan P, Clarke P, Daniel FJ, et al. Efficacy study of video-assisted thoracoscopic surgery pleurodesis for spontaneous pneumothorax. Ann Thorac Surg. 2001 Feb;71(2):452-4.

Cole FH Jr, Cole FH, Khandekar A, et al. Video-assisted thoracic surgery: Primary therapy for spontaneous pneumothorax? Ann Thorac Surg. 1995 60(4):931-3.

de Campos JR, Vargas FS, de Campos et al. : a 15-year experience. Chest. 2001 Mar;119(3):801-6.

Hatz RA, Kaps MF, Meimarakis G, et al.. Long-term results after video-assisted thoracoscopic surgery for first-time and recurrent spontaneous pneumothorax. Ann Thorac Surg. 2000 Jul;70(1):253-7.

Klingman RR, Angelillo VA, DeMeester TR. Cystic and bullous lung disease. Ann Thorac Surg. 1991 52(3):576-80.

Lang-Lazdunski L, de Kerangal X, et al. Primary spontaneous pneumothorax: one-stage treatment by bilateral videothoracoscopy. Ann Thorac Surg. 2000 Aug;70(2):412-7.

Mouroux J, Elkaim D, Padovani B, et al. Video-assisted thoracoscopic treatment of spontaneous pneumothorax: technique

Specific surgical indications for conditions that cause secondary spontaneous PTX Occupational indications after first episode Airline pilots Scuba divers Individuals in remote areas Previous contralateral PTX Bilateral simultaneous PTX Presence of large cysts visible on CXR

Clinical Presentation and CXR

In 80% symptoms occur at rest or during normal activity Pain - most frequent - pleuritic/sharp Dyspnea - second most common symptom

Large PTX Underlying pulmonary disease Pronounced with tension PTX

Orthopnea Hemoptysis Non-productive cough > 25% - diminished motion of chest wall on the affected side Cyanosis Hyper-resonant; tympanitic Decreased breath sounds Subcutaneous emphysema/mediastinal emphysema Tracheal deviation CXR

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and results of one hundred cases. J Thorac Cardiovasc Surg. 1996 Aug;112(2):385-91.

Sahn SA, Heffner JE. Spontaneous pneumothorax. N Engl J Med. 2000 Mar 23;342(12):868-74.

Primary Spontaneous Pneumothorax Young adults, 85% < 40 years 9 per 100,000 Tall, thin male, 25 to 30, smoker, family history Previous history, 90% chance it is on the same side Simultaneous bilateral - 10% Ruptured bleb - 15% seen on CXR at apex, along fissures

85% found at thoracotomy Recurs with increasing frequency

50% after first episode 62% after second episode 80% after third episode

Secondary spontaneous pneumothorax 20% event is related to underlying pulmonary disease

Spontaneous Pneumothorax Neonatal Spontaneous Pneumothorax

Hyaline membrane disease Renal malformation Potter's syndrome Meconium aspiration Cystic fibrosis in children

Catamenial Spontaneous Pneumothorax Occurs during menstruation Third and fourth decades 90% on the R. side Does not occur during periods of non-ovulation Four possible causes

Rupture of bleb Alveolar rupture due to PF2 Air into pleural cavity from GYN tract Pleural/pulmonary endometriosis

Management of Spontaneous Pneumothorax Observation if PTX < 20% Thoracenteses 30-70% effective Thoracostomy Chemical pleurodesis Mechanical pleurodesis Treatment of underlying disease

Caveats One can never be faulted for placing a chest tube (unless the chest tube was placed in the abdomen). The first thing into the chest is your finger. Usually, do not place a chest tube below the nipple line.

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FIGURE 4− . Algorithm for management of primary and secondary spontaneous pneumothorax. (Modified and reproduced from Rivas de Andrés, JJ et al. Guidelines for the Diagnosis and Treatment of Spontaneous PneumothoraxArch Bronconeumol. 2008;44:437-48).

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Thoracic Duct, Anatomy • The duct begins at the cysterna chyli at T12, L1, L2 • Ascends into the right posterior mediastinum through the aortic opening and is extrapleural between the azygos vein and aorta on the vertebral bodies behind the esophagus • Crosses over at T5 (tracheal bifurcation) and then travels behind the arch to the left of the esophagus • In the neck it enters the venous system at the junction of the left internal jugular and subclavian veins • The right duct collects chyle from the right extremity and the right side of the mediastinum • It enters the venous system at the junction of the right internal jugular and subclavian veins • Normal thoracic duct anatomy exists in about 50% of patients In the remainder, 2 or more main ducts are present at some point

Chyle Mechanics of flow: Pressure gradient between the chest and abdomen contributes to the flow of chyle from abdomen to the chest There is also autonomous contraction of the duct and a suction-like (siphon) effect of the veins Valves are present in the thoracic duct to help with unidirectional flow High in triglycerides 0.4 - 6 grams/dL High in protein (1-7 grams/dL) with the albumin to globulin ratio 3:1 The WBC count is usually between 2,000 and 20,000 with a predominance of T-cell lymphocytes (90%) and the remainder being mostly eosinophils

Chyle: 1500-2400 cc/day (up to 200 cc/hr after a fatty meal)

Chylothorax

Etiology of Chylothorax Etiology A chylothorax can be secondary to congenital, traumatic, or non-traumatic causes. Congenital: Birth trauma, congenital fistula or malformation of chyle vessels or ducts. Mostly from leaking vessels. Traumatic: Usually from surgical injury; penetrating or blunt trauma is very rare. Non-traumatic: Malignancies, radiation fibrosis, local inflammation, obstruction of the duct or invasion by tumor. Superior vena cava obstruction. The most common mechanism of nonpenetrating injury is sudden hyperextension of the spine with rupture of the duct just above the diaphragm The duct is most vulnerable during surgery in the upper part of the left chest, particularly during operations that involve mobilization of the aortic arch, left subclavian artery, or esophagus Tumor is responsible for more than 50% of chylothoraces in adults 75% of these are due to a mediastinal retroperitoneal lymphoma The rest are usually lymphosarcomas or lung carcinomas

4 types of chylothorax 1. Congenital. 2. Traumatic non-surgical. 3. Traumatic postoperative. 4. Non-traumatic

Trauma and neoplasms are the most frequent causes of chylothorax

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TABLE 4-6. Pathogenesis of chylothorax Classification Etiology Pathogenesis

Congenital Birth injury Congenital fistula Malformation of lymphatics

• Direct leakage from thoracic duct • Leakage from dilated collateral

pathways Acquired traumatic Postoperative

Blunt trauma Penetrating trauma

• Tear in thoracic duct

Acquired non-traumatic Neoplastic Nonneoplastic

• Ductal compression with increase intraductal pressure & secondary transudation

• Disruption of ductal lumen integrity

• Increased shearing forces on the duct

• Transdiaphragmatic leakage of chyle

SVC obstruction Intravenous catheters • Increase in intraductal pressure with secondary rupture

Source: Baue AE, et al. Glenn's Thoracic and Cardiovascular Surgery. 1996, 1:552

Chylothorax, Diagnosis • Diagnosis: R>L; CXR with effusion, analysis of effusion with the

characteristics above • “Pseudochyle” must be distinguished from true chyle • It is a result of pleural tumor or long-standing empyema • Chyle is milky and nonclotting, and contains fat globules that

clear with alkali and ether and stain with Sudan III • Psuedochyle is a milky turbid fluid in association with thickened

pleura from chronic infection, tuberculosis, rheumatoid arthritis, diabetes, and malignancy

• Most chylous effusions have a triglyceride level above 110 mg/dL

Chylothorax, Management • Initial management should be non-surgical with chest tube

drainage • This allows recording of accurate daily amounts and more

continuous expansion of the lung • Controlled diet with medium-chain triglycerides • These are directly absorbed via the portal system and not the

intestinal lymphatics, which decreases the production of chyle • TPN if dietary control is inadequate • Non-traumatic chylothoraces may be managed with non-

operative therapy for longer periods of time with thoracentesis, chest tube, XRT, sclerosis, or shunts

Modalities used in management of chylothorax Conservative treatment • Treatment of the

causative disease • Repeated thoracentesis • Continuous drainage • Dietary modifications • Medium chain

triglycerides • Exclusively parenteral

nutrition* • Pleurodesis using

endothoracic tube* Surgical treatment • Thoracoscopic pleurodesis • Pleuroperitoneal pump • Fibrin glue to seal the

thoracic or intestinal duct • Thoracic duct ligation via

thoracoscopy or

Indications for surgery: • Drainage over 14 days • Drainage greater than 1500 ml/day or 100 ml/year-age/day in

children • Metabolic complications • Trapped lung • Suspicion of malignancy • Multiple loculated fluid collections

About 20-50% of patients will require surgery Traumatic will more likely need surgical intervention

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If secondary to esophageal resection or pneumonectomy, operation should be performed sooner

thoracotomy • Anastomosis between the

thoracic duct and the azygos vein

• Lung transplant (in lymphangio-leiomyo-matosis) In patients with concurrent ascites

• Peritoneal venous shunts • Closure the diaphragmatic

aperture with fibrin or suture using thoracoscopy In the fetus Intrauterine thoracentesis

• Prenatal pleuroamniotic shunt

Chylothorax, Surgical Technique There are three different surgical approaches. • Direct closure • Ligation of mediastinal pleura The surgeon enters the side with the effusion and mass ligates the area of leak. Give cream pre-operatively to increase the amount of drainage. • Duct ligation Enter the right hemithorax and mass ligate the tissue between the azygos vein and the aorta just above the diaphragm. Explore the left side later only if necessary Some surgeons add pleurectomy and pleurodesis to ligation if the site of the leak cannot be identified

With bilateral effusions, explore the right side first and ligate the duct low in the chest

Infections of The Pleura

Postpneumonic Empyema

Definition Accumulation of pus within the pleural space. It occurs in three phases: exudative/acute phase, fibrinopurulent/transitional phase, and organizing/chronic phase. It may be unilateral or bilateral, may be localized or diffuse, and may be acute or chronic. It is a disease entity. It may be defined as collection of a pleural fluid with the following 7 characters; Sp. gr. > 1.018, WBC > 500 cells/mm3, Ptn. > 2.5 g/cL, pH < 7, glucose < 40 mg/dL, LDH > 1000 U/L and positive Gram stain. It is an acute or chronic suppurative pleural exudate which may be caused by many organisms, eg., pneumococi, streptococci, staphylococci, bacteriodes E. coli, Proteus vulgaris, T.B., fungi and amebas. • Acute empyema: acute suppurative pleura! exudate in which there is

expansion of the lung after evacuation whatever the time factor is. • • Chronic empyema: chronic supurative pleural exudate in which the

lung fails to expand after evacuation of pus; whatever the • time. • • Localized empyema: The pus becomes thick and is walled off by

fibrinous adhesion resulting in a localized abscess. This abscess may be posterior or basal (basal empyema), near the mediastinum (mediastinal empyema), near the chest wall (peripheralempyema), near the apex of

Empyema = collection of pus in a natural body cavity. • Empyema thoracic (pleura! empyema) = Accumulation of pus within the pelural space. • Syn-pneumonic emyema (occurs during bronchopneumonia). • Meta-pneumonic empyema (occurs after resolution of pneumonia). • Para-pneumonic empyema is not related to chest disease.

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chest wall (apical empyema) or in an interlobar fissure (inter-lobar empyema).

FIGURE5−20. Algorithm for the management of parapneumonic pleural effusion. (Modified and reproduced from Garrido, VV et al. Diagnosis and Treatment of Pleural Effusion. Arch Bronconeumol. 2006;42:349-72).

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Etiology · About half are secondary to a pneumonia (postpneumonic empyema) · Other causes include pulmonary or esophageal operations, chest trauma, spontaneous pneumothorax, and subphrenic abscess · Pneumonias caused by S. aureus, E. coli, Pseudomonas, and anaerobes are the most likely to result in empyema -purulent pleural effusion with (+) bacteriologic cultures Post traumatic empyemas -penetration of the chest wall—foreign material carried into the pleural space -presence of a hemothorax—becomes secondarily infected from a chest tube

Complications most likely, e.g., empyema necessitatis-dissection of pus through the soft tissues of the chest wall and eroding through the skin

Stage I-parapneumonic effusion Stage II-fibrinopurulent phase; (+) bacterial invasion Stage III-chronic phase; ingrowth of fibroblasts and capillaries; thick peel

Clinical Presentation · Pleuritic chest pain, fever, tachycardia, and tachypnea are common · Persistent fever after resolution of pneumonia is suspicious for empyema

—fever, tenderness -posterior and lateral—extend to the diaphragm -CXR: inverted D-shaped density on the lateral chest film -most common organisms: S. aureus, G(-) bacteria, and anaerobes; almost 50% are polymicrobial -effusions with pH <7, glucose <50, LDH >1000, should be drained

Diagnosis · CXR may not differentiate pleural collection from lung consolidation or atelectasis · CT or US will help localize the fluid collection · Thoracentesis and aspiration of pus establishes the diagnosis · Chemical analysis of the fluid is critical

Empyema fluid characteristics Empyema fluid characteristics pH < 7.0 Glucose < 40 mg/dL LDH > 1000 IU/dL Positive Gram stain Positive culture (50%) Specific gravity > 1.018 WBC > 500 cells/mm3 Protein > 2.5 g/dL

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FIGURE 4−21. Algorithm for the management of pleural tuberculosis with pleural effusion (Modified and reproduced from Milier J.I., 1989).

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Management Complete drainage of the collection Obliteration of the empyema space Investigation and treatment of the underlying infection Management of associated conditions Decortication-goal is full lung expansion—via extensive debridement

-early aggressive approach—as soon as tube thorocostomy is ineffective Eloesser Flap—long term drainage of empyema

Removal of chest tube tract and carrying the incision down to the ribs Two ribs and the intervening intercostal muscles are removed Skin sutured to the pleura—dependant drainage insured

Clagett procedure—open window thorocostomy excision of the sinus tract instillation of antibiotics in the pleural cavity, closure of the chest wall described for post-pneumonectomy empyema best results (25-60%) when no bronchopleural fistula present Streptokinase Persistence of empyema usually is secondary to inadequate drainage chronic pulmonary disease (TB, Fungus, Neoplasm) immune suppression foreign body

Treatment A. Acute and Transitional Empyema · Thoracentesis followed by chest tube insertion · Ensure complete drainage of all localized collections B. Chronic Empyema · Open drainage with thorough cleansing of the cavity (saline or Dakin's solution) · Open chest tube drainage is then instituted, and the tube is slowly withdrawn at 10-14 day intervals · If the cavity is not closed after 6 weeks, a decortication (good candidates) or open rib resection or Eloesser flap (poor candidates) should be performed In summation for chronic empyema: -Tube thorocostomy -thorocoscopy/open decortication -Eloesser flap -Thoracoplasty/Muscle transposition

Angelillo Mackinlay TA, Lyons GA, Chimondeguy DJ, Barboza Piedras MA,

Angaramo G, and Emery J. VATS Debridement Versus Thoracotomy in the Treatment of Loculated Postpneumonia Empyema. Ann Thorac Surg 1996 61(6): 1626-1630.

Deschamps C, Bernard A, Nichols FC III, Allen MS, Miller DL, Trastek VF, Jenkins GD, and Pairolero PC. Empyema and bronchopleural fistula after pneumonectomy: factors affecting incidence. Ann Thorac Surg 2001 72(1): 243-248.

Gharagozloo F, Trachiotis G, Wolfe A, DuBree KJ, and Cox JL. Pleural space irrigation and modified Clagett procedure for the treatment of early postpneumonectomy empyema. J Thorac Cardiovasc Surg 1998 116(6): 943-948.

Houston MC. Pleural fluid pH: Diagnostic, therapeutic, and prognostic value. American Journal of Surgery 1987 154(3):333-7.

Schneiter D, Cassina P, Korom S, Inci I, Al-Abdullatief M, Dutly A, Kestenholz P, and Weder W. Accelerated treatment for early and late postpneumonectomy empyema. Ann Thorac Surg 2001 72(5): 1668-1672.

Tkebuchava T, Niederhäuser U, Weder W, von Segesser LK, Bauersfeld U, Felix H, Lachat M, and Turina MI. Kartagener's Syndrome: Clinical Presentation and Cardiosurgical Aspects. Ann Thorac Surg 1996 62(5): 1474-1479.

Wiedemann HP and Rice TW. Lung abscess and empyema. Seminars in Thoracic and Cardiovascular Surgery 1995 7(2):119-28.

Pleural Tumors

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Tumors of the pleura • Benign

• Lipomas (the most common). • Endotheliomas. • Angiomas. • Cysts of the pleura. • Benign solitary mesothelioma (1%).

• Malignant • Primary: diffuse malignant mesothelioma (4%) OH invasive localized mesothelioma • Secondary (95%)

• Bronchial carcinoma in males (30%). • Breast Carcinoma in females (30%). • Lymphoma (10%):

• Hodgkin's; • Lymphosarcoma; and • Chronic lymphocytic leukemia.

• Miscellaneous (GIT tumors) (25%): (a) Stomach ; (b) Pancreas; and (c) Colon.

Pleural plaques

Pleural plaques are discrete 1-5 mm areas of thickened parietal pleura of unknown cause The relationship of pleural plaques to the development of mesothelioma is uncertain Primary pleural neoplasms are rare compared to metastatic lesions Lipomas are the most common benign pleural lesions and are usually well encapsulated, free from underlying tissue, and firmly fixed to the pleura Many can be diagnosed and resected by VATS

Mesothelioma

Etiology • Asbestosis exposure is a major risk factor • Asbestos is an inhaled particle 50-100 µm • The particles are only partially engulfed by the pulmonary

macrophages and are not cleared from or metabolized in the lung • Insulation manufacturing is historically the most prominent

exposure • Crocidolite (blue asbestos) is the most harmful form • Cigarette smokers with an asbestos exposure have a higher chance

of developing bronchogenic carcinoma • Smoking is not a risk factor for mesothelioma, although it

contributes to diminishing the patient’s overall condition • Other factors that may contribute to the development of

mesothelioma include COPD, radiation, and zeolite exposure

There is a 20-40 year latent period between exposure and emergence of mesothelioma Intensity of exposure seems more important than the duration

Three separate categories of mesotheliomas: • Benign localized • Malignant localized • Malignant diffuse

Mesotheliomas Benign Localized Asbestos - no; asymptomatic Extra-thoracic - 1/3 Resection Malignant Localized

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Benign • Usually on a stalk, slow-growing and generally asymptomatic • Detected on CXR • Extra-thoracic manifestations are present in about 1/3 of patients

and are strong indicators of benign disease • Pulmonary osteoarthropathy (20%) • Fever • Hyperglycemia • Resection (local) is curative unless incomplete • Lobectomy probably has no benefit over local resection Mesotheliomas, Malignant Localized • 20% of all primary malignant pleural tumors are localized • Often symptomatic with chest pain, cough, dyspnea and fever • Osteoarthropathy almost never occurs • Can be difficult to differentiate from primary chest wall tumors • Wide en bloc excision including lung, chest wall, soft tissue, and

skin is the treatment of choice • When surgical resection is incomplete the prognosis is poor even

with adjunctive therapy (XRT and implanted radioisotopes) Mesotheliomas, Malignant Diffuse • 0.8 - 2.1 cases per million population per year • Hematogenous metastases are seen in 50% • Positive thoracic lymph nodes in 67% • Usually occurs in the 6th to 7th decade of life • 3-5x more common in men • Insidious onset of chest pain and dyspnea in 95%

20% primary malignant pleural tumors Localized, symptomatic Wide en bloc excision Diffuse Malignant 0.8-2.1/million/yr 50% - pure epithelial; 30% - mixed Asbestos - yes; smoking – no Malignant diffuse • Asbestos exposure with

latency period of 20 years • Intensity of exposure more

important than duration. • Usual patient is middle aged

male with pleuritic chest pain/SOB

• CXR shows pleural calcifications

• three main types—epithelial (confused with adenocarcinoma), mesenchymal, and mixed

• median survival 6 to 14 months--most die of local complications

• subtotal pleurectomy—1 year survival—60%

• radical extrapleural pneumonectomy

• adjuvant chemotherapy Clinical Information (1) • Middle aged men presenting with pleuritic chest pain and/or SOB

(50% a clear history of asbestos exposure) • CXR with pleural effusion and pleural thickening often associated

with calcifications • A CT scan is obtained to define the extent of disease and anatomy • A large biopsy - not just fluid - is needed (open or VATS biopsy)

Butchart Staging System Stage Definition I Within the capsule of the

parietal pleura: ipsilateral pleura, lung, pericardium, diaphragm

II Invading the chest wall or mediastinum: esophagus, heart, opposite pleura, positive lymph node within the chest

III Through the diaphragm to peritoneum; opposite pleura, positive lymph node outside the chest

IV Distant blood-borne metastases

Brigham and Women’s Hospital/DFCI Staging System

Stage Definition I Disease confined to

capsule of the parietal pleura: ipsilateral pleura, lung, pericardium, diaphragm, or chest wall disease limited to prior biopsy site

II All of Stage I with positive intrathoracic (N1 or N2) lymph nodes

III Local extension of disease

Clinical Information (2) Differentiate between adenocarcinoma and mesothelioma: • Keratin [+] in mesothelioma but adenocarcinoma will be [-] (except

in breast CA) • Hyaluronic acid is present in the pleural effusion of a mesothelioma • Both tumors stain positive for periodic acid-Schiff stain but after

enzymatic digestion of diastase, mesotheliomas become negative and adenocarcinomas remain positive

• Electron microscopy demonstrates abundant long, slender microvilli and prominent desmosomes in a mesothelioma, whereas short, blunt microvilli are found in adenocarcinoma

• The pure epithelial variety comprises 50% of all malignant mesotheliomas; 30-34% are mixed and the rest (16-20%) mesenchymal

• Most pathologists confidently diagnose mesothelioma only in patients with the sarcomatous or mixed histology

MPM Adeno-carcinoma Histology Mucicarmine stain Negative Positive Periodic acid Schiff (PAS) Negative Positive

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stain Immunostaining CEA Negative Positive (70%) Cytokeratin Positive Negative LeuM-1 Negative Positive Vimentin Positive Negative Calretinin Positive Negative Electron Microscopy Long microvilli Short Microvilli

into chest wall or mediastinum; heart, or through diaphragm, peritoneum; with or without extrathoracic or contralateral (N3) lymph node involvement

IV Distant metastatic disease

Malignant Mesotheliomas • Stage one denotes limited disease, while stages II-IV denote

extensive or metastatic disease. • Stages I, II and III disease have a mean survival of 16, 9 and 5

months • Multiple Variations of Staging and TNM Classification • Most commonly used is Butchart Staging System and the TNM

system of the International Mesothelioma Interest Group (IMIG)

International Mesothelioma Interest Gropu (IMIG) TNM Classification Primary Tumor (T) T1 Tumor limited to ipsilateral parietal and/or visceral pleura T1a (-) visceral pleura T1b (+) visceral pleura T2 Tumor invades any of the following: ipsilateral lung, endothoracic fascia, diaphragm, or pericardium T3 Tumor invades any of the following: ipsilateral chest wall muscle, ribs, or mediastinal organs or tissues T4 Tumor directly extends to any of the following: contralateral pleura, lung, peritoneum, intra-abdominal organs, or cervical tissues (Yang et al., Current Therapy in Thorac and Cardiovasc Surg, 2004) IMIG TNM Classification Regional Lymph Nodes (N) NX Regional lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Metastasis in ipsilateral bronchopulmonary or hilar lymph nodes, including direct extension N2 Metastasis in ipsilateral mediastinal and/or subcarinal lymph node(s) N3 Metastasis in contralateral mediastinal, contralateral hilar nodes or any supraclavicular lymphp node (Yang et al., Current Therapy in Thorac and Cardiovasc Surg, 2004) Distant Metastasis (M) MX Distant metastasis cannot be assessed M0 No evidence of distant metastasis M1 Distant metastasis (Yang et al., Current Therapy in Thorac and Cardiovasc Surg, 2004) IMIG Staging System Stage I T1a

T1b N0 N0

M0 M0

Stage II T2 N0 M0 Stage III Any T3

Any N1

M0 M0

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Any N2 M0 Stage IV Any T4

Any Any

N3

M1

Management Standard Initial Evaluation • Review of pathology to confirm tissue diagnosis (including immunohistochemistry ± electron microscopy) • CT scan of chest and upper abdomen through the adrenals with intravenous contrast • Whole-body FDG PET scan • Pulmonary function tests, including diffusion capacity • Quantitative ventilation/perfusion lung scanning • • Radionuclide stress test •

Resectability, tumor histology, and nodal status Stage I: Resectable tumors without lymph node involvement.

Stage II: Resectable tumors with lymph node involvement.

Stage III: Tumors that have been rendered unresectable as a result of tumor

invading the mediastinum or through the diaphragm Stage IV: Tumors associated with extrathoracic metastasis.

Because any type of planned surgery would be cytoreductive rather than radical , an optimal outcome via surgery alone is unlikely. Accordingly, the current strategy for curing this disease has shifted to multimodal therapy with chemotherapy and/or radiation therapy (RT). There are two types of surgery for MPM. Extrapleural pneumonectomy (EPP) involves en bloc resection of the lung, pleura, pericardium, and diaphragm. Pleurectomy/ decortication (P/D) is a lung-sparing surgery that removes only parietal/visceral pleura. EPP leaves less residual tumor cells compared with P/D; however, it often results in high mortality/morbidity, severe depression of cardiore- spiratory function, and 5-yr survival. It should be offered to very selected patients. Chemotherapy - unclear; some intrapleural therapies may play a role.

• Supportive care • Debulking with subtotal

pleurectomy • Radical surgery • XRT, chemotherapy Emerging Treatments 1. EPP and Intraoperative Hyperthermic Cisplatin Lavage (Heated Chemotherapy) 2. Antiangiogenic Therapy 3. Photodynamic therapy (PDT) 4. Immunotherapy 5. Gene Therapy

poor quality of life. Till date, the risk–benefit ratios of P/D and EPP as part of multimodal therapy have not been clearly elucidated. Furthermore, the decision to perform either EPP or P/D in studies on multimodal approaches has been solely based on surgical conjecture and bias, rather than scientific data. Emerging Treatments è

Results of Extrapleural Pneumonectomy for Malignant Mesothelioma Author Year # pts Operative Mortality

% 2-Year Survival %

Butchart 1976 29 31 10 DeValle 1986 33 9 24 Vogt Moykopf 1987 55 5.5 16 Sugarbaker 1999 183 3.8 38 Rusch 1999 115 5.2 35 Pearson et al. Thoracic Surgery Second Edition, 2002

Mesothelioma, Prognosis Prognosis is poor with a mean survival of 6-14 months. Patients usually die of local complications rather than distant metastases

Results of Surgical Resection for Mesothelioma Study Year #

pts. Type of Resection

% of pts. Surviving 1 Yr 2 Yr 5 Yr

Worn Butchart et al. Wanebo et al. DeLaria et al. Antman

1974 1976 1976 1978 1980

186 62 29 33 11 10

Radical Pleurectomy Radical Pleurectomy Radical Pleurectomy

75 68 30 NS 36 70

34 37 10 30 27 30

9 10 3 15 0 10

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FIGURE4−32. Algorithm for the management of MPM. Modified and reproduced from Butchart,E.G., et al. Pleuropneumonectomy in the management of diffuse malignant mesothelioma of the pleura. Experience with 29 patients. Thorax 1976;31:15-24.

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Operations for The Pleura

Decortication

Thoracoplasty

Thoracoplasty for TB-historic approaches attempted to copy nature’s intentions, i.e., resection of the chest wall to collapse the underlying diseased lung. Types of Thoracoplasty 1. Conventional (extraperiosteal paravertebral) thoracoplasty Bernou-Frachoud (Alexander) from above downward, one or two stages. 2. Sawamura modified thoracoplasty. 3. Plombage thoracoplasty. 4. Shede thoracoplasty and its modifications. 5. Bjork osteoplastic thoracoplasty Conventional (extraperiosteal paravertebral) thoracoplasty Bernou-Frachoud (Alexander) • From above downward, one or two stages. • Most successful was 3 stage approach by alexander • 75% sputum conversion • Multiple pulmonary complications • “Frozen shoulder” • Cosmetically unacceptable results Shede thoracoplasty and its modifications. Plombage-introduced in the 1950’s Various materials placed extrapleurally between the lung and ribs or extraperiostally did not interfere with cough or chest wall movement complicated by infection. 60% conversion rate. Osteoplastic thoracoplasty posterior ends of the upper ribs are resected in increasing lengths back to the tip of the transverse processes which are L. intact ribs are reflected down posterior ends are wired to the uppermost intact rib chest wall stability. Bjork osteoplastic thoracoplasty Muscle transposition into chronic empyema cavity Can also be done as a free flap

Types of Thoracoplasty 1. Conventional (extraperiosteal paravertebral) thoracoplasty Bernou-Frachoud (Alexander) 2. Sawamura modified thoracoplasty. 3. Plombage thoracoplasty. 4. Shede thoracoplasty and its modifications. 5. Bjork osteoplastic thoracoplasty

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Spring 2016 5. Tracheal Diseases

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5 Tracheal Diseases

Table of Contents

5 Tracheal Diseases .................................... 2 Anatomical Considerations .................................... 4

The trachea ........................................................ 4 Anatomic relationships ....................................... 4 Arterial supply ..................................................... 4

Congenital Tracheal Anomalies ............................. 4 Agenesis or atresia ............................................. 4 Tracheoesophageal fistula ................................. 4 Congenital stenosis ............................................ 4

Tracheal Neoplasms .............................................. 5

Primary neoplasms of the trachea ..................... 5 Secondary tracheal neoplasms .......................... 5

Infections and Inflammations ................................. 6 Traumatic Injuries .................................................. 7

Penetrating injuries ............................................ 7 Blunt injuries ....................................................... 7 Postintubation Injuries ........................................ 7

Surgery of Trachea ................................................ 8 Resection and Reconstruction ........................... 8 Tracheostomy .................................................... 9

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Chapter Objective

At the end of this chapter the resident understands the anatomic, embryologic, physiologic and the pathogenesis and management of tracheal diseases

Learner Objectives

Upon completion of this chapter the resident: 1. Understands the anatomical and radiological compartments, as

well as the the lymphatic drainage; 2. Understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for congenital malformations of the trachea; 3. Understands the pathogenesis and management of the tracheal

infections and inflammation; 4. Understands the pathogenesis and management of the tracheal

tracheal injuries; 5. Knows the pathogenesis and management of the tracheal

neoplasms; 6. Knows the indications, contraindications, techniques and results of

tracheal surgery (resection and reconstruction, and tracheotomy).

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Anatomical Considerations

The trachea

· Average length is 11 cm (range 10-13 cm) and shortens with age · 18-22 rings (about 2 rings per cm) · The cricoid cartilage is the only complete ring · Average diameter is 2.3 cm laterally and 1.8 cm anteroposteriorly · In infants, the anterioposterior diameter is greater · COPD will increase the AP diameter ("saber sheath")

Anatomic relationships

· Anterior - thyroid isthmus, innominate artery, aortic arch · Posterior - esophagus · Lateral - azygous, pleura, recurrent laryngeal nerves · Inferior - anterior and posterior subcarinal lymph nodes

Arterial supply

· Three branches of the inferior thyroid artery on either side of the upper trachea · Subclavian, supreme intercostal, internal thoracic, innominate, superior and middle bronchial arteries all contribute to varying degrees · Lateral longitudinal vessel anastomoses feed transverse vessels, which penetrate between cartilage rings to supply submucosa · Mobilize only 1-2 cm circumferentially to avoid ischemic necrosis · Mobilize anteriorly and posteriorly to avoid significant vessels

Congenital Tracheal Anomalies

Agenesis or atresia

· Usually fatal at birth

Tracheoesophageal fistula

· Tracheal lesion easily closed, esophageal portion more difficult · Rarely has associated stenosis

Congenital stenosis

· Webs and diaphragms most often occur at subcricoid level · Three types of stenosis: generalized hypoplasia, funnel-like narrowing, and segmental stenosis · Main bronchi most often normal with generalized hypoplasia, vary in size with funnel-like narrowing, and commonly have anomalies with segmental stenosis · Associated with a wide range of anomalies throughout the body · About 50% of pulmonary sling cases are associated with lower tracheal stenosis

Diagnosis of congenital stenosis · Suspicion in infants with inspiratory and expiratory stridor or more severe respiratory distress · Wheezing, retraction, poor feeding, failure to thrive may also be present

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Management · Operations in infants are high risk, as postoperative edema may obstruct small airways and mechanical ventilation may disrupt anastomoses · A conservative approach is wise, as less than 1/3 of the trachea in a child can be resected · Webs can be dilated or excised endoscopically · Stenoses should not be dilated, as this can create longitudinal tears, resulting in recurrent scarring and stricture · Tracheostomy for palliation is a suitable alternative to allow growth for surgical repair

Tracheal Neoplasms

Primary neoplasms of the trachea

Primary neoplasms of the trachea are rare Squamous cell carcinoma · Most common tracheal neoplasm · Well localized exophytic or ulcerated lesion · Multiple lesions or a superficially infiltrating variety also occur · 1/3 of patients will present with mediastinal or pulmonary metastasis · Spread is usually to regional tracheal lymph nodes and then directly into mediastinal structures

Maziak DE, Todd TRJ, Keshavjee SH, et al. Adenoid cystic carcinoma of the airway: thirty-two-year experience. J Thorac Cardiovasc Surg 1996;112:1522-1532.

Refaely Y and Weissberg D. Surgical management of tracheal tumors. Ann Thorac Surg 1997;64:1429-1432.

Tsai JY, Berkery L, Wesson DE, Redo SF, Spigland NA. Esophageal atresia and tracheoesophageal fistula: surgical experience over two decades. Ann Thorac Surg 1997;64:778-783.

Adenoid cystic carcinoma (cylindroma) · Grows slowly and displaces mediastinal structures rather than invading · Submucosal and perineural spread may occur for quite long distances · Best chance at cure is complete resection at first operation Clinical Features · Dyspnea on exertion · Wheezing · Stridor · Excessive secretions · Hemoptysis · Pneumonia · Cough

Secondary tracheal neoplasms

· Thyroid cancers are the most common indication for tracheal operations for secondary malignancy · Laryngeal carcinoma involves the upper trachea by direct extension · Bronchogenic carcinoma more commonly involves the carina or bronchial origin on the R. · Tracheoesophageal fistula can result from radiation therapy for or from invasion by esophageal cancer · Other tumor include carcinoid, head and neck, breast, and lymphoma

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FIGURE 5−5. Algorithm for the mangement of tracheal neoplasms.

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CHEST WALL, LUNG, M

EDIASTINUM, AND PLEURA

CHAPTER 19

type of the cancer. Factors associated with improved long-term survival include complete resection and use of radiation as adjuvant therapy in the setting of incomplete resection.8 Due to their radiosensitivity, radiotherapy is frequently given postop-eratively after resection of both adenoid cystic carcinomas and squamous cell carcinomas.9 A dose of 50 Gy or greater is usual. Nodal positivity does not seem to be associated with worse sur-vival. Survival at 5 and 10 years is much better for adenoid cystic (73% and 57%, respectively) than for tracheal cancers (47% and 36%, respectively; P < .05). For patients with unre-sectable tumors, radiation may be given as the primary therapy to improve local control, but is rarely curative. For recurrent airway compromise, stenting or laser therapies should be con-sidered part of the treatment algorithm.

LUNGAnatomySegmental Anatomy. The segmental bronchial and vascular anatomy of the lungs allows subsegmental and segmental resec-tions, if the clinical situation requires it or if lung tissue can be preserved10 (Fig. 19-7). Note the continuity of the pulmonary parenchyma between adjacent segments of each lobe.

Lymphatic Drainage. Lymph nodes that drain the lungs are divided into two groups according to the tumor-node-metastasis (TNM) staging system for lung cancer: the pulmonary lymph nodes (N1) and the mediastinal nodes (N2) (Fig. 19-8).

The N1 lymph nodes constitute the following: (a) intra-pulmonary or segmental nodes that lie at points of division

High-index of suspicion(cough, dyspnea, hemoptysis,

stridor and hoarseness)

Complete staging: computedtomography/PET

scan/mediastinoscopy

Flexible/rigid bronchoscopy

Tumor resectable

Performance statusadequate for surgery

Debridement and/orlaser ablation

Principles of tracheal resection

May resect up to 50% of tracheal lengthAnterolateral mobilization onlySuture head in forward flexion for 7 daysLaryngeal and hilar release as needed

for relief of tension

Radiotherapy 50 Gy (±chemotherapy)(primary treatment or postoperatively)

Tumor unresectable

1) Probable grossly positive tracheal resection margin2) Metastatic disease3) Length of resection precludes safe reconstruction4) Invasion of unresectable adjacent organs

Poor performancestatus

Figure 19-6. Algorithm for eval-uation and treatment of tracheal neoplasm. PET = positron emis-sion tomography.

of segmental bronchi or in the bifurcations of the pulmonary artery; (b) lobar nodes that lie along the upper, middle, and lower lobe bronchi; (c) interlobar nodes located in the angles formed by the main bronchi bifurcating into the lobar bron-chi; and (d) hilar nodes along the main bronchi. The interlobar lymph nodes lie in the depths of the interlobar fissure on each side and constitute a lymphatic sump for each lung, referred to as the lymphatic sump of Borrie; all of the pulmonary lobes of the corresponding lung drain into this group of nodes (Fig. 19-9). On the right, the nodes of the lymphatic sump lie around the bronchus intermedius (bounded above by the right upper lobe bronchus and below by the middle lobe and superior seg-mental bronchi). On the left, the lymphatic sump is confined to the interlobar fissure, with the lymph nodes in the angle between the lingular and lower lobe bronchi and in apposition to the pul-monary artery branches.

The N2 lymph nodes consist of four main groups. (a) The anterior mediastinal nodes are located in association with the upper surface of the pericardium, the phrenic nerves, the liga-mentum arteriosum, and the left innominate vein. (b) The pos-terior mediastinal group includes paraesophageal lymph nodes within the inferior pulmonary ligament and, more superiorly, between the esophagus and trachea near the arch of the azy-gos vein. (c) The tracheobronchial lymph nodes are made up of three subgroups that are located near the bifurcation of the trachea. These include the subcarinal nodes, which lie in the obtuse angle between the trachea and each main stem bronchus, and the nodes that lay anterior to the lower end of the trachea. (d) Paratracheal lymph nodes are located in proximity to the

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Infections and Inflammations · Tuberculosis may cause strictures of the lower trachea and bronchi · Strictures are submucosal and the cartilage has a grossly normal caliber · Fibrosing mediastinitis and histoplasmosis may be so extensive as to preclude reconstruction · Idiopathic strictures most often occur in the upper trachea · Other causes include Wegener's granulomatosis, amyloid, sarcoid, relapsing polychondritis, and tracheopathia osteoplastica

Traumatic Injuries

Penetrating injuries

· Usually involve the cervical trachea · Fresh injuries can be closed primarily

Blunt injuries

· Closed trauma may lacerate or sever the trachea · A severed trachea should be re-approximated if possible · The distal trachea should be intubated for security · A tracheostomy tube should be inserted in the distal severed end for complex injuries and repair undertaken later · A vertical split at carina is usually diagnosed by a pneumothorax that fails to resolve with closed suction · Bronchoscopy, thoracotomy, and occasionally cardiopulmonary bypass are required to repair this lesion · Traumatic TEF should be promptly repaired, as inflammation makes repair difficult when the diagnosis is delayed · Always establish the function of the recurrent laryngeal nerves and larynx prior to surgery, if possible

Postintubation Injuries

These are currently the most frequent cause of tracheal stenosis A. Laryngeal level · Sealing cuffs may irritate vocal cords, causing granulomas and fusion of the posterior commissure · Mucosal erosion at the cricoid can cause subglottic stenosis · Most lesions are reversible with time

Cassada DC, Munyikwa MP, Moniz MP, Dieter RA, Schuchmann GF, Enderson BL. Acute injuries of the trachea and major bronchi: importance of early diagnosis. Ann Thorac Surg 2000;69:1563-1567.

Kelly JP, Webb WR, Moulder PV, et al. Management of airway trauma. I: Tracheobronchial injuries. Ann Thorac Surg 1985;40(6):551-5.

Kelly JP, Webb WR, Moulder PV, et al. Management of airway trauma. II: Combined injuries of the trachea and esophagus. Ann Thorac Surg 1987;43(2):160-3.

B. Stomal level · Principle factors include a stoma that is too large, loss of tissue from infection (rare), and pressure erosion from rigid connecting systems (most common) · Avoid injuring the first cartilaginous ring during original tracheostomy to prevent subglottic stenosis · Avoid low placement of the tracheostomy to prevent innominate artery erosion and supracarinal stenosis C. Sealing cuff level · Most common cause is direct pressure necrosis by high-pressure cuff · This has essentially disappeared with use of low-pressure, high-

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volume cuffs · Children may develop granuloma on anterior tracheal wall when ventilated without a cuff D. Tracheomalacia · Usually occurs in segment of trachea between stoma and the cuff · Secretions pool here and inflammation leads to cartilage thinning

E. Tracheoesophageal fistula · Manifested by sudden appearance of secretions in tracheobronchial tree · Pneumonitis, pneumonia, abscess, and gastric dilitation may develop · Methylene blue in tube feedings will appear promptly in the trachea

F. Trachoinnominate fistula · Manifested by sudden exsanguinating hemorrhage into the tracheobronchial tree · Emergent tamponade with high-pressure cuff or digital pressure is necessary

Diagnosis · CXR · Fluoroscopy determines laryngeal function and presence of tracheomalacia · CT to evaluate extramural extension of tumors · Bronchoscopy and biopsy at the time of operation

Surgery of Trachea

Resection and Reconstruction

· All patients should be induced slowly and gently in the OR · Extubation in the OR is desired in order to avoid intubation injury to the suture line

Donahue DM, Grillo HC, Wain JC, WR. CD, Mathisen DJ. Reoperative tracheal resection and reconstruction for unsuccessful repair of postintubation stenosis. J Thorac Cardiovasc Surg 1997;114:934-939.

Grillo HC, WR. CD, Vlahakes GJ, MacGillivray TE. Management of congenital tracheal stenosis by means of slide tracheoplasty or resection and reconstruction, with long-term follow-up of growth after slide tracheoplasty. J Thorac Cardiovasc Surg 2002;123:145-152.

Grillo HC, Zannini P, Michelassi F. Complications of tracheal reconstruction. Incidence, treatment and prevention. J Thorac Cardiovasc Surg 1986;91(3):322-8.

Jacobs JP, Quintessenza JA, Andrews T, et al. Tracheal allograft reconstruction: the total North American and worldwide pediatric experiences. Ann Thorac Surg 1999;68:1043-1051.

Mansour KA, Lee RB, Miller JI Jr. Tracheal resections: Lessons learned. Ann Thorac Surg 1994;57(5):1120-5.

A. Upper half of trachea · Collar incision with partial upper sternal split, if necessary, is used for benign and malignant lesions of the upper trachea · Almost all stenoses above the carina can be reached through this approach as well · Dissect anteriorly from the cricoid cartilage to the carina, staying close to the trachea · The recurrent laryngeal nerves must be identified · Divide the trachea and intubate distally · About 4.5 cm can be removed without additional mobilization · Laryngeal release is preferred, if needed, taking care to avoid injury to the superior laryngeal nerves · Hilar release is more hazardous and is contraindicated in patients with poor pulmonary function · Once the sutures have been placed, the distal tube is removed and the endotracheal tube is readvanced past the anastomosis · All sutures are tied and the anastomosis tested under water for leakage B. Lower half of trachea · Posterolateral thoracotomy in 4th interspace provides optimal exposure and should be performed on the side contralateral to the aortic arch (usually R.) · About 4.5 to 5 cm can be resected without extreme maneuvers · Release technique include hilar dissection, loosening of the carina,

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and cervical flexion · Laryngeal release does not provide any additional mobilization · A pleural or pericardial flap is constructed to protect the anastomosis

Tracheostomy

· Principal indications include upper airway obstruction, management of secretions, prolonged ventilation, and emergent airway · Second and third rings are opened vertically · Avoid high and low stoma placement to prevent subglottic stenosis and innominate artery erosion · Persistent stoma 3 to 6 months after tracheostomy should be closed surgically

Sources for further reading

Textbook Chapters Chapter 12: Congenital Lesions,

Neoplasms, and Injuries of the Trachea. Surgery of the Chest (Sabiston and Spencer), 5th ed., 335-71.

Chapter 38: The Trachea: Tracheostomy, Tumors, Strictures, Tracheomalacia, and Tracheal Resection and Reconstruction. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 665-690.

Results · Small numbers of patients make accurate predictions of survival difficult · Resection of benign tumors and low-grade malignant tumors appears to have high probablity of cure · Single-stage resection and reconstruction is probably the best approach for squamous cell and adenoid cystic carcinoma · Adjunctive radiation is usually indicated for squamous cell and adenoid cystic tumors · Selected patients can be treated effectively with resection and reconstruction for secondary tracheal tumors

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6 The Lungs

Table of Contents

6 The Lungs ................................................. 1 Anatomy 0f The Lungs .......................................... 2

Developmental Anatomy of The Lungs .............. 3 Pulmonary Physiology ........................................... 9

Lung physiology ................................................. 9 Pulmonary Diagnostic Procedures ................... 12 Pulmonary Function Testing ............................. 12 Scalene Node Biopsy ....................................... 16 Mediastinoscopy ............................................... 16 Bronchoscopy ................................................... 16

Congenital Lesions .............................................. 18 Agenesis, Aplasia, and Hypoplasia .................. 18 Congenital Lobar Emphysema (CLE) ............... 18 Pulmonary Cysts .............................................. 19 Cystic Adenomatoid Malformation .................... 19 Bronchogenic Cysts ......................................... 20 Intralobar Pulmonary Sequestration (IPS) ........ 21 Extraobar Pulmonary Sequestration (EPS) ...... 21

Bullous & Bleb Diseases ..................................... 23 Bullous Emphysema ......................................... 23

Infections of the Lung .......................................... 26 Lung Abscess ................................................... 26

Bronchiectasis .................................................. 27 Pulmonary Tuberculosis & Fungus ..................... 27

Pulmonary Tuberculosis ................................... 27 Fungal infections of the lung ............................ 28 Histoplasmosis ................................................. 29 Coccidiomycosis .............................................. 29 Blastomycosis .................................................. 30 Other Fungal and Opportunistic Infections of the Lung ................................................................. 30

Lung Cancer ........................................................ 32 Bronchial Adenomas ........................................... 36

Carcinoid Tumors ............................................. 36 Atypical carcinoid ............................................. 37

Lung Cancer Staging ........................................... 37 Oncogenes in Lung Cancer ............................. 41 Sugery for Lung Cancer ................................... 42

Supeior Sulcus Tumors ....................................... 46 Solitary Pulmonary nodule ................................... 49 Pulmonary Metastases (Mets) ............................. 49 Benign Respiratory Tract Tumors ........................ 50

Benign Tumors of the Lung .............................. 54 Benign Pulmonary Disease .............................. 57

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Chapter Objective

At the completion of this chapter the resident:

• understands the embryology and anatomy of the lungs and their relationship to adjacent structures, the physiology of airway mechanics, gas exchange, and blood flow, and applies the findings of invasive and non-invasive tests to patient management.

• understands the natural history, types, evaluation, and management of pulmonary neoplasms, and performs operative and non-operative treatment.

• understands infectious, inflammatory, and environmental injuries of the lungs and performs operative and non-operative management.

Learner Objectives Upon completion of this chapter the resident:

1. Understand the segmental anatomy and developmental anatomy of the lungs;

2. Knows the pulmonary physiology, and how to interpret pulmonary function tests , and the tolerability of resections;

3. Knows the indications, contraindications and complications of different pulmonary diagnostic procedures

4. Understands the pathogenesis and the management of congenital lesions of the lungs;

5. Understands the pathogenesis and the management of bullous & bleb diseases

6. Understands the pathogenesis and the management of infections of the lungs

7. Understands the pathogenesis and the management of pulmonary TB & fungal pulmonary infections

8. Understands the pathogenesis and the management of lung cancer;

9. Understands TNM staging of lung carcinoma and its application to the diagnosis, therapeutic planning, and management of patients with lung carcinoma and the role of adjuvant therapy for lung neoplasms;

10. Understands the pathogenesis and the management of solitary pulmonary nodule (SPN).

11. Understands the pathogenesis and the management of superior sulcus tumors (SST).

12. Understands the indications for resection of pulmonary metastases (mets).

13. Understands the indications for resection of benign lung neoplasms;

14. Knows iindications, contraindications, and complications of the operations of the lungs.

Anatomy 0f The Lungs

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Developmental Anatomy of The Lungs

A. Intrauterine stage • 4 periods of development: embryonic, pseudoglandular,

canalicular, and terminal sac • Embryonic period begins at 26 days with ventral protrusion of the

foregut and ends at 32 days with appearance of 5 lobar bronchi • Pseudoglandular period lasts from 5th to 16th weeks and is

characterized by rapid branching and formation of all conducting airways

• Canalicular period lasts from 16th to 25th weeks and is characterized by capillary ingrowth and appearance of saccules

• Terminal sac or alveolar period begins at 25 weeks, and alveolar development begins between the 30th and 36th weeks

• Type I and II epithelial differentation typically occurs at 28 weeks

B. Neonatal stage • Precursors of typical acinar unit are present at birth: bronchioles,

transitional ducts, and terminal saccule • Alveolar development continues after birth with remodeling and

multiplication • The total adult number of alveoli are not reached until age 8 • Alveolar enlargement continues until adulthood, although no new

alveoli are added

Mnemonic: Lung development phases "Every Premature Child Takes Air": E−Embryonic period P−Pseudoglandular period C−Canalicular peroid T−Terminal sac period A−Alveolar period Anatomic Variants with Normal Parenchyma Superior segment of lower lobe delineated by separate fissure Medial accessory L. lower lobe Azygous lobe - mesentery of azygous vein forms double fold of visceral pleura that isolates part of R. upper lobe Bilateral bilobation or trilobation Situs inversus thoracis or totalis - totalis form associated with Kartagener's syndrome (situs inversus, bronchiectasis, pansinusitis immotile cilia syndrome).

C. Histology • The mature lung is characterized by closely packed alveoli divided by thin

septa, occupied by capillaries • Capillary endothelium is typically a single cell layer with few organelles and

thin cytoplasmic matrix • Over 95% of alveolar epithelium is type I cells, which are also very thin

with few organelles • Type II alveolar cells are cuboidal and secrete surfactant • Surfactant synthesis peaks at term and decreases to adult levels shortly

thereafter • An increase in the lecithin-sphingomyelin ratio to more than 2:1 occurs just

before birth • Type II cells can be stimulated to produce this pattern of phospholipid by

steroids, thyroxine, estrogens, beta-agonists, and increases in ventilation or tidal volume

• Surfactant stabilizes the alveoli, lowers surface tension to keep alveoli open at low volumes, prevents alveolar wall adhesion, and helps maintain pulmonary compliance

Congenital anomalies of the trachea and lungs

Bronchopulmonary (Lung bud) Anomalies

Combined Lung & Vascular Anomalies

Vascilar Anomalies

A. Congenital bronchial atresia B. Congenital lobar emphysema

(CLE) C. Congenital cystic adenomatoid

malformations (CPAM) D. Pulmonary bronchogenic cysts

(PBFM) E. Tracheal bronchus F. Accessory cardiac bronchus G. Tracheomalacia H. Tracheal stenosis I. Pulmonary hypoplasia

A. Hypogenic lung ( Simitar syndrome)

B. Bronchopulmonary sequestration (IPS, EPS)

A. Absence of a main pulmonary artery

B. Anomalous origin of LPA from R C. APVC (TAPVC. PAPVC) D. PAVFs

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The intrauterine development of airways.

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Segmental Anatomy of The Bronchopulmonary Segments

Arterial, Venous And Bronchial Anatomy of The Lungs

The Lymphatic Anatomy of The Lungs,

The Major Lymphatic Nodal Stations, And Lymphatic Drainage Routes of The Lung Segments

• RUL - lower paratracheal higher paratracheal neck • RML - subcarinal R. paratracheal • RLL - subcarinal R. paratracheal • LUL - subaortic (Botallo's node) Anterior mediastinal or L.

paratracheal • LLL - subcarinal

II- clinical findings often do not correlate with anatomical predictions

Textbook Chapters Chapters 21-7: Lung Carcinomas, Diagnosis and Staging of Lung Cancer, Surgical Treatment of Lung Carcinoma, Superior Sulcus Tumors, Limited Pulmonary Resection, Bronchoplastic Techniques for Lung Resection, Multimodality Therapy of Carcinoma of the Lung: Irradiation, Chemotherapy, and Immunotherapy. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 357-498.

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Pulmonary Resections

A. R. upper lobectomy 1. Incise hilar pleura 2. Visualize pulmonary vein 3. Pulmonary artery lies superior and posterior to the vein 4. Dissection is carried out to the anterior-apical artery 5. Artery and vein are dissected circumferentially being careful of the takeoff of the middle lobe artery 6. Upper lobe bronchus is identified just inferior to the azygos

B. R. middle lobectomy 1. Identify the posterior branch to the RUL & superior segment branch to the RLL through the major fissure 2. Identify two arterial branches to the middle lobe 3. Middle lobe vein most commonly drains to the superior vein 4. Key is to divide the vein first, followed by the bronchus, and finally the artery

C. R. lower lobectomy 1. Dissection begun in the major fissure 2. Artery seen and dissected distally 3. Bronchus is visualized after division of the artery and then divided after identifying the middle lobe bronchus 4. Finally the vein is divided

D. R. pneumonectomy 1. Incise the hilar pleura 2. Dissect the pulmonary veins 3. Dissect the pulmonary artery (may need to open the pericardium) 4. Isolate mainstem bronchus 5. Cover the bronchial stump

E. R. sided lymph node dissection 1. Dissect the area bordered by: Inferiorly - azygos vein Superiorly - subclavian vein Posteriorly - trachea Anteriorly - superior vena cava

F. L. upper lobectomy 1. Incise the hilar pleura 2. Pulmonary artery is the most superior structure in the hilum 3. Ligamentum arteriosum can be divided to obtain length 4. Dissect the artery to the superior segment of the lll 5. Divide the fissure to expose the lingular arteries 6. Divide the artery, then the vein , and finally the bronchus

G. L. lower lobectomy 1. Begin in the fissure if complete, otherwise begin at the hilum

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2. Visualize the superior segment artery 3. Divide the posterior aspect of the fissure 4. Dissect the artery onto the basilar trunk 5. Expose the inferior vein in the inferior pulmonary ligament 6. Divide the artery, vein , and bronchus

H. L. pneumonectomy

1. Identify the pulmonary artery, and vein 2. Traction is needed to expose the proximal mainstem bronchus

3. Divide the artery, vein, and bronchus

I. L. lymph node dissection 1. Dissect lateral to the ligamentum, between the aortic arch and the pulmonary artery 2. Dissect superior to the arch 3. Dissect the inferior pulmonary ligament

J. Pointers for the L. side 1. Aortopulmonary window may be difficult to dissect secondary to infiltration of disease, and a very friable, tethered artery 2. Apical- anterior artery is tenuous 3. L. mainstem is difficult to dissect

Special circumstances under which lobectomy by video-assisted thoracic surgery may be preferable CONDITION EXAMPLES Pulmonary compromise Poor FEV1/Dlco, heavy smoking, sleep apnea, recent pneumonia Cardiac dysfunction Congestive heart failure, severe coronary artery disease, recent myocardial

infarction, valvular disease Extrathoracic malignancy Solitary brain metastasis from lung cancer, deep pulmonary metastases requiring

lobectomy Poor physical performance Performance status equivalent to a Zubrod score of 2 or 3, morbid obesity Rheumatologic/orthopedic condition

Spinal disease, severe rheumatoid arthritis, severe kyphosis, lupus erythematosus, osteomyelitis

Advanced age Age >70 years Vascular problems Aneurysm, severe peripheral vascular disease Recent or impending major operation

Urgent abdominal operation, joint replacement requiring use of crutches, need for contralateral thoracotomy

Psychological/neurologic conditions

Substance abuse, poor command following, pain syndromes

Immunosuppression/ impaired wound healing

Recent transplantation, diabetes

Source: from Demmy TL, Nwogu C. Is video-assisted thoracic surgery lobectomy better? Quality of life considerations. Ann Thorac Surg. 2008;85:S719.

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A

B

A. Diagram of normal frontal CXR: 1. Trachea, 2. R. lung apex, 3. Clavicle, 4. Carina, 5. R. main bronchus, 6. RLLRA, 7. RA, 8. R. costophrenic angle, 9.gastric air bubble, 10. L costophrenic angle, 11. LV, 12. Descending aorta, 13. LLLPA, 14. LA, 15. LULPV, 16. aortic arch. B. L. lateral CXR. 1. ascending thoracic aorta, 2. sternum, 3. RV, 4. LV, 5. LA, 6. gastric air bubble, 7. R. hemidiaphragm, 8. L. hemidiaphragm, 9. RUL bronchus, 10. LLL bronchus, 11. Trachea. Mnemonic: How to read the plain CXR “Some Bastard Pinched My Toy Dinosaur” “Some Body Pinched My Toy Dinosaur” S: Soft tissue B: Bones- clavicles, ribs P: Pleura, Pulmonary Parenchyma M: Mediastinum T: Thorax, Tubes, Lines D: Diaphragm Basics ABCDEF: Analysis ABCDEF: AP, PA or other view Body position/rotation Confirm name Date Exposure/quality Films for comparison

Airways and hilar Adenopathy Bones and Breast shadows Cardiac silhouette and Costophrenic angle Diaphragm and Digestive tract Edges of pleura Fields (lung fields)

How to read the plain CXR and the “bumps” which make up the cardiac silhouette.

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Pulmonary Physiology Preoperative evaluation and perioperative care of a patient includes 1. Tissue diagnosis of primary disease and decision if an operative procedure is indicated 2. Assessment of patient’s general condition 3. Preoperative preparation and postoperative care

The Evaluation of pulmonary function includes assessment of cardiac function, the oxygen carrying red cells, the lungs, chest wall and ventilatory muscular function

Lung physiology

1. Well suited for efficient exchange of O2 and CO2 with a large surface area and low perfusion pressure (300 million alveoli) 2. Gas exchange controlled by two pumps- the R. ventricle and the chest cage-diaphragm 3. Elastic recoil of lungs ejects gas and fibrous skeleton maintains airway patency 4. Clinical evaluation of pulmonary function

a. History and physical- exercise tolerance b. CXR, ABG c. Simple spirometry d. Vital capacity (FVC)- total exhaled volume FEV1- forced expiratory volume at one second- indication of flow Restrictive disease- vital capacity, inspiratory and expiratory reserves are diminished- can result from diseases of the lung, pleura, chest cage and muscles -kyphoscoliosis, ARDS, pleural effusions or fibrosis. Funcitonal residual volume is decreased, limited capacity to expand lungs but no difficulty emptying lungs Obstructive Disease- lung elastic recoil decreases, compromising the force of exhalation - most common form in clinical practice usually due to smoking, damaged alveoli can lead to pulmonary HTN unsupported airways leads to airway trapping and atelectasis

Todd TR. The preoperative selection of patients for emphysema surgery. Eur J Cardiothorac Surg 1999 Sep;16 Suppl 1:S51-6.

Prakash UB. Advances in bronchoscopic procedures. Chest. 1999 Nov;116(5):1403-8.

Miller JI Jr. Rigid bronchoscopy. Chest Surg Clin N Am. 1996 May;6(2):161-7.

FEV1 1000-2000 ml adequate for surgery FEV1 ≤800 ml preclude surgical resection

Ventilatory Pump and Work of Breathing

1. Ventilatory pump consist of the thoracic cage and ventilatory muscles 2. The ventilatory pump is a suction pump which expands the chest cage to pull air into the lungs 3. Dyspnea signals that the work required of the ventilatory muscles has reached a level that exceeds the comfortable capacity of the patient 4. Thoracotomy creates a region of non-contractile muscles which lowers tidal volume and increases respiratory rate 5. Several disease processes can cause ventilatory pump failure

a) central depression b) muscle paralysis

Summary of Evaluation of Gas Exchange Function- background facts for assessing pulmonary function are as follows: 1. there is a large reserve in normal individuals 2. condition of the ventilatory muscles depends on the physical state of the patient 3. as lung volume falls, airways in dependent areas of the lung close 4. with aging and smoking, airways close at higher lung

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c) fatigue d) mechanical defects in the thoracic cage-trauma, post-

surgical 6. Failure of ventilatory pump leads to atelectasis and decreased lung compliance 7. Functional residual volume decreases with loss of functional alveoli 8. Post-operative pain control- epidural can help prevent splinting and therefore atelectasis 9. Work capacity of ventilatory muscles are trainable- sedentary patients will poor muscle function as compared to active patients

volumes 5. V/Q mismatch occurs with airway closure 6. V/Q mismatch requires increased alveolar ventilation to maintain the same amount of gas exchange 7. spirometry measures the volumes o flung and the ability to move air 8. PaCO2 is an indicator of adequacy of ventilation 9. PaO2 is an indicator of adequacy of oxygenation

Fluid Exchange and Lung Water Blood circulating through normal lung capillaries at normal rates and pressure causes a net fluid movement from the capillaries into the lung interstitium. The filtered fluid is picked up by the lymphatics and returned to the circulation Management of fluid therapy is critical in post-operative pulmonary resection patients since this fluid balance is disrupted 1. increased filtration post-operatively 2. decreased capillary bed and lymphatic mass 3. increased cardiac output 4. must carefully titrate fluid balance especially in pneumonectomy patients

Ventilation -Perfusion Incoordination effective gas transfer relies on the coordination of ventilation and perfusion 1. ventilation-perfusion mismatch occurs post-operatively 2. V/Q mismatch is the most common form of post-operative hypoxemia 3. usually secondary to the development of atelectasis

Shunt Fraction Determines the fraction of blood ejected by the L. ventricle that has no gas exchange in the lungs 1. patients with a shunt fraction > 0.15 to 0.20 are vulnerable to a low C.O. 2. tissue oxygen delivery falls 3. pulmonary artery catheter should be placed to optimize C.O.

One Lung Anesthesia OR One Lung Ventilation (OLV) 1. procedure of choice for pulmonary resection 2. videothoracoscopy has increased demand 3. unventilated lung is perfused and is a source of an intrapulmonary shunt that can lead to hypoxemia 4. usually ventilated on 100 % oxygen

Indications for OLV Absolute Relative

Mnemonic: BPOW—blood, pus, oxygen (separate mnemonic for this), and water. B lood: you don’t want blood from one lung contaminating the other side. P us: you don’t want pus from one side of one lung contaminating the other side. Oxygen: you don’t want an oxygen leak or loss from one side making it impossible to ventilate the other side.

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• Prevent contamination of healthy lung

• Purulence • Bleeding • Need for separate ventilation

of lungs • Bronchopleural fistula • Unilateral bullae • Tracheobronchial disruption • Bronchoalveolar lavage

(alveolar proteinosis) • Video-assisted thoracic

surgery

• Lung resection • Thoracic aortic aneurysm

surgery • Thoracic spine surgery • Esophageal surgery

This one has another mnemonic: SUB T. S urgical + If the surgeons have sectioned a bronchus and you don’t have the lung isolated, you will lose all your ventilation. U nilateral lung cyst + This rare beastie is just a giant alveolus, which doesn’t contribute any meaningful oxygen exchange, you need to isolate it. B ronchopleural/ cutaneous fistula + The air leaking out of this will impair ventilation. T rauma + A sectioned bronchus from trauma (call it an “amateur surgical sectioning”) can also lead to “lost ventilation. W ater: you don’t want irrigating fluid (used to treat alveolar proteinosis) from one lung to contaminate the other side.

Pneumonectomy lung reduction surgery 1. derived from the observation of chest wall adaptation in lung transplant patients 2. bilateral stapling of peripheral lung tissue to diminish lung volumes 3. reinforced with bovine pericardial strips to prevent leaks 4. improvement in symptoms and FEV1 5. improves diaphragmatic motion

Textbook Chapters Chapter 1: Preoperative Assessment of Pulmonary Function: Quantitative Evaluation of Ventilation and Blood Gas Exchange. Surgery of the Chest (Sabiston and Spencer), 6th ed., 1-21.

Chapter 2: Perioperative Pulmonary Physiology. Surgery of the Chest (Sabiston and Spencer), 6th ed., 22-68.

Pulmonary Assessing Pre-operative Function

1. History and physical examination 2. CXR 3. laboratory data 4. Room air arterial blood gas 5. pulmonary function tests :

a. FEV1- 1000- 2000 ml acceptable b. MVV if > 50 L/min acceptable, if < 28 L/min severely decreased function c. Split-lung function test predicts post-operative FEV1 based on ventilation scan on each lung if post-operative FEV1 > 800 ml then patient will tolerate pneumonectomy e.g. patient with L. lower lung tumor, FEV1 1.72 Liters with split function of 62 % on R. and 38 % on L. would predict a post-operative FEV1 of 1.0 liters for L. pneumonectomy if PCO2 45 then the patient is not a candidate for resection unless a medical regimen d. Improves gas exchange • Cessation of smoking • Bronchodilators

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• Appropriate antibiotics for bronchitis

• Exercise

Pulmonary Diagnostic Procedures Pulmonary Function Testing

Definition Over 100,000 thoracotomies are performed annually in the United States alone, and preoperative assessment of risk is required. Two concepts are key to risk assessment: resectability, which is the amount of lung tissue than can be safely removed without pulmonary insufficiency, and operability, which is the ability of the patient to survive the procedure and any peri-operative complications. Resectability depends on pulmonary reserve and operability depends on comorbid conditions. The main tests for preoperative assessment are arterial oxygenation, spirometry, and diffusion capacity.

Resectability The amount of lung tissue than can be safely removed without pulmonary insufficiency. Depends on pulmonary reserve.

Operability Ability of the patient to survive the procedure and perioperative complications. Depends on Comorbid conditions

Spirometry • Modern spirometry is complex and sophisticated • Affected by height, age, weight, sex, race and posture • Blacks, Polynesians, Asians - lung volumes 10-12% less than

similarly aged whites • Main stays of preoperative assessment are arterial oxygenation,

spirometry, and diffusion capacity • Spirometry and arterial oxygen not predictive of postoperative

complications

Pulmonary Function Testing Pulmonary spirometric studies Pulmonary hemodynamic response testing Exercise testing

Diffusion Capacity • Is more sensitive as a predictor of postoperative complications • DLCO estimates pulmonary capillary surface area, hemoglobin

content, alveolar micro architecture • DLCO is decreased in emphysema, pulmonary hypertension and

interstitial lung disease • DLCO <60% was the best predictor of mortality

Ventilation/Perfusion Scans • Useful in determination of postoperative lung function • Perfusion scans (Tc99 ) are more predictable than ventilation

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scans (Xe133) • Spirometry and lung scans accurately predict postoperative lung

function • A calculated postoperative FEV1 of less than 40% of predicted

was associated with 50% mortality rate • Absolute minimum postoperative FEV1 should be > 800 cc

Pulmonary Hemodynamics Response Testing

• Not popular in North America • Pulmonary artery pressure and resistance determine survival • Pulmonary arterial pressure > 35 mmHg results in decreased

survival (10 fold) • Pulmonary hypertension is a contraindication to lung resection • PVR > 190 dynes associated with 90% mortality • Unfortunately pulmonary function tests do not identify patients

with high PVR

Guidelines for Patient Selections for Resection FVC < 50% of predicted FEV1 < 50% of predicted FEV1 < 2.0 Liters DLCO < 60% of predicted PAP > 35 mm/Hg PVR > 190 dynes patient cooperation) MVV < 50% of predicted (with good

Exercise Testing • Minimal achievement tests • Submaximal effort, not standardized • Maximum exercise tests • Arterial desaturation with exercise • Maximal oxygen consumption (MVO2) • Blood lactate level during exercise

Arterial Blood Gases • The gas partial pressure in liquid is equal to the barometric

pressure times the fractional gas concentration • The solubility of oxygen and carbon dioxide in blood is affected

by hemoglobin and buffers • Lower temperature, an increase in pH, and a fall in blood PCO2

will all shift the HbO2 dissocation curve to the L., increasing the affinity of hemoglobin for oxygen

• Common causes of increased A-a gradient include: hypoventilation, reduced inspired oxygen, R.-to-L. shunting, and V/Q mismatch from atelectasis or airway obstruction

• A PaCO2 greater than 45 indicates a higher risk of morbidity and mortality, but hypoxemia is unreliable as a predictor of poor outcome

PaO2 Saturation 100

(arterial) 97%

50 84% 40

(venous) 75%

3 points of the curve to remember: · CO2 is usually transported as HCO3-, but can combine with hemoglobin and carbonic anhydrase · Venous PCO2 is about 46;arterial and alveolar PCO2 is about 40 · Normal alveolar-arterial (A-a) PO2 gradient is 10 in young adults and up to 20 in older adults Calculate A-a gradient as follows: PAO2 = 150-PaCO2, then subtract the PaO2

Spirometry • Modern spirometry is complex, sophisticated, and is affected by

height, age, weight, sex, race and posture • Important values include VT, FEV1, FVC, and FEV1/FVC • In restrictive disease, the total lung capacity, vital capacity, and

FEV1 are all reduced, but the FEV1/FVC is normal • In obstructive diseases like emphysema, the total lung capacity

and vital capacity are increased, and the FEV1 and FEV1/FVC

Rao V, Todd TR, Kuus A, Buth KJ, Pearson FG. Exercise oximetry versus spirometry in the assessment of risk prior to lung resection. Annals of Thoracic Surgery 1995 60(3):603-9.

1st International Symposium on Thoracosopic Surgery. Annals of Thoracic Surgery 1993 56(3):683-806.

Weisman IM. Cardiopulmonary

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are reduced • Blacks, Polynesians, and Asians have lung volumes 10-12% less

than similarly aged whites • An FEV1 less than 2.0 liters indicates a higher risk of morbidity

and mortality

exercise testing in the preoperative assessment for lung resection surgery. Semin Thorac Cardiovasc Surg. 2001 Apr;13(2):116-25.

Melendez JA, Fischer ME. Preoperative pulmonary evaluation of the thoracic surgical patient. Chest Surg Clin N Am. 1997 Nov;7(4):641-54.

Diffusion Capacity • Is more sensitive than spirometry as a predictor of

postoperative complications • DLCO estimates pulmonary capillary surface area, hemoglobin

content, and alveolar microarchitecture • The test is particularly useful in patients with dyspnea and

relatively normal spirometry • DLCO is decreased in emphysema, pulmonary hypertension and

interstitial lung disease; it is increased in mitral stenosis, L.-sided failure, and polycythemia

• A DLCO less than 60% is a good predictor of mortality

Overend TJ, Anderson CM, Lucy SD, Bhatia C, Jonsson BI, Timmermans C. The effect of incentive spirometry on postoperative pulmonary complications: a systematic review. Chest. 2001 Sep;120(3):971-8.

Culver BH. Preoperative assessment of the thoracic surgery patient: pulmonary function testing. Semin Thorac Cardiovasc Surg. 2001 Apr;13(2):92-104.

Ferguson MK. Preoperative assessment of pulmonary risk. Chest. 1999 May;115(5 Suppl):58S-63S.

Ventilation/Perfusion Scans • Blood flow (perfusion) is absent in pulmonary vascular

obstruction and ventilation is absent in atelectasis • The perfusion portion (Tc99) is more predictable than the

ventilation portion (Xe133) • When used together with spirometry, lung scans can accurately

predict postoperative lung function

Pulmonary Hemodynamics Response Testing • Pulmonary artery pressure and resistance are determinants of

survival • A pulmonary arterial pressure greater than 35 mmHg results in

a 10-fold decreased survival • Pulmonary hypertension is a contraindication to lung resection • PVR greater than 190 dynes is associated with 90% mortality • Unfortunately, pulmonary function tests do not identify patients

with high PVR

Exercise Testing

• Maximal oxygen consumption (MVO2) may be useful in evaluating marginal patients

• Elevated blood lactate level during exercise may help predict mortality, but is not useful for postoperative complications

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Summary

1. Postoperative lung function (based on spirometry and V/Q scans) can be predicted

2. In pneumonectomy patients the best predictor of death was calculated postoperative FEV1

3. In all patients the best predictor of death was calculated postoperative DLCO

4. Best predictor of overall complications was DLCO and predicted postoperative DLCO

Guidelines for Patient Selections (2) FVC <50% of predicted FEV1 <50% of predicted DLCO <60% of predicted MVV <50% of predicted (with good patient cooperation)

638

SPECIFIC CONSIDERATIONSUNIT IIPART II

VO2max > 15 ml/kg/min

Perform spirometry

Unexplained dyspneaor diffuse parenchymaldisease on CXR/CT?

Perform CPET

Estimate %ppoFEV1 and %ppo

DLCO

Increased risk

VO2max >15 mL/kg/min

VO2max 10 to 15 mL/kg/min

VO2max <10 mL/kg/min

FEV1 >1.5 L lobectomyFEV1 >2 L pneumonectomyFEV1 >80% predicted

FEV1 <1.5 L lobectomyFEV1 <2 L pneumonectomyFEV1 <80% predicted

YesNo

DLCO >80%predicted

DLCO <80%predicted

%ppo FEV1 and%ppo DLCO >40

%ppo FEV1 or%ppo DLCO <40

%ppo FEV1 <30 or%ppo FEV1 x%ppo DLCO <1650

Measure DLCO

Average risk Increased risk

Figure 19-24. Algorithm for preoperative evaluation of pulmonary function and reserve prior to resectional lung surgery. CPET = cardiopul-monary exercise test; CT = computed tomographic scan; CXR = chest radiograph; Dlco = carbon monoxide diffusion capacity; FEV1 = forced expiratory volume in 1 second; %ppo = percent predicted postoperative lung function; V

.o2 max = maximum oxygen consumption. (Repro-

duced with permission from the American College of Chest Physicians from Colice GL, et al: Physiologic evaluation of the patient with lung cancer being considered for resectional surgery: ACCP Evidence-based Clinical Practice Guidelines. (2nd edition) Chest. 2007;132:161S.)

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Scalene Node Biopsy

A. Indicated with a palpable node B. Staging ie. nonpalpable is more controversal 1. SNB vs. Mediastinoscopy a. M&M the same b. Yield: SNB - 10% positive, Mediastinoscopy- 30% positive C. Technique - anatomical bonderies include: inferiorly: subclavian vein medially: internal jugular posteriorly: anterior scalene

Mediastinoscopy

A. Positive node 30-35% of the time B.Controversy 1. Do patients with suspected cancer who are otherwise operable need this procedure 2. What to with a positive node (N2 Disease) C. Studies 1. Maassen -1921 cases of Stage I & II central masses - 23% positve nodes peripheral masses - 19% positive D. CT Scan 1. Negative predictive rate 80-97% 2. Positive predictive rate 44-82% 3. Dales: meta-analysis false negative 20% false positive 21% 4. Kerr: 15% of positive restricted nodes are less than 10mm E. N2 Disease carries a 5 yr survival of 3% (Mountain et al 28%) 1. exceptions: a. limited aortopulmonary nodes and no anterior mediastinal nodes b. non-small cell with limited ipsilateral tracheobroncial intranodal involvement F. Morbidity less than 0.1% G. Technique

Bronchoscopy

Indications • A wide range of diseases are indications for either diagnostic or

therapeutic bronchoscopy, most commonly carcinoma, pulmonary infections, and interstitial lung disease

• The surgeon must perform bronchoscopy prior to thoracotomy on any patient who may undergo pulmonary resection

• Specific indications for the procedure include chronic, persistent cough; hemoptysis; ocalized wheezing; and bronchial obstruction

Harris RJ. Kavuru MS. Rice TW. Kirby TJ. The diagnostic and therapeutic utility of thoracoscopy. Chest 1995 108(3):828-41.

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Diagnostic Bronchoscopy • An 8 x 40mm rigid bronchoscope is suitable for most adults • The flexible bronchoscope is useful for peripheral or upper lobe

lesions, but is limited in the presence of thick secretions and excessive bleeding

• Either topical or general anesthesia may be used depending on patient status and age

• As the tip of the bronchoscope passes the larynx, the patient's head is lowered and extended

• Use ball-tip or cup forceps for tissue sampling, and always biopsy proximal to a gross tumor to define the upper limit of the tumor

• Flexible bronchoscopy allows for transbronchial needle biopsy, washings, and brushings

Therapeutic Bronchscopy • Most foreign bodies can be removed by bronchoscopy;

grasping forceps or a Fogarty catheter are the most useful • Heavy retained secretions can usually be drained using flexible

bronchoscopy with a large side port • Palliation resection of endobronchial obstructing tumors can be

done with the Nd:YAG laser • Other treatments include brachytherapy and phototherapy

Specific Conditions • Bronchogenic carcinoma can present as an endobronchial

mass, submucosal or peribronchial involvement, a peripheral mass, diffuse metastatic disease, or occult tumor

• Masses and metastatic disease should be directly biopsied, while submucosal involvement and peripheral masses requires transbronchial needle biopsy, washings and brushings

• Occult tumors are discovered from positive sputum cytology; a thorough bronchoscopic examination will find the majority of these lesions

• Bronchoscopy is also useful for tuberculosis, fungal infections, and opportunistic infections in the immunocompromised patient

Textbook Chapters Chapter 1: Preoperative Assessment of Pulmonary Function: Quantitative Evaluation of Ventilation and Blood Gas Exchange. Surgery of the Chest (Sabiston and Spencer), 6th ed., 1-21. Chapter 2: Perioperative Pulmonary Physiology. Surgery of the Chest (Sabiston and Spencer), 6th ed., 22-68.

Chapter 3: Endoscopy, Bronchoscopy, and Esophagoscopy. Surgery of the Chest (Sabiston and Spencer), 6th ed., 69-97

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Congenital Lesions Agenesis, Aplasia, and Hypoplasia

A. Agenesis and Aplasia · Agenesis = complete absence of carina, main bronchus, lung, and pulmonary vasculature · Aplasia = development of carina and rudimentary main bronchus; absence of lung and pulmonary vessels · Bilateral pulmonary agenesis extremely rare and uniformly fatal · More than 50% of patients with unilateral agenesis have other associated anomalies · Agenesis does not show a R. or L. predominance · Lobar agenesis is less common that total lung agenesis

B. Hypoplasia · Failure to obtain adequate size; all components are present, but incompletely developed · Severity of hypoplasia determines the degree of respiratory compromise · Major abnormality is a decrease in the number of airway generations and pulmonary artery branchings · Two general causes: large diaphragmatic hernia or primary embryologic defect · Hernia interferes with alveolar development during last 2 months of intrauterine growth · Embryologic defects occur early and may be associated with other syndromes · Associated anomalies include oligohydramnios, prune-belly syndrome, Potter's syndrome, and scimitar syndrome · If associated with diaphragmatic hernia, the hypoplastic lung should not be removed at surgery, as it will recover some function with time

Congenital Lobar Emphysema (CLE)

· Definition: over-expansion of a pulmonary lobe with compression of other lobes and shifting of mediastinum

Pathogenesis · Accounts for about 50% of all congenital lung malformations · Intrinsic causes: bronchomalacia due to abnormal cartilaginous support, mucus plugging, bronchial torsion, redundant mucosa, foreign body aspiration · Extrinsic causes: bronchial compression from pulmonary artery sling, great vessel aneurysms, enlarged cardiac chambers · Pulmonary hypoplasia: decreased number of alveoli become overdistended due to air trapping · Alveolar hyperplasia: excessive number of alveoli overexpanded a polyalveolar lobe · Most common in LUL, then RUL, then RML

Symptoms · Respiratory distress occurs in 50% of patients in the first week of life (50%) · 80% of infants will be symptomatic by 6 months of age · Most common symptoms are tachypnea, cough, cyanosis, and dyspnea · Pulmonary infection in the affected lobe occurs most commonly between 1 and 6 months of age · Presentation is uncommon after age 6 months

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· Physical findings include hyperresonance and decreased breath sounds on the affected side · CXR shows hyperlucency on the affected side and mediastinal shift to the opposite side · Air bronchography demonstrates bronchial wall collapse with expiration if bronchomalacia is the cause Treatment · Indication for surgery is life-threatening progressive insufficiency from normal lung compression · Careful induction of anesthesia and positive pressure ventilation · Immediate thoracotomy necessary in the critically ill infant, as ventilation may worsen normal lung compression by rapidly expanding the affected lung · Surrounding atelectatic normal lung tissue should be preserved · Lobectomy should result in cure · Long-term outlook is good with relatively normal PFT's

Pulmonary Cysts

· Definition: cystic structures within the pulmonary parenchyma. The composition of the cyst wall is determined by its origin: bronchial glands, cartilage, or alveolar epithelium.

Pathology · Congenital cysts are typically unilocular and confined to a single lobe; the lower lobes are more commonly involved · Congenital cysts that persist for more than 1 year are unlikely to resolve spontaneously · Multiple cysts are rarely congenital and are probably acquired; causes include staphylococcal pneumonia and cystic fibrosis. · Multiple cysts may fluctuate in size and can develop rapidly · CXR usually adequate for identification

Symptoms · Expansion results in respiratory distress · Infection causes fever, cough, and sepsis

Treatment · Solitary congenital cysts can be treated with cystectomy or lobectomy if necessary · Infected solitary cysts should be treated with antibiotics and resected when quiescent · Multiple cysts should be treated as part of underlying systemic disease; surgical intervention is rarely required and is contraindicated for pneumatoceles · Chest tube placement or aspiration for diagnosis is discouraged for tension cysts, as this can result in empyema

Cystic Adenomatoid Malformation

Definition: thoracic hamartoma with overgrowth in varying amounts of bronchioles and alveoli, so it characteristically can range from cystic to nearly solid masses within the lung. Cartilage, however, is not present in this lesion.

Pathology · Typically confined to a single lobe · Progressive air trapping in cystic areas leads to over distension of involved lobe · Three subtypes (see table) · High incidence of associated congenital anomalies and death with type II

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Cystic Adenomatoid Malformation Type I Type II Type III Morphology Multiple large

cysts or single large cyst

Multiple, evenly spaced cysts not over 1.2 cm

Multiple small cysts less than 0.5 cm

Contents Air of fluid Air Solid Wall Smooth muscle

and connective tissue

Terminal bronchioles

Adenomatoid hyperplasia or bronchial

CXR Unilateral large cyst or homogenous mass, may have mediastinal shift and air-fluid level

Smaller nonhomogenous cyst

Homogenous mass

Symptoms · Progressive respiratory distress in a newborn infant · Tachypnea, subcostal retraction, cyanosis · Older child - chronic pulmonary infection · Differential should include congenital diaphragmatic hernia, and barium swallow will differentiate the two

Treatment · Identification of the lesion is the indication for surgery, with or without symptoms · Lobectomy is usually adequate · Attempt at segmentectomy is usually met with prolonged air leak and complications

Bronchogenic Cysts

Definition: congenital cystic lesions arising from abnormal budding of primitive tracheobronchial tree; composed of fibrous tissue interposed with normal bronchial elements

Pathology · Cysts can occur either in the parenchyma (70%) or the mediastinum (30%) · The most common locations are paratracheal, carinal, hilar, and paraesophageal · Generally round and unilocular · Lined with ciliated columnar epithelium · Most do not communicate with the tracheobronchial tree · Can mimic lobar emphysema from bronchial obstruction · Can become secondarily infected

Symptoms · Most common presentation in the neonate is progressive dyspnea, wheezing, stridor, and cyanosis · Obstructive symptoms occur most commonly in infants less than 1 year of age · Paratracheal, carinal, and hilar cysts are commonly asymptomatic · Communicating cysts are almost always symptomatic: productive cough, fever, hemoptysis · CXR shows homogenous, sharply delineated cyst; air/fluid levels may be present in communicating cyst · CT is the test of choice for diagnosis and anatomic delineation

Treatment · Indications for surgery include increasing cyst size, air/fluid level, symptomatic cyst, and subcarinal cyst · Small, asymptomatic cysts can be followed with interval CXR

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· Cyst excision with sparing of surrounding pulmonary tissue is the treatment of choice Intralobar Pulmonary Sequestration (IPS)

Definition: segment of lung parenchyma that is within the normal pleural confines, but does not communicate with the tracheobronchial tree and is supplied by the systemic circulation.

Pathology · Unicystic or polycystic parenchyma with extensive fibrosis and vascular sclerosis · Arterial supply is usually from the thoracic aorta (75%) or abdominal aorta (20%) · Venous drainage is usually to the pulmonary veins · R.-sided lesions more often have other venous drainage · Much more frequent than extralobar type

Symptoms · Recurrent episodes of infection in older children and adolescents · CXR shows cystic structure with or without air-fluid level · CT is procedure of choice; aortography and barium swallow may be necessary for anatomy

Comparison of ILS and ELS ILS ELS Gender predominance

Equal Male

Location predominance

L. L.

Pleural investment No Yes Arterial supply Thoracic/abdominal

aorta Thoracic/abdominal aorta

Venous drainage Pulmonary Azygous/hemiazygous Other anomalies Rare Common Age at diagnosis Adolescent/young

adult Neonate

Symptoms Cough, fever Respiratory distress (Adapted from Sabiston and Spencer)

Treatment · Identification of the lesion is indication for surgery · Careful ligation of the anomalous artery followed by resection · Prevents long-term infection and possible neoplastic changes

Extraobar Pulmonary Sequestration (EPS)

Definition: segment of lung parenchyma with distinct and separate pleural investment; does not communicate with the tracheobronchial tree and is supplied by the systemic circulation.

Pathology · Mass of loose, spongy parenchyma with multiple small cysts and dilated bronchioles and ducts · Arterial supply is also usually from the thoracic or abdominal aorta · Venous drainage is usually to the azygous or hemiazygous system · More common on the L. side and usually found between the lower lobe and the diaphragm

Symptoms · Most present in neonatal period due to respiratory distress · CXR shows triangular homogenous mass with apex pointing toward

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hilum · CT may provide additional localization, but vessels often too small to identify Treatment · Identification of the lesion is indication for surgery · Careful ligation of the anomalous artery followed by resection · As with intralobar sequestration, resection prevents long-term infection and possible neoplastic changes

Selected Articles

Bailey PV, Tracy T Jr, Connors RH, deMello D, Lewis JE, Weber TR. Congenital bronchopulmonary malformations. Diagnostic and therapeutic considerations. J Thorac Cardiovasc Surg 1990 99(4):597-602.

Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM. The multiple facets of pulmonary sequestration. J Pediatr Surg. 2001 May;36(5):784-90.

Cioffi U, Bonavina L, De Simone M, Santambrogio L, Pavoni G, Testori A, Peracchia A. Presentation and surgical management of bronchogenic and esophageal duplication cysts in adults. Chest. 1998 Jun;113(6):1492-6.

Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest 1993 103(3):761-4. Evrard V, Ceulemans J, Coosemans W, De Baere T, De Leyn P, Deneffe G, Devlieger H, De Boeck C, Van Raemdonck D,

Lerut T. Congenital parenchymatous malformations of the lung. World J Surg. 1999 Nov;23(11):1123-32. Gustafson RA, Murray GF, Warden HE, Hill RC, Rozar GE. Intralobar sequestration. A missed diagnosis. Ann Thorac Surg

1989 47(6):841-7. Martinod E, Pons F, Azorin J, Mouroux J, Dahan M, Faillon JM, Dujon A, Lajos PS, Riquet M, Jancovici R. Thoracoscopic

excision of mediastinal bronchogenic cysts: results in 20 cases. Ann Thorac Surg. 2000 May;69(5):1525-8.

Olutoye OO, Coleman BG, Hubbard AM, Adzick NS. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg. 2000 May;35(5):792-5.

Ribet ME, Copin MC, Gosselin BH. Bronchogenic cysts of the lung. Ann Thorac Surg. 1996 Jun;61(6):1636-40. Suen HC, Mathisen DJ, Grillo HC, LeBlanc J, McLoud TC, Moncure AC, Hilgenberg AD. Surgical management and

radiological characteristics of bronchogenic cysts. Ann Thorac Surg 1993 55(2):476-81. Winters WD, Effmann EL. Congenital masses of the lung: prenatal and postnatal imaging evaluation. J Thorac Imaging. 2001

Oct;16(4):196-206. Sources for further reading

Textbook Chapters Chapter 24: Congenital Lesions of the Lung and Emphysema. Surgery of the Chest (Sabiston and Spencer), 5th ed., 762-95.

Chapter 13: Developmental Abnormalities of the Airways and Lungs: Thoracic Surgery in Childhood. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 231-46.

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Bullous & Bleb Diseases Bullous Emphysema

Anatomic Classification of Emphysema. 4 types - by the way it involves the acinus Proximal acinar emphysema (centrilobular) Associated with cigarette smoking and inflammation of distal airways Symptomatic chronic airflow obstruction Panacinar (panlobular) Involves entire acinus uniformly Alpha-1 anti-trypsin deficiency and other Pi-associated emphysema Worse in lower zones of the lung Distal acinar (paraseptal) Distal acinus, ducts, and alveolar sacs Fibrosis Subpleural location - PTX and BULLOUS disease Irregular Affects acinus in an irregular manner Always associated with scarring and fibrosis Occurs to some degree in all lungs

Definition WHO and ATS Definition of Emphysema Emphysema is characterized by an increase beyond normal in the sizes of air spaces distal to the terminal non-respiratory bronchiole that arises from the destruction of their walls Characteristics Definition confused by overlap in etiology and symptomatology among

emphysema asthma chronic bronchitis

2/3 of adults show some emphysema at autopsy 10% have severe clinical disease 10% of cigarette smokers have significant chronic airflow obstruction associated with chronic bronchitis

Pathogenesis Earlier studies: Infective, degenerative, obstructive mechanical factors Present studies: Enzymatic mechanisms of tissue destruction

Protease pathogenesis hypothesis Destruction of the interstitium is due to an excess of proteolytic enzymes (elastase) in relation to the availability of proteolytic inhibitors Heritable alpha -1 anti-trypsin deficiency Animal studies with elastolytic proteolases

Cooper JD, Patterson GA, et al. Results of 150 consecutive bilateral lung volume reduction procedures in patients with severe emphysema. J Thorac Cardiovasc Surg. 1996 Nov;112(5):1319-29; discussion 1329-30.

Cooper JD, Trulock EP, Triantafillou AN, et al. Bilateral pneumectomy (volume reduction) for chronic obstructive pulmonary disease. J Thorac Cardiovasc Surg. 1995 109(1):106-19.

Cooper JD. Paying the piper: the NETT strikes a sour note. National Emphysema Treatment Trial. Ann Thorac Surg. 2001 Aug;72(2):330-3.

Cooper JD. The history of surgical procedures for emphysema. Ann Thorac Surg. 1997 Feb;63(2):312-9.

Date H, Goto K, Souda R, et al. Bilateral lung volume reduction surgery via median sternotomy for severe pulmonary emphysema. Ann Thorac Surg. 1998 Apr;65(4):939-42.

DeMeester SR, Patterson GA, Sundaresan RS, Cooper JD. Lobectomy combined with volume reduction for patients with lung cancer

Alpha -1 protease inhibitor (alpha-1 anti-trypsin) Major protease in human lower respiratory tract Cigarette smoke - oxidative inactivation of alpha-1 anti-protease by oxidants in smoke or econdarily by active oxygen species liberated by pulmonary phagocytes

Alveolar macrophage cells of smokers - increased amounts of oxygen radicals and peroxide Decrease elastin resynthesis after elastolytic destruction of lung tissue in animals

Alpha-1 Antiprotease (anti-trypsin)

Plasma protease inhibitor - synthesized in liver Primary site of action is alveolar structure of lung Inhibits PMN leukocyte elastase Protects elastic fibers from hydrolysis

Enzyme deficiency - risk of pulmonary disease 20-30 times that of the general population

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40,000 people in the US 1-2% of those with emphysema Synthesis controlled by autosomal and allelic gene system

Most are homozygous for PiM - nl phenotype PiZ phenotype - severe deficiency of the enzyme Homozygous with PiZ phenotype have 10-15% of nl

and advanced emphysema. J Thorac Cardiovasc Surg. 1998 Mar;115(3):681-8.

DeRose JJ Jr, Argenziano M, El-Amir N, et al. Lung reduction operation and resection of pulmonary nodules in patients with severe emphysema. Ann Thorac Surg. 1998 Feb;65(2):314-8.

Drazen JM. Surgery for emphysema--not for everyone. N Engl J Med. 2001 Oct 11;345(15):1126-7.

Clinical Classification of Emphysema Compensatory emphysema

Diffuse obstructive emphysema (Major component of COPD )

Not really emphysema - no acinar destruction Infantile lobar emphysema; localized emphysema with infection; bronchial tumor or aspirated FB; compensatory changes after pulmonary resection

Type A or dry emphysema: Type B or wet emphysema:

Cough, dyspnea, barrel chest, CXR with overinflation, flat diaphragms, no fibrosis and decreased pulmonary vasculature; pan-acinar type destruction;

No obvious CXR or clinical signs; severe cough; copious sputum production; rales; wheezing; cyanosis; dyspnea later; CXR - moderate hyperinflation; fibrosis in lower lobes; increase in central pulmonary vascularity with tapering Centrilobular changes;

prognosis good

prognosis poor

Surgical Treatment Unsuccessful in providing improvement Associated with high mortality Limited resections have some validity

Bullous emphysema: Congenital cysts to blebs Bleb: Intrapleural airspace - separated from alveoli by a thin pleural covering that can rupture spontaneously Bulla: Subpleural airspace usually larger than a bleb that results from the destruction of pulmonary tissue

Bullous emphysema: Refers to thin walled air sacs under tension that cause compression atelectasis of normal pulmonary tissue

Single/multiple/coalesce Air/fluid/infection

Geddes D, Davies M, Koyama H,

et al. Effect of lung-volume-reduction surgery in patients with severe emphysema. N Engl J Med. 2000 Jul 27;343(4):239-45.

McKenna RJ Jr, Brenner M, Gelb AF, et al A randomized, prospective trial of stapled lung reduction versus laser bullectomy for diffuse emphysema. J Thorac Cardiovasc Surg. 1996 111(2):317-21.

Meyers BF, Yusen RD, Guthrie TJ, et al. Outcome of bilateral lung volume reduction in patients with emphysema potentially eligible for lung transplantation. J Thorac Cardiovasc Surg. 2001 Jul;122(1):10-7.

Meyers BF, Yusen RD, Lefrak SS, Cooper JD. Improved long-term survival seen after lung volume reduction surgery compared to

Bullae Can form all pathologic forms of emphysema Periacinar bullae are probably most common in patients who are referred for surgery Can develop fluid Infection Selection of patients for bullous surgery is based upon clinical, radiologic and physiologic data

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Must compare symptoms of bulla to symptoms of underlying pulmonary disease Dyspnea is most common symptom of bullous disease Presence alone implies good surgical result Cor-pulmonale and R. heart failure does not necessarily contraindicate surgery PFT's: +/- Review old CXR's Bronchography - clumping of airways Perfusion scans - split pulmonary functions Pulmonary arteriography Exercise PFT's CT scanning

continued medical therapy for emphysema. Ann Thorac Surg. 2001 Jun;71(6):2081.

Meyers BF, Yusen RD, Lefrak SS, et al. Outcome of Medicare patients with emphysema selected for, but denied, a lung volume reduction operation. Ann Thorac Surg. 1998 Aug;66(2):331-6.

Naef AP. History of emphysema surgery. Ann Thorac Surg. 1997 Nov;64(5):1506-8.

Ramsey SD, Sullivan SD, Kaplan RM, et al. Economic analysis of lung volume reduction surgery as part of the National Emphysema Treatment Trial. NETT Research Group. Ann Thorac Surg. 2001 Mar;71(3):995-1002.

Rationale and design of the National Emphysema Treatment Trial (NETT): A prospective randomized trial of lung volume reduction surgery. J Thorac Cardiovasc Surg. 1999 Sep;118(3):518-28.

Serna DL, Brenner M, Osann KE, et al. Survival after unilateral versus bilateral lung volume reduction surgery for emphysema. J Thorac Cardiovasc Surg. 1999 Dec;118(6):1101-9.

Stirling GR, Babidge WJ, Peacock MJ, et al. Lung volume reduction surgery in emphysema: a systematic review. Ann Thorac Surg. 2001 Aug;72(2):641-8.

Sundaresan RS, Shiraishi Y, Trulock EP, et al. Single or bilateral lung transplantation for emphysema? J Thorac Cardiovasc Surg. 1996 Dec;112(6):1485-94; discussion 1494-5.

Wood DE, DeCamp MM. The National Emphysema Treatment Trial: a paradigm for future surgical trials. Ann Thorac Surg. 2001 Aug;72(2):327-9.

Sources for further reading Textbook Chapters Chapter 14: Surgical Treatment of Bullous Emphysema. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 247-258.

No single test shows which patients with bullous emphysema will benefit from surgery

Each requires careful study Surgical techniques Surgical objective: Remove as little functioning pulmonary tissue as possible Complications:

Air leaks (prolonged) Residual PTX

Mortality rate: 10-22% Related to severity of underlying disease Better selection, mortality rate decreases

Median sternotomy - less morbidity Intracavitary suction Epidural anesthesia with lateral thoracotomy

Alpha-1 Anti-trypsin Deficiency Onset before 40 years Higher incidence in females Family history important CXR: Emphysematous changes in lower ½ of chest Angiograms and scintigrams show greater perfusion in upper lobes than the bases Ventilation scans show basal air trapping Tx:

Replacement Tx with alpha-1 protease inhibitor - some early success Lung transplantation

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Infections of the Lung Lung Abscess

1. Definition A pyogenic pneumonia develops, causing localized suppuration with parenchymal destruction. There is central necrosis of lung tissue, which then liquefies and communicates with the bronchial tree. This partial internal drainage results in the classic cavity with an air-fluid level.

2. Etiology A. Aspiration: · Location: predilection for the posterior segment of the RUL, the superior segment of the RLL, and the LLL · Bacteriology: anaerobes and aerobes

B. Post-pneumonic · Location: any segment, may be in multiple segments · Bacteriology: staphylococcus and streptococcus

C. Endobronchial obstruction · Neoplasms and foreign bodies can result in distal infection and abscess formation

Mnemonic: Lung Cavity ”CAVITY” C: Cancer A: Autoimmune (Wegeners, RA) V: Vascular (septic emboli) I: Infectious (TB, Abscess) T: Trauma Y: Young (bronchogenic cyst, laryngotracheal papillomatosis) Sources for further reading

Textbook Chapters Chapter 16: The Pleura. Surgery of the Chest (Sabiston and Spencer), 5th ed., 467-76. Chapter 20: Lung Infections and Diffuse Interstitial Lung Disease. Surgery of the Chest (Sabiston and Spencer), 5th ed., 615-22.

Chapter 16: Pneumonia, Bronchiectasis, and Lung Abscess. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 283-302.

3. Clinical Presentation A. Symptoms: Fever, chills, severe cough, hemoptysis, and copious foul smelling sputum

B. Signs: Tachypnea, consolidation, local chest wall tenderness

4. Diagnosis • CXR: pneumonitis pattern early, followed by the air/fluid level

when bronchial communication has been established • Bacteriological/fungal evaluation: aerobic, anaerobic,

fungal, TB all important Bronchoscopy is indicated to rule out obstructing foreign bodies or neoplasms and can provide drainage

5. Treatment · Most abscesses respond to bronchoscopy and appropriate medical treatment · Surgery reserved for medical failure, carcinoma, significant hemoptysis, and complications such as empyema and bronchopleural fistula · Lobectomy is typically required when resection is in order, as segmentectomy is often not possible and pneumonectomy rarely necessary

Mnemonic: Solitary Pulmonary Nodule (SPN) “CASH PLEASE” C: Cancer A: Abscess S: Solitary met H: Hamartoma P: Psuedotumor L: Lymphoma E: Echinococcus A: Actinomycosis

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· Percutaneous drainage can be useful in patients who are not candidates for standard surgical intervention

S: Sequestration

Bronchiectasis Agasthian T, Deschamps C,

Trastek VF, Allen MS, Pairolero PC. Surgical management of bronchiectasis. Ann Thorac Surg 1996 62(4):976-8.

Barker AF. Bronchiectasis. Seminars in Thoracic and Cardiovascular Surgery 1995 7(2):112-8.

Fujimoto T, Hillejan L, and Stamatis G. Current strategy for surgical management of bronchiectasis. Ann Thorac Surg 2001 72(5): 1711-1715.

Definition · Chronic bronchial dilatation with parenchymal infection and inflammatory reaction. There are three types: cylindrical, varicose, and saccular/cystic. Etiology · Acquired infection is the most common cause, typically when occurring in childhood. · Other acquired causes include bronchial obstruction and scarring · Congenital causes include cystic fibrosis, Kartagener's syndrome, various immunodeficiency disorders, and bronchopulmonary sequestration · Typically affects the basal segments of the lower lobes

Clinical presentation · Recurrent pneumonia, persistent cough, copious foul smelling sputum · Hemoptysis is common in adults but rare in children

Diagnosis CXR usually demonstrates nonspecific findings, although a honeycomb pattern may be found Bacteriologic studies typically return H. influenza, E. coli, and Klebsiella as the causative agents Chest CT with fine cuts has replaced bronchography as the test of choice Bronchoscopy can help localize the process, rule out obstructing lesions, and provide pulmonary toilet

Mnemonic: Basilar Lung Disease “BAD RASH” B: Bronchiectasis, BOOP A: Aspiration D: Drugs R: Rheumatoid A: Asbestosis S: Scleroderma H: Hamman-Rich

Treatment · Medical therapy is the primary approach, using antibiotics, humidification, bronchodilators · Surgical intervention is indicated for failure of medical management, persistent symptoms, recurrent pneumonias, and hemoptysis · The ideal surgical candidate has unilateral disease confined to one lobe, usually lower · Most patients, however, have bilateral disease, and surgery should be reserved for localized disease, operating on the worst side first

Pulmonary Tuberculosis & Fungus Pulmonary Tuberculosis

1. Etiology M. tuberculosis is a virulent organism transmitted by airborne droplets that can rapidly destroy lung tissue if L. untreated Three million deaths occur worldwide due to TB The incidence had been declining until 1985 and is now rising Resectional therapy is becoming increasingly utilized with the rise in multidrug resistant organisms

2. Diagnosis Classic symptoms include night sweats, fever, cough, and occasionally hemoptysis

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Fungal infections of the lung Indications for Thoracic Surgical Intervention Establish a diagnosis

Tuberculosis is most common cause of severe hemoptysis Asphyxiation rather than hypovolemia is usual cause of death from hemoptysis The diagnosis is made by acid-fast staining and culture of the sputum The Ghon complex is characterized by a peripheral lesion with associated hilar adenopathy Other forms of mycobacterium, most commonly M. avium, can cause indolent infections that attack diseased pulmonary tissue 3. Medical Therapy First line drug therapy includes isoniazid, ethambutol, pyrazinamide, and streptomycin A minimum of 3 drugs should be initiated when the diagnosis is made The preferred regimen is isoniazid and rifampin for 6 months, with pyrazinamide for 2 months If the sputum is positive after 3 months of treatment, either the patient is noncompliant or the organism is an uncommon mycobacterium or is resistant; the patient should be recultured Pure tuberculous effusions almost always resolve spontaneously or respond promptly to chemotherapy Tube thoracostomy rarely provides a cure because dense pleural reaction interferes with full re-expansion of the underlying lung

Pomerantz BJ, Cleveland JC Jr, Olson HK, Pomerantz M. Pulmonary resection for multi-drug resistant tuberculosis. J Thorac Cardiovasc Surg 2001 Mar;121(3):448-53.

Souilamas R, Riquet M, Barthes FP, Chehab A, Capuani A, Faure E. Surgical treatment of active and sequelar forms of pulmonary tuberculosis. Ann Thorac Surg 2001 Feb;71(2):443-7.

van Leuven M, De Groot M, Shean KP, von Oppell UO, Willcox PA. Pulmonary resection as an adjunct in the treatment of multiple drug-resistant tuberculosis. Ann Thorac Surg 1997 May;63(5):1368-72; discussion 1372-3.

Mouroux J, Maalouf J, Padovani B, Rotomondo C, Richelme H. Surgical management of pleuropulmonary tuberculosis. Journal of Thoracic and Cardiovascular Surgery 1996 111(3):662-70.

Pomerantz M and Brown J. The surgical management of tuberculosis. Seminars in Thoracic and Cardiovascular Surgery 1995 7(2):108-11.

4. Indications for Surgical Resection Persistently positive sputum cultures with cavitation after 5-6 months of continuous optimal chemotherapy with 2 or more drugs Localized pulmonary disease cause by M. avium-intracellulare; other atypical mycobacterium or M. tuberculosis which is drug resistant Mass lesion of the lung in an area of tuberculous involvement Massive life-threatening hemoptysis or recurrent severe hemoptysis (massive = 600 cc or more/24 hr, severe = 200 cc/24 hr) Bronchopleural fistula in association with mycobacterial infection that does not respond to tube thoracostomy 5. Contraindications to Resection in TB Widespread pulmonary or endobronchial disease Children with mycobacterial disease FEV1 less than 800-1,000 cc Active endobronchial disease, as this interferes with healing of bronchial stump (pre-op bronchoscopy in all patients prior to resection) 6. Operative Management Lobectomy or pneumonectomy usually necessary with active mycobacterial disease Extrapleural pneumonectomy for extensive pulmonary parenchymal disease with chronic empyema (rare) Complications of resection include empyema with or without broncho-pleural fistula and bronchogenic spread of mycobacterial disease Both complications aremore frequent when the sputum is positive at the time of operation

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Failure of medical therapy Fungal disease vs. lung carcinoma

Fungal Infections of the Lung

• The three major mycotic infections are histoplasmosis, coccidiomycosis, and blastomycosis

• The fungal agent in each case is dimorphic: exists in nature as mycelium (mold) that bears infectious spores, which enter host and develop into a yeast-like phase that is the tissue pathogen

• These fungi require special staining and culture methods • Amphotericin B is primary therapy for all three fungal infections

Histoplasmosis Systemic disease caused by H. capsulatum, which is found in soil with high concentration of fecal material of chickens, pigeons and bats Endemic areas are the valleys of Ohio, Missouri, and Mississippi rivers Occurs as an intracellular yeast and is seen best on silver stain Farmers, construction workers, and people who enjoy outdoor activities are the most at risk by inhaling spores 1 in 2000 people will develop chronic pulmonary disease Most cases of primary histoplasmosis are asymptomatic or mild flu-like syndrome Progressive pulmonary disease associated with chronic lung disease occurs in middle-aged men CXR demonstrates dense nodules with central calcification Chronic cavitary histoplasmosis is less common Mediastinal histoplasmosis results in fibrosing mediastinitis, which is the most frequent benign etiology of SVC obstruction Healed histoplasmosis causes a solitary pulmonary nodule, which can be confused with carcinoma

Babatasi G, Massetti M, Chapelier A, Fadel E, Macchiarini P, Khayat A, Dartevelle P. Surgical treatment of pulmonary aspergilloma: current outcome. J Thorac Cardiovasc Surg 2000 May;119(5):906-12.

Regnard JF, Icard P, Nicolosi M, Spagiarri L, Magdeleinat P, Jauffret B, Levasseur P. Aspergilloma: a series of 89 surgical cases. Ann Thorac Surg 2000 Mar;69(3):898-903.

Chatzimichalis A, Massard G, Kessler R, Barsotti P, Claudon B, Ojard-Chillet J, Wihlm JM. Bronchopulmonary aspergilloma: a reappraisal. Ann Thorac Surg 1998 Apr;65(4):927-9.

Newsom BD and Hardy JD. Pulmonary fungal infection. Survey of 159 cases with surgical implications. Journal of Thoracic and Cardiovascular Surgery 1982 83(2):218-26.

Haque AK. Pathology of common pulmonary fungal infections. Journal of Thoracic Imaging 1992 7(4):1-11.

Indications for surgery: A. Chronic cavitary pulmonary disease - persistent thick-walled cavity after 2g to 3g course of Amphotericin B for 2-3 months B. Fibrosing mediastinitis with middle lobe syndrome C. SVC syndrome

Coccidiomycosis Causative agent is C. immitis Endemic areas are the Southwestern USA and Mexico The spherules have a thin wall containing endospores which are seen on wet mount slides Infection occurs after spore inhalation and 60% of patients are asymptomatic Acute valley fever is characterized by pneumonitis, erythema nodosum, and arthralgias Only 5% of patients with symptomatic pulmonary disease develop irreversible bronchiectasis, pulmonary nodules, pulmonary abscesses or residual cavities The most frequent long-term complication is a chronic cavitary lesion (solitary, thin-walled) CXR demonstrates nonspecific infiltrates, hilar adenopathy, or pleural effusions Diagnosis is made by serologic testing for IgM or IgG antibodies; a rising titer suggests possible dissemination

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The most important extra pulmonary manifestation is meningitis Most patients require no therapy Amphotericin B is indicated if there is severe, prolonged pulmonary disease; primary disease with risk of dissemination (pregnancy, immunosuppression); symptomatic, chronic cavitary disease; and as an adjuvant to surgical resection Indications for surgery: A. Enlarging cavitary lesion B. Hemoptysis C. Secondary infection Peri-operative Amphotericin B is recommended for these patients

Blastomycosis Causative agent is B. dermatitides The endemic areas are the southeastern and southwestern USA, mostly the Ohio and Mississippi river valleys and the Great Lakes area Classically seen as a round, thick-walled single yeast cell on staining Acute infection typically involves the lower lobes and is asymptomatic, or can result in influenza-like syndrome Chronic infection typically involves the upper lobes and results in a pyogranulomatous process The most common extrapulmonary manifestation is cutaneous ulcers Chronic, disseminated disease typically involves the skin and ribs Primary treatment is typically Amphotericin B - 2 to 2.5g over 2-3 months or ketoconazole as an alternative agent

Indications for surgery: A. Rule out malignancy B. Drainage of large cavitary abscesses C. Closure of bronchopleural fistulas

Other Fungal and Opportunistic Infections of the Lung A. Cryptococcosis Caused by C. neoformans, found in soil contaminated by pigeon droppings India ink staining reveals round, budding yeast which has gelatinous polysaccharide capsule Primarily involves the bronchopulmonary tree, with special predilection for the meninges Lesions often involve the lower lobes and are solid Amphotericin B and 5-fluorocytosine are both effective medical treatment Always examine CSF if C. neoformans is isolated from sputum or surgical specimen 10% of patients develop cryptococcal meningitis after resection of a pulmonary lesion


Textbook Chapters Chapter 20: Lung Infections and

Diffuse Interstitial Lung Disease. Surgery of the Chest (Sabiston and Spencer), 5th ed., 622-44.

Chapter 22: Surgical Treatment of Pulmonary Tuberculosis. Surgery of the Chest (Sabiston and Spencer), 5th ed., 690-707.

Chapters 17 and 18: Thoracic Infections Caused by Actinomycetes, Fungi, Opportunistic Organisms, and Echinococcus; Surgical Treatment of Tuberculosis and Other Pulmonary Mycobacterial Infections. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 303-30.

B. Aspergillosis Most are caused by A. fumigatus, an organism found in hay and grains Appears as mixture of coarse, fragmented hyphae and ball-like clusters on histology Three clinical syndromes occurs: aspergillar bronchitis, aspergilloma, and invasive aspergillosis

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Aspergillomas are the most common surgically resected lesion of this type and usually occur in a upper lobe cavity These may be asymptomatic for years or cause hemoptysis Hemoptysis occurs in 50% of patients with Aspergilloma, in 10% the hemoptysis is severe and recurrent Once hemoptysis develops, the aspergilloma should be resected Medical therapy usually not effective because Amphotericin penetrates aspergillus cavities poorly Prophylactic resection in asymptomatic patients is generally not indicated because of significant complication rate

C. Actinomyocis Causative agent is usually A. israelii, a microaerophilic organism Appears as branching hyphae which contain "sulfur granules" Thoracic infection occurs after aspiration of oropharyngeal organisms Presents as empyema, infiltrate, consolidation, or hilar mass Treatment of choice is high dose penicillin for 1-3 months

D. Nocardiosis Causative agent is N. asteroides Appears as long-branching filaments that can be confused with M. tuberculosis Opportunistic infection which occurs in immunocompromised patients CXR shows solitary nodules, nonspecific infiltrates, or cavitations Chest wall sinus tracts and empyema may occur, as well as CNS dissemination Treatment of choice is sulfadiazine, sulfisoxasolem, minocycline, or Bactrim for 2-3 months

E. Candidiasis Causative agent is C. albicans, which is normal flora in GI tract, oral cavity, and female genital tract Most common fungal infection in humans Becomes invasive in immunocompromised host Can cause deep thoracic infections or endocarditis Treatment of choice is Amphotericin B

F. Protozoal Infections Most common causative agent is P. carinii Occurs as a diffuse interstitial pneumonitis in immunocompromised patients CXR shows diffuse infiltrates radiating from the hilum Often causes hypoxemia, hypocapnia, and pneumothoraces Open lung biopsy may be required to establish diagnosis Treatment of choice is pentamidine or Bactrim

G. Pulmonary Echinococcosis Causitive agent is T. echinococcus, a small tapeworm Reults in intrathoracic cyst that can rupture, causing asphyxiation or allergic reaction

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CXR shows homogenous, oval shaped densities with clearly defined borders Surgical therapy involves cystectomy or pericystectomy with instillation of formalin or 10% NaCl solution Some success has been reported with medical treatment using benzimidazole derivatives Lung Cancer Epidemiology of Lung Cancer • 190,000 new cases in 2000 • 149,000 patients will die of lung cancer • 50 deaths per 100,000 per year • 5 yr survival 1981-87 - 13%. 1950-54 - 6% • Lung cancer is second to ischemic heart disease as the most

frequent cause of death • Heart disease mortality is decreasing • Cancer mortality is increasing mostly due to lung cancer


Textbook Chapters Chapter 19, Section I: Neoplasms of the Lung - Carcinoma of the Lung. Surgery of the Chest (Sabiston and Spencer), 6th ed., 635-49.

Chapters 21-7: Lung Carcinomas, Diagnosis and Staging of Lung Cancer, Surgical Treatment of Lung Carcinoma, Superior Sulcus Tumors, Limited Pulmonary Resection, Bronchoplastic Techniques for Lung Resection, Multimodality Therapy of Carcinoma of the Lung: Irradiation, Chemotherapy, and Immunotherapy. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 357-498.

Surgeon General's landmark report (1964) on smoking (85% of lung cancers) • Asbestos exposure is the most common occupational cause - 5%

1964 smoking incidence 54%, 1989 - 33% (80 million) • NCI target was 15% by 1990 • In spite of decreased incidence of smoking there is a long • latency period between smoking cessation and normalization of

risk (15 years) • Risk of CAD is decreased by 50% after one year of abstinence. Pathology of Lung Cancer A. Squamous cell carcinoma · Most common (40-70%), centrally located, more common in men · Local metastases, plentiful eosinophilic cytoplasm, keratin "pearls", bridging. B. Adenocarcinoma · Less common (5-15%), peripherally located, more common women · Distant metastases, vacuolization, mucus synthesis, glandular differentiation C. Undifferentiated carcinoma · Two subtypes (20-30%) · Large cell carcinoma: aggressive clinical behavior, moderate cytoplasm, no mucus or keratin · Small cell carcinoma: nonsurgical lesion, high incidence of metastases, spindle or oat shaped cells, dense nuclei, sparse cytoplasm D. Bronchoalveolar carcinoma · Uncommon (3-7%) adenocarcinoma variant, favorable prognosis, alveolar "scaffolding", tends to recur as a second primary tumor

E. WHO Classification Clinical Manifestation of Lung Cancer Cough (most common 75%), hemoptysis (33%), pain (50% poor prognostic signs), anorexia and weight loss (poor prognostic sign), shortness of breath, pleural effusion, hoarseness (1-8%) Cushing's syndrome (most common), inappropriate ADH secretion,

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eosinophilia (tumor necrosis) and neuromyopathies (15%) Primary Tumor (T) Full TNM Staging Classification

TX Primary tumor cannot be assessed, or tumor proven by the presence of malignant cells in sputum or bronchial washings but not visualized by imaging or bronchoscopy T0 No evidence of primary tumor Tis Carcinoma in situ T1 Tumor 3 cm or less in greatest dimension surrounded by lung or visceral pleura, without bronchoscopic evidence of invasion more proximal than the lobar bronchus (not in the main bronchus) T2 Tumor with any of the following features of size or extent:

More than 3 cm in greatest dimension Involves main bronchus, 2 cm or more distal to the carina Invades the visceral pleura Associated with atelectasis or obstructive pneumonitis that extends to the hilar region but does not involve the entire lung T3 Tumor of any size that directly invades any of the following: chest wall (including superior sulcus tumors), diaphragm, mediastinal pleura, parietal pericardium; or tumor in the main bronchus less than 2 cm distal to the carina, but without involvement of the carina; or associated atelectasis or obstructive pneumonitis of the entire lung

T4 Tumor of any size that invades any of the following: mediastinum, heart, great vessels, trachea, esophagus, vertebral body, carina; or separate tumor nodules in the same lobe; or tumor with a malignant pleural effusion.

Lymph Node (N) NX Regional lymph nodes cannot be assessed

N0 No regional lymph node metastasis N1 Metastasis to ipsilateral peribronchial and/or ipsilateral hilar lymph nodes and intrapulmonary nodes including involvement by direct extension of the primary tumor N2 Metastasis to ipsilateral mediastinal and/or subcarinal lymph node(s) N3 Metastasis to contralateral mediastinal, contralateral hilar, ipsilateral or contralateral scalene, or supraclavicular lymph node(s)

Distant Metastasis (M) MX Distant metastasis cannot be assessed

M0 No distant metastasis M1 Distant metastasis (includes synchronous separate nodule(s)

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in a different lobe)

Staging of Lung Cancer Staging of Lung Cancer Stage Ia All T1 tumors and T2 tumors without lymph

node metastasis Stage Ib Stage IIa T1 and T2 tumors with involvement of ipsilateral hilar

nodes Stage IIb Stage IIIa Ipsilateral mediastinal disease Stage IIIb More widespread disease Stage IV Unresectable disease

Important for prognosis and therapy.

Complete Pulmonary Resection • Surgeon is morally certain he or she has encompassed all tumor

disease • Proximal margins of resected specimen are microscopically free

of tumor • Within each major lymphatic drainage region, the most distal

node is microscopically free of tumor • Capsules of resected nodes are intact.

Lymph Node Mapping in Lung Cancer • Only 50% of patients with lung cancer are surgical candidates

50% of surgical patients have mediastinal N2 disease Mediastinal lymph node dissection is:

• Only definitive way of staging lung cancer Identifies patients with skip metastasis (33% incidence especially in adenocarcinoma) Identifies intranodal V/s perinodal metastasis Identifies multilevel disease (poor prognosis) Is required by many neoadjuvant and adjuvant protocols Is a part of a complete resection

CT Staging of the Mediastinum • Current imaging techniques determine size not histology

Malignant mediastinal nodes are not larger than benign lymph nodes (58% > 15 mm benign)

• Small mediastinal nodes (< 10 mm) are not infrequently malignant (15%)

• Benign adenopathy is more common in patients with acute pulmonary inflammation

• Pathologic confirmation rates higher than radiologic estimation • CT identification of enlarged hilar/mediastinal nodes is not

diagnostic of advanced stage disease. CT, therefore adds an extra cost without contributing to the management plan

Survival after Surgical Resection in Lung Cancer Survival after Surgical Resection in Lung Cancer Stage IA (511) Stage IB (549) Stage IIA (76) Stage IIB (375) Stage IIIA (399)

- 67% - 57% - 55% - 39% - 23%

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Factors influencing survival in N2 disease 1. Multiple levels of involvement 2. Nodal vs extranodal disease 3. Superior vs inferior mediastinum 4. Bulky clinical vs discrete CT nodes 5. Recurrences - 80% within 2 years 6. Second primary 3-4% per year, especially in high risk patients Manifestations of Preoperative Inoperability • Distant metastases (absolute) • Malignant pleural effusion (absolute) • Superior vena caval syndrome • Horners syndrome • Vocal cord paralysis • Phrenic nerve paralysis

Miller JD, Gorenstein LA, Patterson GA. Staging: The key to rational management of lung cancer. Annals of Thoracic Surgery 1992;53:170-8.

Nesbitt JC, Putnam JB Jr, Walsh GL, Roth JA, Mountain CF. Survival in early-stage non-small cell lung cancer. Annals of Thoracic Surgery 1995;60:466-72.

Schreurs AJ, Westerman CJ, van den Bosch JM, Vanderschueren RG, Brutel de la Riviere A, Knaepen PJ. A twenty-five-year follow-up of ninety-three resected typical carcinoid tumors of the lung. Journal of Thoracic and Cardiovascular Surgery 1992;104:470-5.

Tedder M, Anstadt MP, Tedder SD, Lowe JE. Current morbidity, mortality, and survival after bronchoplastic procedures for malignancy. Annals of Thoracic Surgery 1992;54:387-91.

Warren WH, Faber LP, Gould VE. Neuroendocrine neoplasms of the lung. A clinicopathologic update. Journal of Thoracic and Cardiovascular Surgery 1989;98:321-32.

Neoadjuvant Therapy for Lung Cancer Neoadjuvant therapy has been successful in anal, bladder and esophageal cancers. Head and neck cancers do not respond to neoadjuvant therapy

Rationale for neoadjuvant therapy Surgical resection disrupts blood supply and adjuvant therapy may not be deliverable Preoperative therapy may minimize seeding Preoperative therapy may accomplish downstaging Tumor growth is inversely related to size. Micrometasteses grow faster Chemotherapy related killing follows first order of kinetics Goldie Coldman hypothesis - with each cell division cells become resistant due to continued mutation Completed Neoadjuvant Therapy Trials in Stage II and III NSCLC

Study Therapy No. of Patients

Overall Survival P value

Roth et al, 1994

CT+S+CT/S 60 <.008

Rosell et al, 1994

CT+S+RT/S+RT 60 <.001

Yoneda et al, 1995

CT+RT+S/S 83 NS

CALGB, 1997

CT+S+CT+RT/RT+S+RT 57 NS

Steven M. Keller, MD (1998) Current Neoadjuvant Therapy Trials

Study Therapy Accrual Goal

Eligibility

RTOG 9309

CT+RT+S+CT/CT+RT+CT 510 Stage IIIA (T1-3N2)

EORTC 08941

CT+S/CT+RT 812 Stage IIIA (N2)

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MRC LU20

CT+S or CT+RT/RT 350 Stage IIIA (T3N1, T1-3N2)

MRC LU22

CT+S/S 450 Resectable disease

Despierre et al

CT+S/S 406 Stage I-IIIA

Steven M. Keller, MD (1998) Conclusions from randomized trials Well tolerated with high response and resectability rates (70%) Trend towards increased disease free survival and even overall survival I believe some form of neoadjuvant therapy is the best we have to offer today and recommend it to every patient with N2 disease

Chest Wall Invasion of Lung Cancer

• Incidence of about 5% • Does not imply a hopeless prognosis • Mortality and morbidity for surgical resection are acceptable • Extensive resections possible

Bronchial Adenomas Long natural histories 5% of all primary pulmonary neoplasms Two groups

Carcinoid tumors Bronchial mucous gland tumors

Most are not benign adenomas but are malignant neoplasms

Classification Carcinoid tumors Typical Atypical Bronchial gland tumors Adenoid cystic carcinoma (Clindroma) Mucoepidermoid carcinoma Bronchial gland adenoma

Carcinoid Tumors APUD tumor Mostly in lobar bronchi (70%); others quote higher incidence in periphery or lung More frequent on R. and in lower lobes "Mulberry" lesion Small cell uniform tumor cells in a vascular stroma Atypical carcinoid 10-15%

Pleomorphism Nuclear irregularity Increased mitotic activity

85-90% or bronchial adenomas Neuroendocrine tumor from Kulchitsky's cells (argentaffin)

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Atypical carcinoid More active biologic behavior 50-60% lymph node metastases (5-10% with typical carcinoids) 10% distant metastases Poorer prognosis Most patients- 5th to 6th decade; slight female predominance 25-30% asymptomatic- present with abnormal CXR Sx: Cough, hemoptysis, recurrent pneumonitis

Carcinoid syndrome- 1-2%; usually associated with metastases Cushing's syndrome; hyperinsulism

60-80% seen bronchoscopically: Biopsy (?)

Treatment Resection: lobectomy, pneumonectomy Resect recurrent and metastatic disease Bronchoscopic resection- high rate of recurrence Ten year disease-free interval

Typical carcinoids- 85-90% Atypical carcinoids or typical carcinoids with lymph node metastases- 50%

Bronchial Gland Tumors

Adenoid Cystic Carcinoma (Cylindroma) Occurs in trachea 30% lymph node metastases Submucosal and perineural lymphatic spread Sx: cough, hemoptysis, wheezing, obstructive pneumonitis in 6th decade Tx: wide surgical excision with lymph node dissection- RT palliative

50% long-term survival with wide surgical resection

Mucoepidermoid Carcinoma

Epidermoid and mucous producing elements Behavioral correlates with microscopic appearance Tx: Complete resection Prognosis

Low-grade: good with adequate resection High-grade: poor despite therapy

Rare; mainly in lobar/ mainstream bronchi

Mixed Tumors

Rare; similar to salivary gland tumors Require tracheal reconstruction Bronchial Mucous Gland Adenomas

Rare; benign; conservative resection

Lung Cancer Staging

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I. Staging Process

A. Histology - small cell vs.. non-small cell

B. Sputum 1. 20- 70% sensitive, but tumor location plays a significant role 2. histology is predictive of yield, i.e. squamous is more often positive followed by adenocarcinoma, and finally small cell 3. when cytology is positive it predicts the cell type with 85% accuracy

C. Bronchoscopy 1. direct visualization or positive biopsy in 25-50% of patients with lung cancer

D. Fine needle aspiration 1. percutaneous or transbronchial 2. 84-95% accurate with peripheral lesions

E. VATS

F. Thoracotomy

II. Staging Classification

A. International Staging System for Non- Small Cell Carcinoma 1. T Primary Tumor Tx - positive cytology only To - no evidence of tumor Tis - carcinoma in situ T1 - size < 3 cm no pleural invasion distal to lobar bronchus T2 - size > 3 cm any size invading the visceral pleura associated atelectesis or pneumonitis to the hilum >2 cm from the carina T3 - any size with chest wall, diaphragm, mediastinal pleura, or pericardium, (i.e. locally metastatic to resectable ipsilateral hemithorax) < 2 cm from the carina T4 - invasion of the mediastinum, heart, great vessels, vertebral body, esophagus, or carina

2. N Nodal Involvement N0 - no nodes N1 - peribronchial or ipsilateral hilar N2 - ipsilateral mediastinum or subcarinal N3 - any contralateral node ipsilateral supraclavicular or scalene nodes

3. M Distant Metastasis

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Mo - no mets M1 - distant mets

B. Small Cell Carcinoma

1. localized - disease of the ipsilateral hemithorax including the supraclavicular nodes and a positive pleural effusion 2. extensive - disease beyond the ipsilateral hemithorax

III. Clinical Presentation A. Sputum 1. bronchopulmonary 2. extrapulmonary intrathoracic 3. extrapulmonary metastatic 4. extra pulmonary nonmetastatic ( i.e. paraneoplastic) a. carcinomatous neuromyopathy is the most common paraneoplastic syndrome with 15% of patients with lung cancer affected 1) mysthenia gravis - like syndrome 2) polymyositis b. Cushing's - small cell c. SIADH - small cell d. hypercalcemia - squamous e. gynecomastia - small cell f. Gonadotropin - undifferentiated large cell

B. Signs 1. clubbing is the most common 2. Hypertrophic pulmonary osteoarthropathy a. periosteal elevation at the ends of long bones b. 2-12% of all lung cancer patients c. not seen in small cell

IV. Tumor Makers/ Oncogenes 1. generally not help for diagnosing lung cancer

V. Diagnostic Evaluation

A. CXR - CXR findings proceed symptoms by 7 months - sensitive to 1 cm - nodule most common finding 1. squamous a. obstructive pneumonitis b. collapse c. consolidation d. 1/3 are peripheral e. 20% have cavitation

2. adenocarcinoma a. peripheral b. < 3 cm c. bronchoalveolar have parenchymal changes 3. Large cell (undifferentiated) 1. 60% are peripheral

Selected Articles Miller JD, Gorenstein LA,

Patterson GA. Staging: The key to rational management of lung cancer. Annals of Thoracic Surgery 1992;53:170-8.


Textbook Chapters Chapter 19, Section I: Neoplasms of the Lung - Carcinoma of the Lung. Surgery of the Chest (Sabiston and Spencer), 6th ed., 635-49.

Chapters 21-7: Lung Carcinomas, Diagnosis and Staging of Lung Cancer, Surgical Treatment of Lung Carcinoma, Superior Sulcus Tumors, Limited Pulmonary Resection, Bronchoplastic Techniques for Lung Resection, Multimodality Therapy of Carcinoma of the Lung: Irradiation, Chemotherapy,

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2. 2/3 > 4 cm

4. small cell 1. 80% hilar abnormalities 2. 2/5 associated parenchymal changes

B. Other Studies 1. CT a. best for evaluating the mediastinal adenopathy and adrenals b. chest wall invasion is poorly seen c. paraesophageal and inferior pulmonary nodes not well seen d. nodes < 1 cm have a 7% chance of being malignant e. nodes > 1 cm have a 55-65% chance of being malignant 2. MRI a. better than CT at evaluating vascular invasion and chest wall invasion esp. superior sulcus 3. Ultrasound a. ? TEE for evaluating mediastinal adenopathy 4. PET a. may help determine malignant vs. benign peripheral nodules 5. Bone Scan a. helpful in stage IIIA and IIIB disease

and Immunotherapy. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 357-498.

VI. Lymph Nodes

A. Biopsy 1. biopsy palpable neck nodes 2. mediastinoscopy is controversial

B. FNA 1. 85-95% sensitive

C. VATS 1. good for evaluating aortopulmonary window

VII. Completion of Staging

-25% of patients worked up will be resectible -25% will have stage IIIB -50% will have stage IV

VIII. Posthoracotomy provides the definitive stage and should be the basis of treatment plans

IX. Functional Status

A. Associated with prohibitive operative risk 1. FEV1 < 40% 2. Predicted postop FEV1 < 30% 3. MVV < 45-50% 4. DLCO < 40% 5. PCO2 > 45 mmHg

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6. peak VO2 < 10 ml/kg

. Oncogenes in Lung Cancer I. Classification A. Dominant oncogenes 1. Alter one allele of a gene 2. Mutation results in overproduction of a protein or loss of regulatory function of protein production 3. Both result in uncontrolled cell growth and division B. Tumor-supressor anti-oncogenes 1. Mutation on two alleles of a gene 2. Lose ability to control growth 3. Results in malignant transformation of the cell 4. Include inherited retinoblastoma

II. ras A. Rat sarcoma B. Expression frequently enhanced in pulmonary neoplasm (all histologic types, especially NSCLC)

III. myc A. L-myc (homolog found in lung cancer), N-myc, c-myc, etc. B. Expression is associated with uncontrolled proliferation C. Cells express only one homolog at a time D. SCLC - Three levels of expression:

1. Low 2. Medium - amplification from chromosomal duplication 3. High - gene amplification E. Enhanced expression 1. Associated with treatment (chemo-tx), and with shortened survival 2. Probably a secondary event

IV. A. Supressor gene B. Found in many types of carcinoma C. Mutations found frequently in lung cancer cell lines

V. HER -2/neu (c-erb-2) A. Encodes for protein

1. Transmembrane protein related to tyrosine kinase activity 2. Significant homolgy with epidermal growth factor receptor

B. Expression in adenocarcinoma associated with ¯ survival C. expressed more frequently in NSCLC D. Radiolabeled monoclonal antibody may help image tumor

Gene SCLC NSCLC Oncogene myc 10% rare ras rare 20%

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Anti-oncogenes gene not yet identified

100% 50-80%

rb gene 100% 15% gene 100% 47%

Sugery for Lung Cancer Introduction Lung carcinoma is the second most common form of cancer in the U.S. and is the leading cause of death in men 1. (33% of cancer related deaths) 2. Lung cancer is the most common cause of death in women (23%) breast ca (18%) 3. Overall cure rate is 10-13 % of patients at 5 years 4. 1909 intratracheal anesthesia was introduced 5. Graham and Singer in 1933 reported the first successful pneumonectomy for lung carcinoma 6. In 1950, Churchill proposed that a lobectomy could be effective in the resection of lung carcinomas

Lung Cancer Study Group randomized 247 patients to compare segmentectomy versus lobectomy in the treatment of lung cancer a. for segmentectomy the local recurrence rate was 17.2 % versus 6.4 % for lobectomy b. 5 year survival was 50 % for segmentectomy versus 68 % for lobectomy 1. for incomplete resection for bronchogenic carcinoma the 5 year survival is 4 % 2. mediastinal lymph node dissection should be included in the resection

a. important for pathological staging b. adds minimal time and morbidity to the procedure c. compartments include :

i. superior mediastinal ii. A-P window iii. subcarinal and inferior mediastinal

Surgical Treatment of Occult (TX NO MO) Non- Small Cell Lung Carcinoma 1. Low incidence, 1.5% in Memorial Hospital experience 2. these are individuals who participate in early screening programs and submit sputum for cytological analysis on a routine basis, or have hemoptysis with normal CXR 3. 33 % of patients with positive sputum cytology and negative CXR will have head and neck cancer 4. detailed head and neck examination is important in the diagnosis 5. bonchoscopy

a. careful examination required b. endobronchial brushing of each segmental bronchus required c. fluorescent staining is possible with parenterally administered hematoporphyrin derivative

Treatment f. photoablation has been described (3 yr survival 50- 70 %)

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g. surgical resection is the treatment of choice h. local recurrence is low, median survival is 9 years i. close follow up is required since up to 45 % of patients will develop a second primary, most of which will be airway carcinomas

Surgical Treatment of stage I (T1 N0 M0, T2 N0 M0) Non-Small Cell Lung Carcinoma 1. 20% of patients 2. includes patients with tumors:

a. 3 cm size or less surrounded by lung or visceral pleura, without extension proximal to a lobar bronchus b. tumors > 3 cm, or tumor of any size that invades visceral pleura or has associated atelectasis extending to the hilum, and is >2 cm distal to carina (T2) c. Have no nodal metastases (N0 M0)

3. Staging c. Patients should be preoperatively and intraoperatively staged d. Pre-op includes H&P, LFT’s, CXR, CT scan e. Controversy exists over pre-op routine bone and brain scans for asymptomatic patients f. Intra-op node dissection g. Prior to 1986, the TNM classification included T1N1 tumors under stage 1, therefore reported overall survival was lower

Results of surgery Overall reported 5 year survival is 50-85% (82-85% T1, 67-68% T2) Histology not a prognostic factor in survival Close follow up required since recurrence rate is 27-39%

i. 60% recur within 2 years and 91% in 5 years ii. 34% develop second primaries: 33% lung, 16% breast, 13% head and neck, 8% colorectal, 7% bladder

Adjuvant therappy not warranted Some evidence to suggest Vitamin A supplementation may have an effect on lowering the incidence of second primary tumors

Surgical Treatment of Stage II (T1N1M0, T2N1M0) Non-Small Cell Lung Carcinoma 1. 10% of patients 2. Includes patients with tumors: a. Primary tumors confined to the lung and >2 cm distal to carina, with metastases to peribronchial or ipsilateral hilar lymph nodes b. Treatment is lobectomy, bilobectomy or pneumonectomy with MLND Results of surgery Overall survival is 39-49% 5 year Prognostic factors include size of primary and number of metastatic lymph nodes Recurrence rate in one study (Martini et al) was 55% with 21% loco-regional and 79% distant No adjuvant therapy has improved survival Postoperative radiotherapy has been shown to decrease locoregional recurrence rate

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Adenocarcinoma tends to recur distally more often while SCCA tends to recur locally Surgical Treatment of Stage IIIA (T3 N0-1 M0, T1-3 N2 M0) Non-Small Cell Lung Cancer 1. Includes patients with tumors: a. With limited, circumscribed extrapulmonary extension of the primary tumor (T3) b. And/or metastases confined to the ipsilateral mediastinal of subcarinal lymph nodes (N2)

Surgical Treatment of T3 (Chest Wall Invasion) Non-Small Cell Lung Carcinoma (exclusive of superior sulcus tumors) 1. 5% tumors invade parietal pleura and chest wall 2. Surgical treatment

a. Includes pulmonary resection with contiguous soft tissue and rib resection and chest wall reconstruction b. When peripheral tumors is attached to parietal pleura, extrapleural resection can be attempted with good success or en bloc resection will be required c. Marlex mesh and methylmethacrylate can be utilizied for reconstruction d. Overall operative mortality is 4-12% e. Overall 5 year survival is 26-40% f. Lymph node status and depth of invasion correlates with survival g. Most important prognostic factor is whether a complete resection can be performed

Surgical Treatment of T3 (Proximity to carina) Non-Small Cell Lung Cancer 1. Lesion within 2 cm of carina Treatment includes a. Pneumonectomy b. Sleeve lobectomy c. Sleeve pneumonectomy 1. Most important diagnostic procedure is bronchoscopy in order to determine proximity of the tumor to the carina 2. Results of surgical treatment a. Sleeve lobectomy

i. Overall mortality 0-% ii. Overall 5 year survival is 30-64%

a. Sleeve pneumonectomy ii. Overall mortality 4-27% iii. Overall 5 year survival 16-23% iv. Indication is for bulky tumors in proximity to or involving the carina or tracheobronchial angle v. Major complication is anastamotic dehiscence with a mortality of 100%

Surgical Treatment of N2 Disease (Mediastinal Lymph Node Metastases) in Patients with Non-Small Cell Lung Cancer 1. 45% of presenting patients 2. Overall 5 year survival is 20-30%

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3. Memorial Sloan Kettering experience (1974-1981 with 1598 patients) a. 151 cases completelly resectable b. Post-operative XRT used in 90% patients c. Mediastinoscopy not routinely performed d. 79% underwent lobectomy, 17% pneumonectomy and 4% segmentectomy e. Overall 5 year survival was 30% f. No difference in survival between SCCA or adenocarcinoma g. Patients presenting with obviouos N2 disease had poorer survival h. Number of nodes affected survival, upper paratracheal nodes affected survival with an overall 5 year survival of 20% i. 73% patients developed recurrent disease 1. Adjuvant therapy d. Lung Cancer Study Group

i. Stage II and III patients ii. Found that post-operative radiotherapy significantly decreased local recurrence but no affect on survival iii. Also randomized patients with adenoca and large cell ca to receive postop BCG and levamisole vs. Chemotherapy and found increased disease free survival in those patients that received chemotherapy a. Stage IIIA patients ii. Three randomized trials of preoperative chemotherapy

plux surgery vs. Surgery alone revealed survival benefit in patients receiving preoperative chemotherapy with a response rate 60%

Surgical Treatment of Stage IIIB Non- Small Cell Lung Cancer

Surgical Treatment of T4 (pleural Effusion) Non-Small Cell Cancer 1. patients with malignant pleural effusions 2. if cytologically negative, the effusion is excluded as staging element and is staged asT1, T2 or T3 lesion 3. evaluation of patients with pleural effusions

a. Thoracentesis b. thoracoscopy- perform if thoracentesis cytologically negative fluid

i. if pleural metastases is found then patient is non-operable (T4) ii. if no pleural metastases are found then the patient is an operable candidate (only 6 % of patients)

1. median survival for patients with malignant pleural effusions is 6 months 2. tube thoracostomy , pleurodesis, VATS may be required to control recurrent pleural effusions

Surgical Treatment of T 4 (Mediastinum) Non-Small Cell Lung Cancer 1. patients with tumors of any size invading heart, great vessels, trachea, esophagus, vertebral body or carina

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2. most patients are considered inoperable if biopsied pre-operatively or are found unresectable intra-operatively 3. MSKCC experience

c. 225 patients in their review d. overall survival 22% at 2 years, 13% at 3 years and 7% at 5 years e. therapy was either complete resection, incomplete resection with brachytherapy, brachytherapy alone of incomplete resection alone d. 5 year survival for patients that underwent complete resection was 9 %

4. patients should receive pre-op chemotherapy if possible in attempt to down stage the tumor 5. a small study by Macchiarini (23 patients) utilizing induction chemotherapy with patients with T4 tumors demonstrated a 3 year survival of 54%

Surgical Treatment of Stage IV (T1-4, No-2, M1) Non-Small Cell Cancer 1. this includes any patient with distant metastatic disease (M1) 2. small role for surgical therapy limited to patients with solitary brain metastases

a. if both lesions are resectable (brain metastases and lung tumor) then craniotomy should be performed followed by thoracotomy b. patients who receive post-operative whole brain radiation have an improved median survival 9.2 months vs. 3.4 months

The Problems of Surgical Therapy For Small Cell Carcinoma 1. in general is not a surgical disease 2. surgery indicated in only a small number of patients 3. it is usually discovered intra-operatively in a patient with presumed non-small cell cancer 4. if at thoracotomy the diagnosis of stage I small cell cancer is made, then complete resection should be attempted 5. the VA Surgical Oncology Group demonstrated 5 year survival rates of 60% for T1N0 and 31% for T1N1 disease (54% received post-op chemotherapy) 6. post-operative chemotherapy is recommended 7. patients with stage II or III are best served by chemotherapy and radiation

Supeior Sulcus Tumors Definition a bronchogenic carcinoma located in the extreme apex of the lung

Pancoast Tumor (Superior Sulcus Tumor)

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which invades the pleura and adjacent structures and produces classic symptoms and signs. The presenting symptom most frequently cited is pain localized to the shoulder. If L. untreated the pain becomes unremitting and spreads medially to the scapula, extends along the ulnar nerve distribution of the arm to involve the elbow, forearm and hand. Other involved structures include the cervical sympathetics (Horner’s syndrome), vagus and phrenic nerves, carotid artery, and the vertebral bodies. Squamous cell is the most common cause followed by adenocarcinoma and large cell. Small cell is rare

· Primary lung cancer which arises from the apex of the lung · Structures susceptible to invasion include pleura, brachial plexus, sympathetic chain, ribs, and vertebra · The Pancoast syndrome, or superior sulcus tumor syndrome, is characterized by ipsilateral shoulder/arm pain · Horner's syndrome (ipsilateral anhidrosis, ptosis, enopthalmus) develops with invasion of sympathetic nerves

Location All are T3 since they invade the chest wall; classified as T4 when mediastinal and/or cervical invasion has taken place. Posterior—stellate ganglion, posterior ribs, brachial plexus (upward extension), and vertebral bodies (medial extension) Anterior—1st rib, scalene muscle, subclavian vessels, phrenic nerve Resection possible even with brachial plexus, stellate ganglion, rib, transverse process, subclavian artery (adventitia), vertebral body (<25%). Mediastinal invasion (vena cava, vertebral foramina, vagus nerve) precludes cure Nodal involvement is the key to potential curability once resectability is established—lymph node involvement is usually late in these tumors

MSK series--129 patients 109 patients—negative mediastinal nodes median survival—20 months 5yr—29% 20 patients—positive mediastinal nodes median survival—9 months 5yr—10% Paulson—131 patients

78 patients resected (pre-op RT)—5yr survival 44% 17 patients with positive lymph nodes—0% survival at 2 years Radiologic evaluation —usual CXR finding mass in apex clouding the lung markings above the clavicle when contrasted to the clarity of the opposite side; however, may only resemble pleural thickening. Bony destruction may be apparent. CT will identify involvement and invasion of the brachial plexus, the chest wall, vertebral bodies, vena cava, trachea, esophagus, and the subclavian vessels. Will also depict lymph node involvement MRI is recommended to delineate the extent of cervical invasion and some consider routine in the preoperative evaluation.

Operative approach Posterior Incision follows the contour of the scapula

Enter pleura 3rd or 4th intercostal space Vascular structures identified Brachial plexus involvement identified and resected Vertebral bodies are assessed

Treatment of Pancoast Tumor · Preoperative radiation therapy followed by operative resection 4 weeks later (Paulson protocol) · Contraindications include extensive involvement of the brachial plexus, subclavian artery, or vertebrae; SVC or recurrent

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Lobectomy performed Along with the possibility of usual complications, one other possibility is spinal cord leakage that may lead to meningitis or pneomoencephally—from air leaks and causes severe headaches

Anterior transcervical approach Usually combined with the posterior approach Sternocleidomastoid incision Scalene fat pad dissection Clavicular resection Vein dissection Jugular and subclavian veins freed—exposure of thoracic duct and vertebral veins facilitated Arterial dissection Subclavian, IMA, thyrocervical trunk, vertebral artery Nerve dissection Brachial plexus Overall, major advantage is the ability to deal with the invasion of the subclavian vein and related structures. It is not effective for tumors that invade the posterior aspects of the ribs and their transverse processes, the stellate ganglion and sympathetic chain, and the vertebral bodies.

laryngeal nerve involvement; distant metastasis; and mediastinal node involvement (relative)

Radiation therapy Primary therapy for unresectable or inoperable patients Excellent for pain relief No long term survival if primary tumor not controlled Most common site of recurrence is the brain—consider prophylactic cerebral RT if local control achieved and histologic dx is adeno or large cell

PRE-OP RT MSK—126 patients; 100 resected; 117 pre/post op RT; 102 brachytherapy 69 complete resection (49 of these had brachytherapy) 22 had lobectomy; 47 large wedge resection 5yr survival—60% for lobectomy; 33% for wedge resection Intraoperative brachytherapy had no influence on loco-regional recurrence or survival in patients with completely resected tumors Adverse prognostic factors—Horner’s syndrome, N2, N3, and vertebral body invasion This series indicates that pre operative RT is useful in patients when combined with lobectomy. Unresectable disease should be treated with external RT. +/- Intraoperative brachytherapy in patients who are explored but incompletely resected

POST OP RT Not indicated in patients who are completely resected and have no nodal metastasis. There are some retrospective studies that show benefit in patients with nodal disease, however the LCSG showed no survival benefit in completely resected patients—it did decrease the incidence of local (intrathoracic) recurrence MSK—Post operative RT following immediate operation and brachytherapy was as effective as pre-op RT and brachytherapy in achieving complete resection, loco-regional control, and ultimate curability There are no studies documenting the usefulness of chemotherapy in this disease.

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Solitary Pulmonary nodule Mnemonic: Apical Lung Disease “SET CARP” S: Sarcoid E: EG, Eosinophilic pneumonia T: Tuberculosis C: Cystic Fibrosis A: Ankylosing spondylitis R: Radiation therapy P: Pneumoconiosis, PCP (cystic) Mnemonic: “CARPETS” C: Cystic fibrosis A: Ankylosing spondilytis R: Radiation therapy P: Pneumoconiasis E: Eosinophilic pneumonia T: TB S: Sarcoid

Mnemonic: Solitary Pulmonary Nodule (SPN) “CASH PLEASE” C: Cancer A: Abscess S: Solitary met H: Hamartoma P: Psuedotumor L: Lymphoma E: Echinococcus A: Actinomycosis S: Sequestration

Pulmonary Metastases (Mets) Pathogenesis · The lung is the first capillary bed draining most primary sites, with tumor cells usually depositing in the periphery · 10-20% of patients with pulmonary metastases have disease confined to the lungs (especially with sarcomas)

Mnemonic: Carcinomas that have a Propensity to Metastasize to Bone (7) "KINDS OF TUMOURS LEAPING PROMPTLY TO BONE" Kidney, Ovary, Testis, Lung, Prostate, Thyroid, Breast “BE PATIENT TO BE KIND” B: Breast P: Prostate, Pulmonary T: Testis, Thyroid B: Bone K: Kidney

Diagnosis · CT scanning is sensitive but not specific, and may underestimate the number of malignant nodules · Needle biopsy rarely adds additional information · Sarcomas and melanomas are the most likely to cause a solitary metastasis

Patient Selection · There are four criteria which should be met prior to resection of pulmonary metastases: 1. Resection should only be performed if removal of all disease is possible 2. The patient must have adequate pulmonary reserve to tolerate resection 3. Local control of the primary tumor 4. Absence of metastases elsewhere in the patient

Mnemonic: Calcifying Metastases “BOTTOM ” B: Breast O: Osteogenic carcinoma TT: Thyroid (papillary) O: Ovarian M: Mucinous adenocarcinoma

Prognostic Factors · Histologic cell type affects the pattern of metastasis as well as outcome · Tumors with longer doubling time have better survival · The number of metastases, the disease-free interval, and unilateral vs. bilateral disease are not prognostically significant · Complete resectability is the most important indicator of improved survival

McCormack PM, Bains MS, Begg CB, Burt ME, Downey RJ, Panicek DM, Rusch VW, Zakowski M, Ginsberg RJ. Role of video-assisted thoracic surgery in the treatment of pulmonary metastases: Results of a prospective trial. Annals of Thoracic Surgery 1996 62(1):213-7.

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McAfee MK, Allen MS, Trastek VF, Ilstrup DM, Deschamps C, Pairolero PC. Colorectal lung metastases: Results of surgical excision. Annals of Thoracic Surgery 1992 53(5):780-5.

Staren ED, Salerno C, Rongione A, Witt TR, Faber LP. Pulmonary resection for metastatic breast cancer. Archives of Surgery 1992 127(11):1282-4.

Sources for further reading Textbook Chapters Chapter 19, Section V: Neoplasms of the Lung - Surgical Management of Pulmonary Metastases. Surgery of the Chest (Sabiston and Spencer), 6th ed., 669-75.

Chapters 25 and 28: Superior Sulcus Tumors and Indications for Resection of Pulmonary Metastases. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 445-58 and 499-510.

Operative Technique · Wedge resections should be performed wherever possible to preserve parenchymal tissue · Manual exploration is preferred to thoracoscopic examination to identify all nodules · Bilateral disease may be treated either by staged bilateral thoracotomy or median sternotomy for a single operation Results · Outcomes vary according to primary tumor type

Tumor type 5-year survival

Soft tissue sarcoma 25% Osteogenic sarcoma 20-40% Colon/rectal carcinoma 8-37% Renal cell carcinoma 13-50% Breast carcinoma 14-49% Head/neck carcinoma 40-50% Melanoma 25%

Benign Respiratory Tract Tumors Respiratory Tract Tissue examination Most lesions are peripheral Radiographic features- major diagnostic aid

Calcification "Popcorn" type Well defined margins Lobulated Growth (?) Wedge resection Frozen section

Tumors of Epithelial origin

Papilloma- 5 sib-classifications Solitary benign papilloma Multiple benign papillomas Combined bronchial mucous gland and surface papillary tumor In situ papillary bronchial carcinomas Bronchiolar papillomas

Proximal Squamous, stalk

Distal Clara cells

One of few lesions that can be managed by bronchoscopic resection Recurrence is high Rare malignant transformation

Epithelial tumors Mesodermal tumors Vascular tumors Bronchial tumors Neurogenic tumors Developmental or unknown origin tumors Inflammatory and other pseudo0tumors

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Polyps

Related to inflammatory polyps or URT Squamous metaplasia; ciliated columnar epithelium and granulation tissue Bronchoscopic removal Tumorlet

Epithelial proliferation Originates from Kulchitsky cells Multifocal: < 4 mm; associated with pulmonary fibrosis

Form of peripheral carcinoid with rare metastases

Tumors of Mesodermal Origin

Hemangioma Subglottic area of larynx or upper trachea of infants Airway obstuction Dx: Bronchoscopy Other vascular lesions of skin, mucous membranes Tx: Radiation therapy

Lymphangioma Upper airway obstruction in infancy Associated with other lesions- cystic hygroma, hemangioma in the neck Tx: surgical excision

Endothelioma

More solid Lymphatic or vascular origin

Angiomatous formation- may ne found only in lung Most often described in newborns Lethal course over a short period or time Lymphangiomatosis

Rare- may be related to tuberous sclerosis Slowly progressive- death within 10 years from pulmonary insufficiency Young females presenting with dyspnea Spontaneous PTX; chylothorax, hemoptysis

CXR: fine, multinodular lesions in bases- honeycombing

A-V Fistula

Lower lobes Associated with hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome) in 50% of cases R > L shunting PA-PV

Cyanosis Clubbing Polycythemia DOE Pulmonary murmur

Brain abcess or peripheral embolization Dx: CXR,; pulmonary angiogram Tx: Surgical resection; angiographic embolization A-V Fistula

Sclerosing Hemangioma

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Uncommon; originates from Type II pneumocyte precursors Coin lesion, lower lobes Hemoptysis Associated with hemorrhagic telangiectasia

Intravascular Bronchoalveolar Tumor Rare; females < 30 years old Originates from vascular endothelium CXR: multiple pulmonary nodules- incidentally discovered Mild dyspnea Stable, slowly growing metastases reported Tumor considered to be malignant

Hemangiopericytoma

Solitary, encapsulated, asymptomatic Originates from pericytes associated with pulmonary capillaries Considered malignant Tx: Surgical resection for cure or radiation therapy for palliation

Fibroma

Mostly tracheobronchial in origin Most common benign tumor of mesodermal origin in adult and pediatric age group Collagenous/ spindle cells- myxomatous/ adipose elements Tx: Bronchoscopic resection if stalk is present vs conservative pulmonary resection

Chondroma and Osteochondroma

Arise in major bronchi May appear as true enchondrosis of bronchial cartilage Firm, translucent; ossified/ calcified material Second most common mesodermal tumor Carney's triad: associated with extra-adrenal paragangliomas and gastric leiomysarcomas

Lipoma

Rare, intrabronchial lesion; male predominance Slowly growing, avascular, obstructive, pedunculated Tx: Bronchoscopic removal for small lesions; bronchotomy for larger ones Arise in fat cells Associated with bronchiectasis (chronic obstruction)

Leiomyoma

Fourth in order of frequency Originates from smooth muscle cells

Bronchiolar smooth muscle cells Pulmonary vessels

Sx: cough, hemoptysis, pneumonia, bronciectasis

Order of Frequency of Mesodermal Tumors Fibroma Chondroma Lipoma Leiomyoma

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50% intrabronchial- 50% peripheral Can be associated with similar cutaneous lesions Tx: Conservative surgical approach

Malignant variant: Leiomyosarcoma Granular Cell Tumors (Myoblastoma)

Previously thought to originate from myoblasts Originates from Schwann cells or histiocytes Arises from the tongue or skin 6% originate endobronchially Tx: Surgical removal with wide margins

Bronchoscopic removal associated with recurrence < 8 mm

Developmental or Unknown Origin

Hamartomas Most common benign tumor of the lung 8% of coin-shaped lesions 0.25% of general population CXR: "Popcorn" lesion Malignant transformation- rare True neoplasm Malignant transformation of the epithelial component

Chondromatous hamartoma Adenomatous hamartoma of infancy Pulmonary blastoma

Teratoma

Rare: 50% are benign Characteristics of all germ layers Must be differentiated from metastatic lesion

Testicle Direct invasion from mediastinum

Chemodectoma

One reported case Arise from chemorecptor cells in the lung

Clear Cell (sugar) Tumor

Resembles hypernephroma Abundant glycogen content Characteristics of Type II glycogen storage disease

Thymoma

Ectopic thymus tissue (rare) Intrapulmonary thymoma may be associated with Myasthenia Gravis

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Inflammatory and Other Pseudotumors

Plasma Cell Granulomas (Histiocytoma) Solitary pulmonary nodule May be associated with: systemic disease, plasma protein imbalance, NS local inflammatory reaction Micro: Plasma cells and lymphocytes Local exclusion/ lobotomy- cure May be associated with multiple myeloma or other malignancies Do not confuse with plasmacytoma- a malignant lesion

Pseudolymphoma

Discrete localization Unilateral Resembles lymphoid interstitial pneumonitis Rare conversion to malignant lymphoma Tx: Lobectomy/ segmental resection with follow-up

Xanthoma

Post-inflammatory lesion- encapsulated Micro: foam cells Conservative resection

No reports of recurrence

Amyloid

Deposition may be diffuse of localized Occasionally may be obstructive Tx: Bronchoscopic removal/ resection

Tracheobronchopathia Osteoplastica

Multiple cartilaginous/ osseous projections into lower tracheal lumen Usually found at autopsy Rarely obstructive Tx: Bronchoscopic removal/ tracheal resection

Benign Tumors of the Lung 1. 5% of all tumors of the tracheobronchial tree 2. most are within lung parenchyma 3. usually asymptomatic unless associated with bronchial obstruction

Classification of benign tumors of the lung and

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tracheobronchial tree (see chart)

Clinical features

1. mode of presentation depends on location and size 2. most are peripheral and found on routine CXR 3. chronic cough and chest pain are most frequent symptoms (40%) 4. central lesions are more likely to be symptomatic 5. diagnosis

a. CXR, CT scan b. bronchoscopy for central lesions c. peripheral lesions- needle biopsy, thoracoscopy, open biopsy d. conservative resection is indicated unless there is a surrounding destructive process

Mnemonic: Interstitial Lung Disease Affecting Upper Lobe Lung "SHIT FACED" S: Sarcoidosis H: Histocotosis X I: Inflammatory T: Tuberculosis C: Cystic fibrosis A: Autoimmune E: Environmental dust D: Drugs

Tumors of epithelial origin

Papillomas of the tracheobronchial tree

1. most common laryngeal tumor in children 2. rare in adults 3. single or multiple lesions 4. subclasses

a. solitary benign b. multiple benign c. benign combined with bronchial mucous gland and surface papillary tumors d. papillary bronchial CIS e. bronchial papillomas

5. Approximately 50% of solitary bronchial papillomas are associated w/ lung carcinoma

Tumors of Mesodermal Origin

Hemangioma

1. infrequent in lung, more common in trachea and mainstem bronchi 2. multiple one third of cases 3. 60% of cases are associated with generalized hereditary telangiectasia (Oler-Rendu-Weber disease 4. surgical treatment is conservative resection, YAG laser for endobronchial lesions

Lymphangioma

Hemangioendothelioma

Hemangiopericytoma

1. may arise anywhere in the body 2. lesions are large and 50 % malignant 3. originate from capillary endothelium 4. centrally located, highly vascular 5. treatment is conservative surgical resection unless malignant

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Pulmonary Lymphangiomyomatosis

1. extensive hamartomas involving smooth muscle of the lungs, lyphatics, hilar,abdominal and lower cervical lymph nodes 2. progressive disorder of childbearing age females with diffuse interstial proliferation of smooth muscle in the lungs, lymph nodes and thoracic duct 3. lesions can produce SOB, emphysema, pneumothoraces, pulmonary hemorrhage with hemoptysis and chylothorax 4. treatment is conservative surgical resection

Bronchial Tumors

Fibroma

1. extremely rare 2. may arise from the peripheral parenchyma of the lung of from the walls of the trachea and bronchi 3. treatment is bronchoscopic removal or YAG laser therapy

Chondroma and Osteochondroma

Lipoma

1. least common benign tumors of the lung 2. may occur endobronchially or in parenchyma 3. 90% are in middle aged males 4. Typically present as solitary nodules

Granular Cell Myoblastoma

1. arise in tongue, skin, subcutaneous breast tissue and occasionally in the lungs 2. occurs in larger bornchi producing obstructive symptoms 3. high recurrence rate if not completely excised

Leiomyoma

1. primary neoplasm of muscular origin 2. sheets of smooth muscle cells predominate 3. rare

Tumors of Developmental or Unknown Origin Hamartoma

1. most common benign tumor of the lung- 75% of all benign tumors 2. constitute 80 % of all coin lesions 3. derived form embryological remnants 4. 90 % are located in the periphery 5. most commonly in subpleural position 6. conservative resection treatment of choice

Pulmonary Teratoma

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Chemodectoma

Inflammatory and other Pseudotumors of the Lung

Plasma cell Granuloma (Histiocytoma)

1. usually in younger patients 2. composed of plasma cells, lymphocytes and fibrous tissue 3. conservative surgical resection

Pseudolymphomas of the Lung

1. Presents as a solitary nodule that can mimic bronchogenic carcinoma 2. composed of lymphoid cells including mature lymphocytes and plasma cells 3. diagnostic feature is the presence of germinal centers 4. may lead to malignant lymphoma 5. long term follow up required

Amyloid Tumors

1. may be localized in bronchus or multiple or difuse bronchial deposits 2. can be a diffuse or localized parenchymal process 3. disease may eventually prove fatal 4. tumors are shelled out readily from surrounding parenchyma

Benign Pulmonary Disease Interstitial Lung Disease

A. Classifications: 1. Acute vs. chronic 2. Acinar vs. interstitial

B. Acute, Diffuse, Benign Pulmonary Disease 1. Acute infectious pneumonias 2. ARDS 3. Acute cardiogenic pulmonary edema 4. Vasculitic-immunologic syndrome

C. Chronic Diffuse Benign Pulmonary Disease 1. Sarcoidosis 2. Hypersensitivity pneumonitis (organic ducts) 3. Pneumoconiosis 4. Connective tissue diseases 5. Lung Irradiation 6. Drug-induced toxicity 7. Eosinophilic granuloma 8. Congenital (neurofibromatosis, tubular sclerosis) 9. Carcinoma with lymphatic spread 10. CHF

D. Interstitial Pneumonias 1. Affects the supportive structure of the alveolar walls and

Mnemonic: Bilateral Hilar Lymph-adenopathy (BHL) "PLEASE HELEN, LICK MY POPSICLE STICK." P: Primary TB H: Histoplasmosis L: Lymphoma M: Metastases P: Pneumoconiosis S: Sarcoidosis

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terminal airways 2. Symptoms: chronic dyspnea and nonproductive cough 3. PFT's: Restrictive disease, decreased DLCO, decreased compliance 4. Sarcoidosis is most common cause of interstitial pneumonia

E. Nomenclature: 1. UIP (Usual Interstitial Pneumonia) a. thickened alveolar wall with fibrosis and an inflammatory exudate 2. DIP (Desquamative Interstitial Pneumonia) a. large # of macrophages in the alveoli b. very responsive to steroids because underlying lung architecture is intact 3. LIP (Lymphocytic Interstitial Pneumonia) a. Lymphocytes accumulate around the bronchioles and blood vessels b. may have a monoclonal gammopathy which may be detectable via serum electrophoresis c. unpredictable response to steroids d. may go unto lymphoma of the lung e. other causes lymphocytic infiltrate AIDS, sarcoidosis, Sjogren's 4. GIP (Giant Cell Interstitial Pneumonia) a. look for exposure to organic dust oral pneumonias b. viral pneumonias 5. BO (Bronchiolitis Obliterans) a. variety of insults b. unresponsiveness to steroids c. associated with rheumatoid arthritis and connectiv tissue disorders 6. BOOP (Bronchiolitis Obliterans Organizing Pneumonia) a. plugs of fibrous tissue (Masson bodies) b. many associations but, most are idiopathic c. responsive to steroids

F. Hypersensitivity Pneumonitides 1. increase in T lymphocyte CD-8 (suppressor cell) in the lung secondary to inhalation of organic antigens 2. Biopsy: noncaseating granulomas typically around terminal bronchioles with a mixed inflammatory infiltrate

G. Inorganic Pneumoconiosis a. inhalation of nonorganic dusts and fibers b. three most common: Mnemonic: CSA 1) Silicone 2) Coal Dust 3) Asbestos

c. Asbestosis 1) characteristic pleural plaques in the chest wall and diaphragm on CXR 2) increases the risk of malignant mesothelioma 3) lung carcinoma increased synergistically with asbestos and

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tobacco 4) affects lower lobes/ pleural thickening 5) Biopsy: ferruginous bodies 6) long delay between exposure and onset of disease

H. Connective Tissue Diseases a. almost all connective tissue disorders have a pulmonary component b. Biopsy: does not aid in differentiating between diseases

I. Pulmonary Drug Toxicity a. Abx: Bleomycin, sulfa b. Alkylating Agents: Cyclophosphamide, Melophan c. Immunosuppressive: Immuran, Methotrexate d. Antiarrhythmic: Procainamide, Amiodarone(foamy cells) e. Diuretic: HCTZ f. Antihypertensitive: Hydralazine g. Opiates: Methadone, Heroin, Propoxyphene

J. Chronic Eosinophilic Pneumonia a. elements of interstitial & acinar infiltrates b. steroid response in the early phase c. diagnosis by blood Cx and biopsy with eosinopiles d. Biopsy Charcot Leyden crystals

K. Pulmonary Vasculitides & Granulomas a. rule out infection before beginning steroids

L. Idiopathic a. 20-30% of interstitial lung disease b Sx: dyspnea, cough, clubbing and arthralgias c. Bx: collagen deposition with inflammatory responsive d. 4- 5 yr. survival e. DIP pattern has a better response time

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7 Esophageal Diseases

Table of Contents

7 Esophageal Diseases .............................. 2 Anatomical Considerations .................................... 4

Developmental Anatomy .................................... 4 Surgical Anatomy ............................................... 4 Arterial Supply .................................................... 5 Venous Drainage ................................................ 5 Lymphatic Drainage ........................................... 5 Innervation .......................................................... 6 Nerve Supply (Nerves: central) .......................... 6

Physiological Considerations ................................. 6 Peristalsis ........................................................... 6 Normal function .................................................. 6 Esophageal Body ............................................... 7 Lower esophageal sphincter (LES) .................... 7

Esophageal Diagnostic Procedures ...................... 9 Radiographic Examples ..................................... 9 Esophagoscopy .................................................. 9 Endoscopic Ultrasound .................................... 12 Gastroesophageal Reflux Evaluation ............... 12

Congenital Esophageal Disease ......................... 12 Congenital esophageal stenosis ...................... 12 Esophageal atresia +/- TEF .............................. 13

Congenital Esophageal Abnormalities ................. 13 Esophageal Atresia .......................................... 13 Esophageal Atresia With Tracheoesophageal Fistula ............................................................... 14 Isolated Esophageal Atresia ............................. 15

H-Type Fistula .................................................. 16 Esophageal Stenosis ....................................... 16 Esophageal Duplications .................................. 16 Esophageal Diverticula .................................... 17 Gastroesophageal Reflux ................................. 17 Achalasia .......................................................... 17

Esophageal Motility Disorders ............................. 17 Oropharyngeal Dysphagia ............................... 18 Idiopathic Motor Disorders ............................... 18 Achalasia .......................................................... 18 Diffuse Esophageal Spasm .............................. 19 Nutcracker or Supersqueeze Esophagus ........ 19 Idiopathic Gastroesophageal Reflux ................ 20 Reflux Disease and Scleroderma ..................... 20 Idiopathic Gastroesophageal Reflux ................ 20

Hiatus Hernias ..................................................... 21 Paraesophageal Hiatal Hernia .................... 21

Esophageal Injuries ............................................. 23 Esophagus - Caustic Burns .............................. 23 Esophagus - Strictures ..................................... 23

Benign Esophageal Disease: Benign Strictures , Tumors, Cysts & Duplication ............................... 24

Hiatal herniae ................................................... 24 Barrett’s esophagus ......................................... 26

Malignant Tumors ................................................ 28 Molecular Biology of Lung and Esophageal Cancer ............................................................................. 30

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Chapter Objective

At the completion of this chapter the resident • understands the embryology and anatomy of the esophagus and

their relationship to adjacent structures, the physiology of deglutition and swallowing, and applies the findings of invasive and non-invasive tests to patient management;

• understands the natural history, types, evaluation, and management of esophageal neoplasms, and performs operative and non-operative treatment.

• understands infectious, inflammatory, and environmental injuries of the esophagus and performs operative and non-operative management.

Learner Objectives

Upon completion of this chapter the resident: 1. Understand the segmental anatomy and developmental anatomy

of the esophagus; 2. Knows the esophageal physiology, and how to interpret

esophageal function tests , and the tolerability of resections; 3. Knows the indications, contraindications and complications of

different esophageal diagnostic procedures 4. Understands the pathogenesis and the management of

congenital lesions of the esophagus; 5. Understands the pathogenesis and the management of

esophageal motility disorders (EMDs); 6. Understands the pathogenesis and the management of hiatal

hernias; 7. Understands the pathogenesis and the management of Barrett’s

esophagus; 8. Understands the pathogenesis and the management of cancer

esophagus; 9. Understands the molecular biology of lung and esophageal

cancer; 10. Understands the indications for resection of benign esophageal

neoplasms; 11. Knows iindications, contraindications, and complications of the

operations of the esophagus.

Sources for further reading Textbook Chapters

Chapter 3: Endoscopy, Bronchoscopy, and Esophagoscopy. Surgery of the Chest (Sabiston and Spencer), 6th ed., 84-97.

Chapter 25: Surgery of the Esophagus in Infants and Children. Surgery of the Chest (Sabiston and Spencer), 6th ed., 885-910. Chapter 27: Disorders of the Esophagus in the Adult. Surgery of the Chest (Sabiston and Spencer), 6th ed., 958-65.

Chapter 9: Esophagoscopy and Endoscopic Esophageal Ultrasonography. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 153-168. Chapter 39: The Esophagus - Anatomy and Functional Evaluation. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 691-728.

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Anatomical Considerations

Developmental Anatomy

Embryology • Muscular epithelial-lined tube • Derived from primitive foregut • Second week of embryologic development • Mesoderm forms and separates ectoderm from endoderm--

providesmaterial necessary for connective tissue, muscular coats, serous coverings

Histology and Final Development

• Adventitia: outer loose connective tissue containing nerves, lymphatics, blood vessels

• Muscularis: two layers of muscle--outer longitudinal and an inner circular

• Submucosa: connects muscularis with the mucosa--strongest layer--elastic tissue; collagenous fibers; network of vessels & nerves

• Mucosa: squamous, columnar; Z-line

Surgical Anatomy

Begins (transition from pharynx to esophagus) at lower end of sixth cervical vertebra/cricoid cartilage Ends (transition to stomach) at 11th thoracic vertebra Esophagus is midline, passing to the L. in lower neck and upper thorax, then back to midline, then to L. again in lower thorax to pass through diaphragmatic hiatus Follows curve of vertebral column except to pass anteriorly to pass through diaphragmatic hiatus

Sites of perforation during rigid esophagoscopy: i) Cricopharyngeus ii) Terminal L. anterior deviation

Measurements i) Incisors to cardia = 38-40cm (men), 36-38 (women) ii) Cricopharyngeus to cardia =23-30cm, avg. 25 iii) Incisors to cricopharyngeus = 14-15cm iv) Incisors to tracheal bifurcation/indentation of aortic arch = 24-26cm

Anatomic relations of esophagus i) Trachea and cervical spine ii) Recurrent laryngeal nerves - in tracheoesophageal groove - L. is closer to esophagus iii) Above tracheal bifurcation, esophagus passes to the R. of the aorta iv) From arch down, esophagus lies to the R. of the aorta v) 8th vertebra - L. wall of esophagus is covered only by mediastinal pleura - common site of perforation in Boerhaave’s syndrome vi) Passing through diaphragmatic hiatus, phrenoesophageal

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membrane surrounds vii) 2cm of abdominal esophagus between membrane and cardia - subjected to positive pressure viii) Thoracic duct - through diaphragm behind aorta - in thorax, dorsal to esophagus, from 5th thoracic vertebra up, it passes to L., then departs from esophagus in neck to join L SCV at junction of IJV Musculature of the esophagus i) Opening is collared by cricopharyngeus muscle ii) Outer longitudinal layer and inner circular layer iii) Circular muscle is elliptical iv) Upper esophagus is only striated muscle v) At upper/middle 1/3 junction - 50% smooth muscle

Arterial Supply

3 sources 1. Inferior and superior thyroid arteries: cervical esophagus 2. Tracheobronchial, aortic arch and esophageal branches: body of esophagus 3. L. gastric and splenic arteries: GEJ

3 parts i) Cervical = inferior thyroid artery (mainly) + common carotid, SCA ii) Thoracic = bronchial arteries (75% have one R and 1-2 L) iii) Abdominal = branches of L. gastric and inferior phrenic arteries iv) After penetrating esophagus, arteries branch in T to form longitudinal anastomoses v) Esophagus can be mobilized from stomach to aortic arch w/o devascularization

Venous Drainage

Veins: drainage pattern similar to lymphatics Submucosal plexus--communicating veins-- perforating veins that pierce the muscularis Eventually drain into inferior thyroid, azygous, hemiazygous, L. gastric, splenic, L. gastroepiploic systems

3 Venous drainage i) Cervical = inferior thyroid ii) Thoracic = bronchial, azygos and hemiazygous veins iii) Abdominal = cardiac vein

Lymphatic Drainage

Submucosal lymphatics form long channels that run parallel to esophageal axis May travel long distances before draining into regional nodes Submucosal plexus - lymph flow is longitudinal - extensive submucosal spread (of tumor) can occur

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Innervation

Nerves: extraesophageal Sympathetic: cervical, thoracic chains; celiac plexus and ganglia Parasympathetic: vagus nerve muscular plexus around the circular layer of the muscularis (Auerbach's plexus) Submucosal plexus (Meissner's plexus) Auerbach's and Meissner's plexus are interconnected and are responsible for the fine- control mechanism of esophageal function

Innervation i) Parasympathetic = vagus ii) Cricopharyngeus and cervical esophagus - recurrent laryngeal nerves iii) RLN injury causes vocal cord paralysis and dysfunction of cricopharyngeus and of cervical esophageal motility, predisposing to aspiration iv) Esophageal plexus receives fibers from vagus and from thoracic sympathetic chain

Nerve Supply (Nerves: central)

Nucleus ambiguous Dorsal motor nucleus of Vagus through the myenteric plexus Both work together on the longitudinal and circular muscles to propel a bolus--peristalsis

Physiological Considerations

Peristalsis

Primary: normal propulsive wave in response to the stimulation of normal voluntary deglutition Secondary: normal wave without voluntary deglutition: best defense Tertiary: abnormal; may occur spontaneously or following deglutition

Normal function

Upper Esophageal Sphincter (UES) Level of cricoid cartilage C 5-6 Composed of cricopharyngeus and inferior pharyngeal constrictors Remains contracted between swallows due to continuous stimulation by IX and XI Resting tone: 45 - 65 mmHg (range 32 -101 mmHg) Swallow: inhibition of all motor nerve stimulation; UES opens; closes; rebound; baseline pressures

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Esophageal Body Proximal striated muscle: direct innervation to its motor end plate from nucleus ambiguous Smooth muscle: indirect neural input from dorsal motor nucleus (X) via myenteric plexus Innervation: longitudinal muscle shortens; circular muscle contracts; peristalsis Duration and amplitude: weaker in proximal esophagus; stronger, longer in distal esophagus

Lower esophageal sphincter (LES)

Specialized muscle arrangement 3 - 4 cm above gastroesophageal junction (GEJ) High pressure zone with resting tone 15 - 25 mmHg (24.8 mmHg) Influenced by neural and hormonal factors; drugs Relaxes at time of swallowing; closes with passage of contraction through sphincter Manometry

Anatomy of the esophagogastric junction a) Phrenoesophageal membrane i) Fascial attachments of the esophagus to the diaphragm ii) Attached to esophagus several centimeters above G-E jxn iii) Maintains intraabdominal esophagus

Normal structure and function a) Pharyngeal phase of swallowing

i) Tongue is piston - propels food bolus as soft palate is closed ii) Swallowing is reflex, once initiated iii) Larynx is elevated and epiglottis covers opening of larynx iv) Pharyngeal pressure increases to 45mm Hg v) Food propelled by pressure gradient into thoracic esophagus vi) Upper, striated portion of esophagus relaxes, then contracts within 0.5 seconds to twice its resting level of 30mm Hg vii) A peristaltic wave of 30mm Hg begins in the esophagus viii) Afferent nerves of pharynx are glossopharyngeal and superior laryngeal branch of vagus ix) Efferent nerves arise from CN V, VII, X, XI, XII and C1-3 x) Motor disorders of pharyngeal swallowing:

a. Incomplete upper sphincter relaxation b. Loss of skeletal portion of cervical esophagus

b) Esophageal phase of swallowing

i) Pressure gradient of -6mm Hg in thoracic esophagus to +6mm Hg intraabdominal ii) Lower 1/3 of esophagus is most important

a) Peristaltic wave of 30-120 mm Hg b) Rises to a peak in 1 sec, lasts 0.5 sec, then subsides in

1.5 sec c) Wave moves down the esophagus at 2-4 cm/sec,

reaches distal esophagus 9 sec after swallow starts d) Vagal modulated wave e) If vagi are preserved, muscle can be divided and

propagate wave f) Vagal fibers end in myenteric plexus

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g) No known sympathetic innervation of the esophagus iii) Pathologic states

a) Diffuse esophageal spasm - simutaneous contraction b) Achalasia - failure of LES relaxation c) Scleroderma - loss of contraction of smooth muscle

portion of esophagus The antireflux mechanism a) LES

i) No distinct anatomic sphincter, but muscular architecture of cardia acts like a sphincter ii) Gastric contraction results in increased LES pressure

b) Resting LES pressure

i) Correlates with incidence of GERD ii) Truncal vagotomy has no effect iii) Atropine (and other anti-cholinergics) reduces LES tone but

does not cause GER iv) In pharmacologic doses:

a) Secretin, cholecystokinin, glucagon, prostaglandins reduce LES pressure

b) Gastrin, bombesin, motilin augment it v) Low LES tone in GERD is probably due to abnormal myogenic function vi) Results of antireflux operations are independent of changes in resting LES pressure vii) Myotomy can be performed along the length of the LES without resulting in reflux

c) Phrenoesophageal ligament d) Intra-abdominal esophagus

i) Laplace’s law - pressure required to distend a soft tube is inversely proportional to its diameter

ii) Small-diameter esophagus requires high intragastric pressure to allow reflux

iii) LES competence directly proportional to length of intra-abdominal esophagus in cadaver studies by DeMester

GERD a) Results from decrease in LES pressure, shortening of the intra-abdominal esophagus or both b) Competence of cardia

i) Requires adequate LES pressure + intrabdominal length ii) 80% prob of GERD when LES <5mmHG (independent of

length) iii) 80% prob of GERD when length <1cm (independent of

pressure) iv) Low incidence when pressure > 20mmHg and >2cm abd

length c) Gastric function

i) Delayed gastric emptying ii) intragastric pressure and distention shorten intraabdominal

length d) Overall LES length is also a factor in GERD e) Esophageal clearance

i) Gravity, salivation and swallowing ii) Pts w/complications of GERD (Barrett’s and stricture) have

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higher proportion of weak amplitude and simultaneous contractions iii) frequency of swallows (0.87à 2.59/min) during episodes of

reflux iv) Any impairment of motility may exposure time

f) LES relaxation-abnormal will à increased exposure g) Hiatal hernia i) Phrenoesophageal ligament and snug hiatus prevent distention of abdominal esophagus h) Antirreflux operations restore to normal the failed components of a mechanically defective sphincter Esophageal Diagnostic Procedures There are several procedures utlitized for the diagnosis of esophageal disease, and the approach must be tailored to the specific disease entity. Contrast swallow and esophagoscopy are commonly used for most patients with esophageal disorders, with CT scanning, endoscopic ultrasound, and reflux testing reserved for more specific indications.

Diagnostic Techniques Radiology Endoscopy Reflux Disease Esophageal Malignancies Esophageal Biopsies Motility Studies 24 Hour pH Monitoring Acid Perfusion Tests (Bernstein's Test) Radionuclide Emptying Studies Molecular Pathology

Radiographic Examples A. Schatzki's ring B. Achalasia C. Diffuse Esophageal Spasm D. Leiomyoma E. Carcinoma

Esophagoscopy

Indications · Dysphagia, odynophagia, regurgitation, hematemesis, chest pain, foreign body ingestion, or history of traumatic esophageal tear · Should usually be preceded by contrast swallow/cineesophagogram to help localize the site of disease · Contraindications include aortic aneurysm (can rupture), recurrent nerve paralysis, esophageal diverticulum (can perforate blindly), corrosive strictures (can perforate - stop when you see the stricture), and kyphoscoliosis (may be impossible) · Use rigid technique when Zenker's diverticulum or disease of the upper third is suspected, as flexible esophagoscopy is done blindly and can perforate in these areas

Technique - Rigid Esophagoscopy · Topical or general anesthesia may be used; general anesthesia generally provides better relaxation, lowering the risk of perforation · The 9mm scope is adequate for most adult patients · The patient is positioned supine with head and shoulders over the end of the table · Introduce the esophagoscope into the R. side of the mouth and

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rest the shaft on your L. thumb · The scope is advanced behind the R. arytenoid cartilage into the R. pyriform fossa · Lower the patient's head as the scope is advanced past the cricopharyngeus · Lower the head further and move to the R. to pass through the gastroesophageal junction · Full examination is done on withdrawal, as folds of mucosa may hide pathology during advancement of the scope Technique - Flexible Esophagoscopy · Topical anesthesia with sediation is usually adequate · The patient is placed in the L. lateral position · The esophagoscope is introduced blindly with gentle pressure as the patient swallows · Insufflation of air distends the esophagus for complete visualization · Advance scope into upper stomach and perform thorough examination upon withdrawal

Complications · Perforation occurs in 0.1-0.25% of patients · Most commonly occurs posteriorly at the upper opening of the esophagus when forceful pressure is applied against the cricopharyngeus · Other sites include the diaphragmatic hiatus and diverticuli · Perforation can also occur after deep biopsy, forceful dilation of strictures, or during removal of foreign bodies · Chest pain after esophagoscopy is an indication of perforation and should be promptly evaluated

Findings in Disease

Stages of Reflux Esophagitis

Reflux Esophagitis Stage I localized spots of erythema, some with exudate Stage II confluent areas of erythema Stage III circumferential areas of erythema, friable, bleeds

readily when touched Stage IV deep ulcers, stenoses and columnar metaplasia

· Barrett's esophagus: stratified squamous epithelium replaced by columnar epithelium and may become discrete ulcer; biopsy should be performed to look for malignancy · Stenosis: congenital stenoses usually have normal mucosa; acquired stenoses are usually associated with esophagitis or ulcers · Corrosive esophagitis: acute inspection shows edematous, friable walls which are easily perforated; stop at first area of injury · Diverticulum: exclude ulcers and neoplasms at the site of the diverticulum · Varices: range from small bluish elevations to large dilated veins at the lower end of the esophagus--commonly found in cirrhotics · Hiatal hernia: redundant folds in the lower esophagus and lack of diaphragmatic support are characteristic only in true hiatal hernia

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· Achalasia: markedly dilated, inflamed esophagus with thickened walls; GE junction has normal tone but may be hard to negotiate · Carcinoma: typically large fungating mass that bleeds easily, less commonly a smooth stenosis with edematous mucosa. Microinvasive carcinoma presents as slight discolorations of the mucosa, known as leukoplakia or erythroplakia. · Benign neoplasms: leiomyomas, fibromas, and lipomas are all covered with normal mucosa Therapeutic Esophagoscopy A. Removal of Foreign Bodies · Rigid esophagoscope is best · Most common sites are just below the cricopharyngeus and at the diaphragm · Sharp objects carry the highest risk of perforation

B. Dilation of Strictures · Savary-Gilliard dilators are the safest · A metal guidewire is passed through the stricture using the esophagoscope · The stricture is then dilated using progressively larger dilators passed over the guidewire · Retrograde dilation may also be done using Tucker dilators over a string passed through a gastrostomy and out the mouth

C. Corrosive Esophagitis · Esophagoscopy should be performed to confirm the burn, but do not pass the injured area · Dilation can be performed after burn have healed (usually 3-4 weeks) if strictures have formed

D. Carcinoma · Palliative dilation usually is only temporary, and should be followed with either laser resection or stenting · The Nd:YAG laser can be used from above or below to core a passage through tumor and permit swallowing · Brachytherapy can be applied after endoscopic dilation for inoperable carcinoma

E. Achalasia · Dilation can be performed of the GE junction if surgical myotomy is contraindicated · Perforation, however, is a definite risk and can present as either chest or abdominal pain

F. Variceal bleeding · Electrocautery and laser therapy of bleeding varices do not prevent rebleeding · Sclerotherapy is probably best and obliterates current varices; however, rebleeding occurs in 40% of patients

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Endoscopic Ultrasound

· Particularly applicable in defining tumors and varices · May become useful in staging of esophageal cancer · 5 layers are identified: mucosa, deep mucosa, submucosa, muscularis, and adventitia · Extension of tumors into periesophageal structures and lymph nodes can be evaluated · Carcinomas appear as indistinct, echo-poor lesions; varices appear as round, echo-poor lesions

Gastroesophageal Reflux Evaluation

A. Manometry · Intraluminal pressures are measured using a continuous infusion catheter system while the patient is lying supine · This catheter is withdrawn at 1-cm intervals to obtain resting pressures · The catheter is reinserted, and pressures measured after swallowing at 1-cm intervals · This test is essential in delineating the various esophageal motility disorders

Note: Radiographic tests for GE reflux are not highly reliable for pathologic reflux, as up to 25% of patients will have reflux without associated pathology. Such tests can rule out patients with no reflux, however.

B. pH Reflux Test · A pH probe is placed 5 cm above the GE junction · 200 to 300 ml of 0.1N HCl is instilled in the stomach · A fall in pH below 4.0 during various maneuvers indicates GE reflux

C. Acid Perfusion Test · The distal esophagus is perfused in an alternating fashion with 0.1N HCl and saline · The test is positive if atypical chest pain occurs during acid perfusion and resolves during saline perfusion · High rates of false positivity and false negativity make the test somewhat unreliable

D. 24-hour pH Monitoring · a pH probe is placed 5cm above the GE junction · The patient records any symptoms and pH changes are monitored constantly over 24 hours · Analysis includes percentage of time pH was less than 4.0, and percentage of time patient was upR. and supine · The number of reflux episodes, the duration of the episodes, and the longest episode of reflux are also evaluated · This test gives the most objective evidence of reflux

Congenital Esophageal Disease

Congenital esophageal stenosis

Esophageal web: middle 1/3, complete or incomplete, dilatation Intraluminal cartilaginous rest: tracheobronchial origin, local resection with primary repair Segmental hypertrophy: mucosa and submucosa, residual web

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Laryngotracheoesophageal cL. Varying degrees of communication between larynx-trachea and esophagus High incidence of GER 10 - 40% mortality

Esophageal atresia +/- TEF

Most common congenital esophageal anomaly Salivary regurgitation, abdominal bloating, pneumonia Dx: cannot pass NG tube, radiologic confirmation Mortality related mostly to other congenital lesions

Esophageal Anomalities 3. Esophageal atresia +/- TEF: types

Atresia with distal TEF: 87% Atresia without TEF: 8% TEF without atresia (H type): 4% Atresia with proximal TEF: 0.8% Atresia with prox/distal TEF: 0.7%

4. Waterston Classification (pneumonia increases one level)

Congenital Esophageal Atresia [table] 5.

Anderson PE, Cook A, Amery AH. A review of the practice of fibreoptic dilatation of oesophageal stricture. Annals of the Royal College of Surgeons of England 1989 71(2):124-7.

Azoulay D, Regnard JF, Magdeleinat P, Diamond T, Rojas-Miranda A, Levasseur P. Congenital respiratory-esophageal fistula in the adult. Report of nine cases and review of the literature. J Thorac Cardiovasc Surg 1992;104:381-384.

Deurloo JA, Ekkelkamp S, Schoorl M, Heij HA, Aronson DC. Esophageal atresia: historical evolution of management and results in 371 patients. Ann Thorac Surg 2002;73:267-272.

Dunn JC, Fonkalsrud EW, Atkinson JB. Simplifying the Waterston's stratification of infants with tracheoesophageal fistula. Am Surg 1999;65(10):908-10.

Congenital Esophageal Abnormalities

Esophageal Atresia

1. embryology a. esophagus first appears at four weeks gestation b. esophageal atresia results from incomplete separation of the laryngotracheal groove between the third and sixth weeks of gestation

2. common forms of esophageal atresia 3. esophageal atresia with fistula occurs in ~1/8000 births 4. incidence of associated anomalies is about 50%

c. cardiac defects are important to identify preoperatively d. VATER association is common- three or more of the following: vertebral defects, anal atresia, tracheoesophageal atresia and radial and renal dysplasia e. CHARGE association: choanal atresia, heart anomalies, esophageal atresia and GI tract anomalies

Tsai JY, Berkery L, Wesson DE, Redo SF, Spigland NA. Esophageal Atresia and Tracheoesophageal Fistula: Surgical Experience Over Two Decades. Ann Thorac Surg 1997;64:778-783.

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Esophageal Atresia With Tracheoesophageal Fistula

Signs and Symptoms 1. related to esophageal obstruction and respiratory complications resulting from reflux 2. infants have copious salivary secretions and tend to choke during feeds 3. gastroesophageal reflux causes aspiration pneumonia and respiratory distress 4. air is eventually forced through the TEF and creates abdominal distension

Diagnosis 1. some are diagnosed on prenatal ultrasound 2. most are diagnosed after birth 3. if suspected then a rigid nasogastric tube should be placed and a CXR should be obtained which will demonstrate blind esophageal pouch 4. can also use water soluble contrast to study pouch to delineate the anatomy 5. if a R. aortic arch is suspected, then echocardiography should be performed

Treatment- Preoperative Management 1. place baby in the semisitting position 2. place sump tube for control of saliva or use frequent suctioning 3. resuscitate in the ICU, correct electrolyte and acid base status 4. antibiotics are given 5. mechanical ventilation if required

a. in patients with increased pulmonary resistance, high frequency ventilation with low mean airway pressure may be more effective than conventional ventilation b. lower mean airway pressures should help control abdominal distension

Surgical Treatment 1. surgical approach and timing of surgery depend upon the size of the infant and condition 2. primary repair is preferable for almost all infants unless weight is less than 1500 g or they have associated severe respiratory distress syndrome for which delayed primary or staged repair should be completed 1-8 weeks after birth 3. some infants may be delayed secondary to critical congenital cardiac disease 4. most patients do not require gastrostomy before thoracotomy unless a staged procedure is planned 5. in patients that require high ventilatory airway pressures, a gastrostomy may become a source of an air leak exacerbating respiratory status 6. if the infant has an associated doudenal atresia or imperforate anus, these defects are corrected first then the esophageal fistula (author had no deaths in 24 patients with this sequence) 7. approach:

a. R. thoracotomy- standard approach b. L. thoracotomy- when there is a R. aortic arch c. can be extrapleural or transpleural d. division of azygous vein and identification of vagus nerve will facilitate locating fistula e. fistula is closed utilizing running suture

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f. esophagus is reapproximated with either a single or double layer anastomosis g. the esophagus can be elongated with circular or longitudinal myotomies, but a staged procedure may be required if enough length cannot be attained

Complcations 1. most common complications are pneumonia and atelectasis 2. anastomotic leak 3. anastomotic strictures

a. occur most commonly after leak has occurred b. most can be dilated

4. gastroesophageal reflux d. may be associated with laryngospasm and apnea spells e. 10-50% patients require Nissen fundiplication

Results 1. risk classification by Waterston

group A- birth wt > 2.5 kg in good medical condition group B- birth wt 1.8-2. Kg with mild pneumonia or an associated congenital anomaly group C- birth wt < 1.8 kg or higher with severe pneumonia or life threatening anomaly

2. in author’s experience with 100 patients there was no mortality with group A or B patients and a 15% early mortality in group C patients

a. overall early mortality was 4.6% b. late mortality was 10% over 10 year period

Isolated Esophageal Atresia

Signs and Symptoms 1. similar presentation with those with a tracheoesophageal fistula 2. most weigh less than 2.5 kg 3. almost all have associated maternal polyhydramnios 4. scaphoid, gasless abdomen

Surgical Treatment 1. lower esophageal segment is usually short 2. primary repair is not feasible 3. gastrostomy should be performed 4. daily lengthening of the upper esophageal pouch is completed with an orogastric tube 5. after 6 weeks to 3 months repair is attempted 6. it is usually necessary to perform myotomy to gain length

Esophageal Replacement 1. if esophageal replacement is necessary, then a gastrostomy and cervical esophagostomy should be performed 2. these patients are then treated until the age of 1 year or more with G-tube feeds 3. techniques:

a. jejunal interposition is no longer used b. colonic inerposition with R. or L. colon i. substernal ii. intrathoracic a. gastric transposition

3. Complications a. most common complication of colonic replacement is leak

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b. most serious complication is gangrene due to congestion c. peptic ulceration of the cologastrostomy can occur

Results 1. 30 patients operated on without mortality 2. 12 patients received esophageal lengthening, myotomy and delayed primary anastomosis with stricture formation in 10 patients (9/10 dilated successfully)

H-Type Fistula

Signs and Symptoms 1. have symptoms of aspiration 2. may present later in life up to 1-2 years of age 3. have difficulty with thin feeds

Diagnosis 1. some advocate use of contrast studies to demonstrate fistula 2. author utilizes endoscopy to make diagnosis 3. bronchoscopy may also demonstrate fistula 4. most fistulae are located low in the neck and rarely in the thorax

Treatment/Results 1. performed electively 2. preliminary gastrostomy not required 3. fistula can usually be approached via a neck incision unless it is thoracic 4. preoperative placement of a fogarty catheter may facilitate identification 5. esophagus is dissected from the posterior trachea and the fistula closed with two layered closure and paraspinous muscle interposition 6. recurrent laryngeal nerve may occur 7. no mortality in 24 patients, one leak

Esophageal Stenosis

1. usually acquired but can be congenital 2. stenosis presents as a web or diaphragm amenable to dilation 3. if associated with cartilaginous tracheobronchial remnants, resection is required 4. may have associated anomalies such as esophageal atresia or anorectal malformations 5. most webs occur in the upper and middle esophagus; those associated with esophageal atresia tend to be located in the lower esophagus

Esophageal Duplications

1. generally lie in the posterior mediastinum 2. usually asymptomatic, found on CXR 3. approximately 30% of mediastinal masses in children are of

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foregut origin 4. usually do not communicate with the lumen of the esophagus

Diagnosis 1. barium esophagram may reveal filling defect 2. CT scan is procedure of choice 3. USG of abdomen to rule out associated abdominal duplications

Treatment/Results 1. preferred approach is complete resection 2. may be necessary to divide diaphragm or make separate abdominal incision to resect associated abdominal duplication cyst 3. no long-term sequlae encountered, no deaths or serious complications

Esophageal Diverticula

1. extremely rare 2. have complete muscular wall (unlike Zenker’s) 3. risk of aspiration 4. resection is treatment of choice

Gastroesophageal Reflux

1. in patients with intact esophagus and no other esophageal anomaly, the most likely cause of GER is failure of maturation of the LES 2. the LES is usually competent by 6-7 weeks of life 3. most common presenting symptoms are frequent regurgitation, upper GI tract bleeding, apneic spells, and chronic intermittent pneumonia 4. if symptoms persist for > 6 months then surgical intervention is recommended 5. diagnosis can be made by 24 hour pH monitoring, upper GI swallow and endoscopy 6. most patients respond to medical therapy- H2 blockade, bethanechol, reglan 7. Nissen fundiplication is procedure of choice

Achalasia

1. uncommon in childhood 2. diagnosed with barium swallow and esophageal manometry 3. trail of pneumatic dilatation is recommended first then surgical therapy if recurrence occurs rapidly (90% success rate is author’s experience) 4. cricopharyngeal achalasia may also occur in children which requires myotomy via cervical approach

Esophageal Motility Disorders

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Oropharyngeal Dysphagia

Neurologic: central vs peripheral Myogenic Cricopharyngeal Muscle Dysfunction Iatrogenic Lower esophageal disease

Idiopathic Motor Disorders

Hypomotility Disorders Achalasia

Hypermotility Disorders Diffuse Esophageal Spasm

Hyperperistalsis (Nutcracker; Supersqueeze) Hypertensive LES Nonspecific Esophageal Motility Disorders

Achalasia

Young adults -- 1 in 100,000 -- Cause unknown Loss of control at the postganglionic, nonadrenergic and noncholinergic inhibitory nerves -- LES dysfunction and esophageal body changes ? denervation; Chagas' Disease Compounded by physical & psychologic stress LES: normal or increased resting pressure Incomplete or absent relaxation

Symptoms: dysphagia; odynophagia; regurgitation; aspiration and its complications; made worse by cold liquids and stressful situations Squamous cell carcinoma 1-10% Peristalsis absent in esophageal body; high resting pressure Contractions weak at all recording levels Esophageal dilatation; megaesophagus Barium swallow: "bird's beak" esophagus

CXR: widened mediastinum; air-fluid level in posterior mediastinum; absence of gastric bubble Endoscopy: changes vary with stage

Normal Food/fluid retention in fasting state Thickened mucosa with wide folds Hyperemia: stasis esophagitis Achalasia Upper GI Achalasia Manometry

Treatment Medication: calcium channel blockers--decrease LES resting pressures--improves symptoms, not emptying

Early disease with minimal symptoms Dilatation: stretches/ruptures fibers of the LES

Lowers LES resting pressure; improves emptying Good to excellent short term results in 65% of patients Esophageal rupture seen in 4%

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Reflux seen in 7 - 17% Esophageal myotomy: improves obstructive symptoms more effectively than dilatation Can be done via L. thoracotomy, laparotomy, or scope 5 - 7 cm myotomy on distal esophagus Extends 1 cm onto gastric wall Mucosa dissected from muscularis 90% relief of dysphagia short and long term ? concomitant antireflux procedure: partial fundoplication

Diffuse Esophageal Spasm Rare--cause is unknown; muscle hypertrophy; degenerative changes in Vagus branches Symptoms: odynophagia, dysphagia, unexplained chest pain Anxious individuals Symptoms worsened by stress Must differentiate from CAD

Simultaneous segmental contractions on x-ray Corkscrew / rosary bead esophagus Diverticulum: intermittent or epiphrenic Endoscopy: usually normal Peristalsis:>30% repetitive tertiary contractions Duration and amplitude occasionally abnormal LES: occas. hypertensive; occas. incomplete relaxation Spasm Upper GI Spasm Manometry

Treatment • Nitroglycerin • Calcium channel blockers: decreases amplitude of contraction &

reduces LES pressure • Control anxiety and precipitating factors • Esophageal myotomy: results not as good as in achalasia; good to

excellent results in 67 - 70% • If LES is transected must do a partial fundoplication • Dilatation if hypertensive LES is documented

Nutcracker or Supersqueeze Esophagus

Normal peristalsis Contraction amplitude is > 2 standard deviations above normal > 180 mmHg in distal esophagus Duration of contractions >6 sec. LES: occas. hypertensive; usually normal Primary symptoms is chest pain Strong emotional influence / hypochondriacal Nutcracker Esophagus Manometry

Treatment Psychologic assessment and support Calcium channel blockers Dilatation or myotomy are of little or no benefit Hypertensive LES

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Resting pressure > 45 mmHg; normal relaxation and peristalsis Conservative medical / psychiatric management

Idiopathic Gastroesophageal Reflux

Peristalsis and contraction amplitude are normal LES is weak (gradient < 6 mmHg) allowing gastric acid to reflux and bathe the lining of the lower esophagus--responsible for 60 - 70 % of all GERD Sphincter failure Primary muscle dysfunction Increased exposure to acid further weakens the LES leading to further reflux and mucosal damage Esophageal contraction abnormalities with poor amplitude and aperistalsis Correlation between the severity of sphincter hypotension and extent of functional abnormalities in the esophageal body

(DeMeester) ? correction cures abnormalities

Reflux Disease and Scleroderma

Seen in 90% of patients with scleroderma Atrophy of smooth muscle components Fibrous infiltration Incompetence of LES with disappearance of propulsive and emptying mechanisms Antireflux procedures not as successful though some improvement is seen Reflux

Idiopathic Gastroesophageal Reflux

Frequent association with Type I hiatal hernia Alterations in the anatomy of the hiatus Phrenoesophageal membrane

Secondary causes Delay of gastric emptying Pyloric stenosis Gastric mass Poor esophageal wall muscle tone (scleroderma)

Reflux: two factors must occur Acid-peptic or pancreaticobiliary secretions must reach the esophagus with increased frequency Esophagus must be unable to clear those refluxed materials back into the stomach Treatment

Medical treatment Surgical treatment if medical treatment fails

Diagnosis Barium swallow Fiberoptic / rigid endoscopy Multiple biopsies if findings consistent with severe esophagitis, stricture, Barrett's epithelium, ulceration Esophageal function test (manometry, acid reflux, perfusion

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and clearing tests, 24 hr pH monitoring) Indications for surgery 1. Symptomatic after 3 months of medical therapy 2. Persistent esophagitis, stricture, aspiration, bleeding 3. Positive 24 hr pH study 4. Manometry suggesting dysfunctional LES and adequate esophageal

motility (peak amplitude > 30 mmHg)

5. Barrett's mucosa if biopsies are benign (no CIS)

Operation of choice 1. Restoration of the anatomic and physiologic relationships of the

LES at the GEJ 2. 360 degree wrap (normal esophageal motility) 3. 270 degree wrap (dysfunctional esophagus) 4. Esophageal resection (rare) 5. Totally unyielding (fibrotic) esophagus 6. Barrett's esophagus with CIS or frank malignancy 7. Gastroplasty: falling out of favor

Various authors. Endoscopy in upper GI bleeding. Endoscopy 1986 18 Suppl:1-68.

Young MM, Deschamps C, Allen MS, Miller DL, Trastek VF, Schleck CD, Pairolero PC. Esophageal reconstruction for benign disease: self-assessment of functional outcome and quality of life. Ann Thorac Surg 2000;70:1799-1802.

Procedures • Nissen Fundoplication • Belsey Mark IV • Hill Fundoplication • Collis Gastroplasty • Collis-Belsey Procedure Hiatus Hernias

Paraesophageal Hiatal Hernia

All herniations of the fundus/body of the stomach into the chest which are anterior or lateral to the esophagus are paraesophageal hernias. They account for 3-6% of operations for hiatal hernia. The presence of a paraesophageal hernia, regardless of the size or symptoms, is an indication for repair S

Types: #1—true sliding hernia; the phrenoesophageal ligament fails to keep the esophagogastric junction below the diaphragm and within the abdomen. The LES is usually inadequate and reflux esophagitis exists #2—true paraesophageal hernia with the esophagogastric junction in its normal location below the diaphragm. The fundus/body of the stomach is rotated into the chest with the greater curve as the leading point; usually no esophagitis present. #3—combination of upward movement of the esophagogastric junction above the diaphragm and herniation of the stomach into the chest. The patient usually has symptoms of esophagitis.

Anatomy: -Intact posterior fixation of the esophagus to the preaortic fascia and the median arcuate ligament

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-The reason why the greater curve of the stomach herniates is because it is the most mobile portion—gastric cardia is fixed by the L. gastric vessels, the gastrosplenic and gastrohepatic ligaments; the pylorus is fixed by the duodenum. -As the hernia enlarges the stomach moves upward and to the R. utilizing the fixed lesser curve as an axis of rotation—organoaxial rotation. This is the path of least resistance because the aorta lies to the L. and the heart lies L. and anterior. -This is a true anatomic hernia with a sac Symptoms: -produce few symptoms when small, which is why the defects are large when discovered -long history of postprandial distress/discomfort -substernal fullness and belching -true dysphagia uncommon -absence of heartburn/esophagitis -pulmonary complications are common: recurrent pneumonia; chronic atelectasis; dyspnea classically after a large meal—from pleural space compression by the huge hernial sac -ulceration of the herniated stomach with resultant bleeding and anemia -incarceration, obstruction, torsion, gangrene, and perforation -most feared and lethal complication is gastric volvulus with strangulation which usually occurs post-prandially—this is a true surgical emergency if the stomach cannot be decompressed. Almost 30% of paraesophageal hernis present in this fashion. The stomach becomes twisted and angulated in its midportion just proximal to the antrum. Most prominent manifestation is the inability to swallow or regurgitate. -Borchardt’s triad: chest pain, retching but unable to vomit, and inability to pass a nasogastric tube indicate gastric volvulus

Diagnosis/Therapy -CXR--retrocardiac air-fluid level -Barium Swallow to show an intrathoracic upside down stomach; look for signs of peptic esophagitis/position of GE junction -Technical points:

antireflux procedure—routinely vs. those with signs of peptic esophagitis surgical approach—transthoracic [ease of hernial sac dissection and esophageal mobilization when necessary] vs. abdominal [placement of a gastrostomy tube]

-Technique—principals of repair are reduction of the hernia and its contents to the abdominal cavity along with repair of the defect -mobilize esophagus; GE junction below diaphragm -narrow the hiatus posteriorly first until tip of finger can be admitted -fix stomach below the diaphragm (Hill repair—stomach fixed to median arcuate lig) -+/- Nissan fundoplication -gastrostomy -resection for gangrene/perforation

Results -elective repair has ~1% mortality -emergent procedures (volvulus) has ~15% mortality -long term results are generally excellent whether or not an anti-reflux procedure is performed

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Esophageal Injuries

Esophagus - Caustic Burns Elleson DA and Rowley SD.

Esophageal perforation: its early diagnosis and treatment. Laryngoscope 1982 92(6 Pt 1):678-80.

Estrera A, Taylor W, Mills LJ, Platt MR. Corrosive burns of the esophagus and stomach: a recommendation for an aggressive surgical approach. Ann Thorac Surg 1986;41:276-283.

Foglia RP. Esophageal disease in the pediatric age group. Chest Surg Clin N Am 1994;4(4):785-809.

Foker JE, Linden BC, Boyle EM Jr, Marquardt C. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997;226(4):533-41.

Hawes RH. Normal endosonographic findings. Gastrointestinal Endoscopy 1996 43(2 Pt 2):S6-10.

Henderson RD, Henderson RF, Marryatt GV. Surgical management of 100 consecutive esophageal strictures. J Thorac Cardiovasc Surg 1990;99:1-7.

Acid or alkaline injury Accidental - children < 5 yrs, household cleaners Intentional - adults with psych disorders, suicide attempts Severity related to strength / duration of exposure (prox < distal) Laryngeal edema - respiratory distress Phases Inflammation, edema, necrosis - initial few days Ulceration, sloughing, granulation - 3 - 4 weeks Cicatrization, scar formation - weeks - months Long-term

Recurrent strictures Respond to dilatation

Pre-cancerous: may develop up to 40 yrs later Increased cancer risk 1000 fold (debated)

Treatment Appropriate dilution, no emesis Fluids, antibiotics, (steroids controversial) Fiberoptic laryngoscopy / intubation Esophagoscopy - 48 hours, stop at first evidence of burn Barium swallow 3 - 4 weeks, intermittently thereafter Later dilatation for strictures Surgery usually not necessary early Sepsis / full-thickness necrosis: cervical esophagostomy, gastrostomy with isolation, later reconstruction, (resection controversial)

Esophagus - Strictures

Causes Malignancy Peptic stricture - at GE junction Mid-esophageal stricture - usually related to Barrett's esophagus Caustic stricture Schiatzki's ring - submucosal fibrous band at squamocolumnar junction Post-operative/anastomotic Congenital Motility disorders, vascular rings

Little AG, Naunheim KS, Ferguson MK, Skinner DB. Surgical management of esophageal strictures. Ann Thorac Surg 1988;45:144-147.

Mercer CD and Hill LD. Surgical management of peptic esophageal stricture. Twenty-year experience. J Thorac Cardiovasc Surg 1986;91:371-378.

Oakes DD, Sherck JP, Mark JB. Lye ingestion. Clinical patterns and therapeutic implications. J Thorac Cardiovasc Surg 1982;83:194-204.

Evaluation and Therapy If cause not obviously apparent, malignancy must be ruled out Barium swallow Esophagoscopy, brushings, biopsy If peptic: anti-reflex therapy If benign: dilatation - up to 50 Fr If mucosa / bx normal: external compression, motility disorder, submucosal lesions Bougienage Usually successful for benign strictures Safest with rigid esophagoscopy, general anesthesia (rarely practiced) May worsen gastroesophageal reflux

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Retrograde via G-tube (children) Other methods: fluoroscopic bougienage, pneumatic dilatation, stents For tight stricture, wire-guided Savory dilators Surgical Considerations Tight strictures, severe scarring, repeated dilatations increase risk of perforation Consider surgery, particularly in young patient Anti-reflux procedure (peptic stricture) High stricture - intra-op dilatation + anti-reflux procedure Consider motility disorder, pre-op motility studies

Esophageal lengthening procedure (Collis-Nissen) for shortened, scarred esophagus For extensive scarring, multiple or long strictures, Barrett's with dysplasia Consider esophageal replacement Gastric pull-up (intrathoracic anastomosis assoc. with recurrent reflux) Colon interposition Jejunal interposition

Benign Esophageal Disease: Benign Strictures , Tumors, Cysts & Duplication

Hiatal herniae

a) Type I (sliding) i) Phrenoesophageal ligament is stretched ii) G-E jxn can migrate to an intrathoracic position iii) G-E jxn remains cephalad to stomach iv) Reducible, unless esophagus is foreshortened from stricture b) Type II (paraesophageal hiatal hernia) i) Rare ii) Phrenoesophageal ligament remains firm, binding esophagus to preaortic fascia and median arcuate ligament iii) G-E jxn at or near normal location iv) Fundus and body of stomach roll into chest alongside esophagus c) Type III (sliding and rolling hernia) i) E-G jxn and most of greater curve and body of stomach herniate d) Etiology i) Most are acquired ii) Obesity and pregnancy increase intraabdominal pressure, stretching phrenoesophageal ligament

Pathophysiology of G-E reflux a) LES - resting pressure, length, site b) Esophageal clearance - gravity and esophageal peristalsis c) Mucosal resistance d) Salivary neutralization e) Gastric secretion i) Sensitivity to gastric acid, pepsin, alkaline bilious material ii) Additive effect iii) Gastric emptying (40% of pts w/reflux esophagitis f) Complications i) Anemia (chronic blood loss) ii) Stricture iii) Barrett’s esophagus g) Pts w/G-E jxn incompetence should be treated the same as those w/ concomitant hiatal hernia

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Symptoms a) Most patients are free of symptoms b) Very small risk of incarceration, strangulation, or obstruction of bowel (seldom involved) c) Retrosternal pain, heartburn, postural regurgitation, gaseous eructation d) Symptoms worse after a meal or when supine e) Pain may radiate to jaw, neck, ears or arms f) Respiratory involvement in 20% i) Acid alone may exacerbate asthma g) Shatzki ring i) Membrane or diaphragm on swallow ii) Most are asymptomatic, but may produce intermittent or persistent dysphagia (solid > liquid) iii) Always associated with hiatal hernia iv) Squamous mucosa on top and gastric mucosa on bottom v) Smooth muscle, fibrous tissue and scar between vi) Tx a) Dilatation w/balloon or bougie b) If symptomatic from hiatal hernia-repair hernia and dilate h) Barrett’s esophagus i) “Invariably” associated w/sliding hiatal hernia and severe GE reflux 5. Diagnosis a) Radiographic - Ba swallow w/maneuvers to assess reflux b) EGD for all respiratory or GER symptoms c) Formal testing of esophageal function i) Not necessary for typical symptoms of GER ii) 24hr pH monitoring and manometry prior to surgical intervention

Medical treatment a) Lifestyle modification i) Elevation of head of bed a) Head 6-10 inches above the feet b) Reduces number of reflux episodes and increases rate of clearance ii) Weight reduction - can improve symptoms iii) Smoking a) Can decrease LES pressure and frequency of GER episodes b) Cessation reduces frequency of episodes iv) EtOH - Lowers LES pressure and impairs peristalsis v) Diet modification a) Coffee, chocolate, peppermint and fatty foods - ¯ LES pressure b) Citrus and tomato juice - direct mucosal irritation c) Cola and milk products stimulate acid secretion d) Timing of meals - avoid recumbency vi) Medications a) Theophylline, dopamine, nitrates, opiates, diazepam, calcium channel blockers - all lower LES pressure b) Pharmacologic therapy i) Cytoprotective agents - sucralfate a) Controversial data on efficacy b) Primary use is adjunctive (add to acid suppression) ii) Antacids - increase LES pressure and alkalinize gastric acid iii) Acid suppression - H2 blockers a) Decrease volume and concentration of gastric acid b) Less effective for GER than for PUD c) 50-60% healing in grade II-III esophagitis (cimetidine) d) Healing is slow-treat for 3-6 months iv) Acid suppression - proton pump inhibitors - omeprazole a) Suppresses basal and stimulated gastric acid secretion

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b) Superior to placebo, ranitidine and cimetidine for GER c) Recommendation is cessation after 2 months v) Prokinetic agents a) Metachlopramide-dopamine antagonist - disappointing b) Cisapride- enhances acetylcholine release from myenteric plexus (1) Better than placebo for GER symptoms (2) w/ cimetidine is better than cimetidine alone c) Strategy of medical therapy i) First phase = lifestyle changes - behavior, food and drugs ii) Second phase = Pharmacologic management a) H2-blocker, then add cisapride or sucralfate b) Proton pump inhibitor for failure of combo tx c) Continue tx for 3-6 months d) Up to 90% will relapse by one year iii) Surgical treatment for failure of medical tx or complications (stricture, bleeding, severe ulceration) Surgical therapy a) Failure of medical tx or complications (stricture, bleeding, severe ulceration) b) Significant symptoms and esophagitis in a young pt. c) Operative approach i) Restore 4-6cm of intraabdominal esophagus ii) Thoracic approach a) Shortening of the esophagus - to immobilize esophagus b) Prior esophageal procedure c) Esophageal dysmotility requiring myotomy d) Suggested mechanisms for improvements i) LES manipulation ii) Accentuation of angle of His iii) Increased opening of the cardia e) Procedures i) Complete fundoplication - Nissen/Nissen-Rosetti a) 87% free of symptoms long-term b) 3cm loose Nissenà4% free from post-op symptoms ii) Partial fundoplication a) Belsey Mark IV - 240° wrap b) Lind - 300° c) Toupet - 180° iii) Angelchick prosthesis a) 20% persistent dysphagia b) Migration

Barrett’s esophagus

Anatomy and physiology a) Definition=esophagus is lined w/columnar mucosa more than 3cm proximal to the distal end of the muscular esophageal tube b) 3 types of mucosa - gastric fundic, junctional, specialized columnar (80%) c) Acid (and pepsin and gastrin) is produced, but amount is insufficient to explain peptic ulceration of Barrett’s

Pathogenesis a) Nearly every patient has pathologic reflux b) Metaplasia of pleuropotential cells in submucosa c) Migration of gastric mucosa not felt to be mechanism

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Epidemiology a) 1% in pts w/o symptoms who undergo endoscopy b) 10-20% in pts w/symptoms of reflux c) 8-13% of children endoscoped for GER d) Age 50-60 e) Men=3xwomen f) Relative lack in African-Americans

Diagnosis a) GER symptoms b) Symptoms may improve with progression of Barrett’s c) >80% - hiatal hernia d) 75% - stricture e) Nearly 50% - ulcer f) Endoscopy i) Irregular squamocolumnar junction ii) Strictures typically involve squamocolumnar junction iii) Biopsy at multiple levels g) LES pressure is lower in pts w/Barrett’s than normal people and lower than pts w/reflux but w/o Barrett’s

Benign (non-neoplastic) complications i) Stricture, bleeding, mucosal ulceration ii) Severe bleeding - 25% (rare in GER) iii) Penetrating ulcer in 10% a) May be successfully treated w/acid suppression b) Resection for mediastinal perforation

Dysplasia and adenocarcinoma i) Persistent acid reflux responsible for Barrett’s is involved in dysplasia and malignant change ii) Other premalignant markers a) Increased G2 tetraploidy b) DNA aneuploidy c) ras oncogenes (H- ras, K- ras, N- ras) - not a factor iii) Risk of malignant degeneration related to a) Duration and severity of GER b) Tobacco c) Overall extent of columnar spread iv) Cancers a) Arise almost exclusively in specialized columnar epithelium b) Most have transmural extension with + nodes at time of dx v) 1/140 pt years = 40x usual risk in this country = 10-20% of all esophageal ca

Medical management a) Lifestyle changes - behavior, food and drugs b) Medications c) Resolution of symptoms does not correlate with regression of Barrett’s

Surveillance a) Surveillance allows detection at an early stage and improves long-term survival b) Endoscopy at least every year

Surgical treatment of benign disease

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a) Indications = failure of medical tx or complications (same as for GERD) b) Unknown whether regression of Barrett’s or reversion of dysplasia can be expected c) Fundoplication and resection if necessary i) Remove all Barrett’s mucosa ii) Cervical esophagogastrostomy (intrathoracic esophagogastrostomy carries a high d) Surgical treatment of dysplasia and adenocarcinoma i) High grade dysplasia (separate from carcinoma in situ) ii) Intensive med tx w/rebiopsy q 3months or resection iii) When resecting, remove all Barrett’s epithelium Malignant Tumors

Esophageal Cancer Palliation

Of patients amenable to resection, 72% have LN mets, 25% are resectable for cure

I. Palliative resection or bypass A. Bypass

1. Mortality=20-40% 2. Morbidity 25% 3. Orringer noted increased incidence of anastomotic leak with bypass 4. Retrosternal route offers best conduit to neck

a) Portals for palliative RT can avoid neoesophagus b) Reduces possibilty for malignant dysphagia c) Remove upper manubrium and clavicular heads to enlarge thoracic inlet & decrease anastomotoic leak and stricture

5. Substernal if retrosternal unavailable 6. Gastric-emptying procedure recommended 7. Kirschner - Roux-en-Y drainage of esophageal remnant - others say it is unnecessary

B. Indications 1. Esophagorespiratory fistula in young, fit pts

a) Avoids constant aspiration b) Other option is esophageal intubation

2. Disease unresectable for cure (discovered at operation) when bypass or resection will add little to morbidity

Summary A. Palliation reserved for: 1. Medically unfit for resection 2. Incurable disease on pre-op evaluation 3. Unresectable at operation 4. Refusal of surgical tx

II. Esophageal dilatation A. Multiple dilatations and return trips to the hospital are usually required B. Mercury-filled, red rubber (Maloney or Hurst) are most common

1. Short strictures with visible lumen 2. Fluouroscopy increases safety

C. Guide-wire systems 1. For long, angulated, or eccentric strictures 2. Puestow - supplanted by hollow-core polyvinyl (American, Savary-Gillard) 3. Require endoscopic or fluouroscopic placement of guide-wire into stomach

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D. Balloons 1. Potentially decreased risk of perforation (radial vs. Vector force!)

E. Dilate to max 45 French

I. Esophageal intubation (intentional) A. Tubes have a proximal funnel and a lumen >= 10mm B. Traction method (Celestin tube)

1. Surgical procedure 2. Pilot bougie is passed through gastrotomy and tube is sutured to lesser curve over a teflon pledget

C. Pulsion method 1. Shorter hospital stay (8.4 vs. 18.6 days) and lower mortality (14% vs. 23%) c/w traction 2. Tube is inserted endoscopically over a guidewire w/fluouroscopic control 3. Celestin tube, Atkinson, Wilson-Cook 4. Savary-wire reinforced polyvinyl - resistant to compression

D. Expandable wire stents 1. For Ca and esophagorespiratory fistula 2. Dilating tumor not nedcessary prior to insertion 3. Problems: expense, inability to move after placement, tumor ingrowth

E. Complications 1. Perforation 4-12%, 4-9% of patients die.

a) Surgery usually ontraindicated b) Tx w/NPO, IV Abx, nutrition

2. Reflux esophagitis a) Elevate HOB b) Omeprazole

3. Tube displacement 10-20% 4. Tumor overgrowth

a) Nd:YAG ablation b) Replacement w/longer tube

I. Chemotherapy A. Single agents: 5-FU, cisplatin 15-20% response rate B. Multi-drug regimens (+/- RT) 33-50% and up to 77% respectively C. 44% life-threatening side effects

II. Brachytherapy A. Radioactive sources afterloaded into fluouroscopically placed PVC catheters B. 3500 cGy at surface of catheter, 1500cGy 1cm from center C. Benefits

1. Cesium-137 and iridium 192 - more exact dosing 2. Proximity to tumor minimizes radiation to normal tissues

D. Patients with dysphagia due to extrinsic malignant compression more likely to fail E. 75% have improvement in dysphagia score F. 1-yr survival: 10% for SCC, 20% for adeno G. Complications = sore throat, esophagitis, epigastric pain H. Combined w/external beam (Flores) complete restoration of swallowing in 62%

1. Radiation esophagitis common I. Compared to laser tx: shorter hospital stay and less likely to require

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re-treatment III. Laser photoablation A. Nd:YAG is most common B. Works by tissue vaporization and thermal necrosis C. Authors recommend:

1. Snare cautery debridement of exophytic tumor first 2. Routine re-treatment 2-4 days post-procedure

D. All tumor types, any location - exophytic more successful, extrinsic compression less successful E. Relatively safe, effective (80%), improves quality of life F. Complications (1-2.7% mortality)

1. Related to experience 2. Minor - 10-50% 3. Perforation - 5% (experienced operator), bleeding 4% (tx w/laser)

IV. Photodynamic therapy A. IV photosensitizing agents (hematoporphyrin derivatives & phthallocyanines) B. Dye lasers tuned to appropraite wavelength 2-3 days later - photochemical prrocess C. Mean survival 6.8 mo D. Complications

1. Photosensitivity 2. Failure of tx

Molecular Biology of Lung and Esophageal Cancer Introduction 1. tobacco consumption is believed to be responsible for > 90% of lung cancers in men and 80 % in women 2. tobacco has been implicated in the development of esophageal carcinoma acting synergistically with ethanol consumption 3. since only a minority of smokers develop lung cancer implies that there are other genetic and enviromental factors that contribute 4. epidemiologic studies have demonstrated familial risk of lung cancer in some patients who develop cancer at an earlier age (<50 Yrs) 5. in the U.S., the incidence of esophageal adenocarcinoma has risen faster than any other cancer a. tends to occur in young male patients with no history of tobacco or ethanol abuse b. chronic reflux, hiatal hernia, and Barrett’s esophagus are thought to be predisposing factors c. Barrett’s esophagus increases the risk of developing esophageal cancer 40-fold

Cell Cycle 1. cell proliferation normally proceeds in an orderly fashion (GO,G1,S,G2 and M phases) 2. multiple regulatory proteins known as cyclins (A,B etc) are involved in cell proliferation

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3. perturbation of cell cycle integrity due to alterations in various cyclin levels due to mutations involving oncogenes or tumor suppressor genes induces genomic instability, DNA amplification, and malignant transformation

Dominant and Recessive Oncogenes 1. mutations associated with carcinogenesis may occur in dominant or recessive (tumor suppressor) genes 2. dominant oncogenes are genes in which mutation results in constitutive growth stimulation 3. tumor suppressor genes tend to control cell proliferation Growth Factors and Growth Factor Receptors 1. lung and esophageal cancers have been associated with abnormal expression of a variety of growth factors and growth factor receptors 2. growth factors secreted by tumor cells may influence distant cells (endocrine stimulation)

Gastrin-Releasing Peptide 1. 27 amino acid homolog of bombesin 2. GRP receptors are found on small cell cancer cells but are absent on non-small cell cancer cells 3. in vitro proliferation of small cells can be inhibited by antibombesin monoclonal antibodies or antagonists

Epidermal Growth Factor Receptor 1. 170-kd tyrosine kinase glycoprotein 2. activaiton of EGFr ligand results in cell proliferation 3. overexpression of EGFr has been associated with 45% lung cancers and 80% of esophageal cancers 4. EGFr overexpression in patients with non-small cell cancers is associated with diminished survival

ErbB2/Neu 1. erbB2/neu gene encodes for a 185 kd transmembrane tyrosine kinase receptor molecule 2. structurally related to EGF 3. present on normal ciliated epithelium, mucus cells, and type II pneumocytes of the lung 4. overexpression had been associated with scca and adenoca of the lung

Platelet-derived and Insulin-like Growth Factors and Their Respective Receptors 1. both are important cell cycle progression in mammalian cells 2. PDGF, PDGFr expression have both been documented in association lung and esophageal cancers 3. normal lung tissue does not express PDGF ligand, but lung cancer cells do express PDGF 4. insulin growth factor ligands and receptors have been identified in lung and esophageal cancer cell lines 5. IGF-I,IGF-II or insulin stimulated mitogenesis in lung or esophageal cancer lines can be competitively inhibited by Mabs to the receptors or ligands

Cyclin D 1. the cyclin D gene appears to be amplified but not expressed in ~ 10% of large cell and squamous cell lung cancers 2. amplification and overexpression is seen in 32% of squamous cell esophageal cancers 3. overexpression of cyclin D disrupts G1 cell cylce kinetics

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resulting in damaged DNA and malignant transformation RAS 1. H,K, and N-ras genes are members of a super gene family encoding for plasma membrane proteins that are important in signal transduction from cell surface receptors invovled in mitogen-induced proliferation 2. Ras mutations are among the most common oncogene defects in human cancers 3. K-ras mutations are relatively common in pulmonary adenocarcinomas, esp. in patients with a smoking history

Myc 1. family of genes which are expressed during mammalian development and human carniogenesis 2. encode for DNA transcription factors that are critical for initiating movement of (G0) cells into and through the G1 phase of the cell cycle initiating DNA synthesis 3. aberrant C-myc expression has been primarily documented in small cell cancers 4. myc amplification occurs late in the process of carcinogenesis, enhancing tumor progression and metastasis

Tumor Suppressor Genes 3p 1. lung and esophageal cancers are associated with multiple genetic alterations 2. the majority of these neoplasms are aneuploid 3. deletions of 3p have been detected in nearly 100% of small cell lung cancers and >50% of non-small cell tumors and ~ 70% of esophageal cancers

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Spring 2016 8. The Mediastinum

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8 The Mediastinum

Table of Contents

8 The Mediastinum ...................................... 2 Anatomy ................................................................ 4 Congenital Malformations Of The Mediastinum (Primary Cysts) ...................................................... 4

Pleuropericardial Cysts ...................................... 4 Bronchogenic Cysts ........................................... 4 Esophageal/Enteric Cysts .................................. 4

Mediastinal Imfections ........................................... 4 Mediastinitis ........................................................ 3 Mediastinal Hemorrhage .................................... 3

Superior Vena Cava Syndrome (SVC Syndrome) . 3 Primary Mediastinal Tumors .................................. 5

Mediastinal Thymoma ........................................... 6 Thymic Carcinoid ............................................... 7

Mediastinal Lymphomas (HL) ................................ 7 Mediastinal Germ Cell Tumors (GCTs) ................. 7 Mediastinal Endocrine Tumors .............................. 8

Intrathoracic Thryoid .......................................... 8 Parathyroid ......................................................... 9

Mediastinal Primary Cysts ..................................... 9 Bronchogenic Cysts ........................................... 9 Esophageal/Enteric Cysts .................................. 9 Pleuropericardial Cysts ...................................... 9

Mediastinal Neurogenic Tumors ............................ 9

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Chapter Objective

At the end of this chapter the resident understands the anatomic, embryologic, physiologic and the pathogenesis and management of mediastinal diseases.

Learner Objectives Upon completion of this chapter the resident: 1. understands the anatomical and radiological compartments, as

well as the the lymphatic drainage; 2. understands the etiology, evaluation, differential diagnosis, and

diagnostic criteria for congenital malformations of the mediastinum;

3. knows the operative management of infections of the mediastinum;

4. knows the pathogenesis and management of the superior vena cava syndrome;

5. knows the pathogenesis and management of the primary mediastinal tumors;

6. understands the pathogenesis and management of the thymic abnormalities;

7. knows the pathogenesis and management of mediastinal lymphomas (hl & nhl);

8. understands the pathogenesis and management of primary mediastinal germ cell tumors (gcts);

9. knows the pathogenesis and management of endocrine tumos (thyroid & parathyroid);

10. understands the pathogenesis and management of the neurogenic tumors (intercostal nerve, sympathetic ganglia and para-sympathetic cells).

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Anatomy A. Compartments · Mediastinal borders: thoracic inlet (superior), diaphragm (inferior), sternum (anterior), spine (posterior), pleura (lateral) · Anterosuperior compartment is anterior to pericardium · Contents include thymus and great vessels · Middle, or visceral, compartment is between anterior and posterior pericardial reflections · Contents include heart, phrenic nerves, tracheal bifurcation, major bronchi, lymph nodes · Posterior, or paravertebral, compartment is posterior to posterior pericardial reflection · Contents include esophagus, vagus nerves, sympathetic chains, thoracic duct, descending aorta, and azygos/hemiazygos


Textbook Chapters Chapter 37: The Mediastinum. Glenn's Thoracic and Cardiovascular Surgery (Baue, Geha, Hammond, Laks, and Naunheim), 6th ed., 658-9.

Congenital Malformations Of The Mediastinum (Primary Cysts)

Pleuropericardial Cysts · Uncommon, classically occur at the pericardiophrenic angles, 70-80% on the R. side · Usually asymptomatic and may communicate with the pericardium · Guided needle aspiration is the initial therapy of choice · Surgical excision is indicated if the cyst recurs or if the diagnosis is in doubt

Mediastinal Cysts Congenital • Mesothelial cysts: Pericardial, Pleural • Foregut cysts: Bronchogenic, Esophageal, Gastroenteric. • Lymphatic cysts: Lymphangiomatous, Thoracic duct Acquired • Inflammatory • Thymic • T eratogenous • Dermoid • Parathyroid • Thyroid

Bronchogenic Cysts · Most common primary cysts in the mediastinum (5%) · Arise from ventral foregut and are usually located in the subcarinal or R. paratracheal region/a> · Two-thirds are asymptomatic; symptoms include tracheobronchial or esophageal compression and infection from tracheobronchial communication · Complete excision is recommended, even if asymptomatic, to prevent late complications

Esophageal/Enteric Cysts · Comprise 3-5% of mediastinal tumors · More common in children and tend to occur in the lower third of the esophagus · Dysphagia is the most common symptom · CT scanning is essential in patients with vertebral anomalies to evaluate for possible spinal cord involvement (neuroenteric cyst) · Avoid endoscopic biopsy, as this may cause cyst perforation and infection · Complete excision is indicated; a thoracoscopic approach can be used for uninfected cysts

Mediastinal Imfections

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Mediastinitis

· Occurs in about 1% of patients after median sternotomy · Risk factors include prolonged surgery or CPB, re-exploration, wound dehiscence, shock, and use of bilateral internal mammary artery grafts in patients who are older or have diabetes · Presents as fever, elevated WBC, and tachycardia · Best treatment results with wound debridement and tissue flaps

Braxton JH. Marrin CA. McGrath PD. et al Northern New England Cardiovascular Disease Study Group. Mediastinitis and long-term survival after coronary artery bypass graft surgery. Ann Thorac Surg. 70(6):2004-7, 2000 Dec.

Catarino PA. Chamberlain MH. WR. NC. et al. High-pressure suction drainage via a polyurethane foam in the management of poststernotomy mediastinitis. Ann Thorac Surg. 70(6):1891-5, 2000 Dec.

Berg HF. Brands WG. van Geldorp TR. Et al/ Comparison between closed drainage techniques for the treatment of postoperative mediastinitis. Ann Thorac Surg. 70(3):924-9, 2000 Sep.

Baskett RJ. MacDougall CE. Ross DB. Is mediastinitis a preventable complication? A 10-year review. Ann Thorac Surg. 67(2):462-5, 1999 Feb.

Milano CA. Georgiade G. Muhlbaier LH. Smith PK. Wolfe WG. Comparison of omental and pectoralis flaps for poststernotomy mediastinitis. Ann Thorac Surg. 67(2):377-80; discussion 380-1, 1999 Feb.

Bitkover CY. Gardlund B. Mediastinitis after cardiovascular operations: a case-control study of risk factors. Ann Thorac Surg. 65(1):36-40, 1998 Jan.

Mossad SB. Serkey JM. Longworth DL. Cosgrove DM 3rd. Gordon SM. Coagulase-negative staphylococcal sternal wound infections after open heart operations. Ann Thorac Surg. 63(2):395-401, 1997 Feb.

Loop FD. Lytle BW. Cosgrove DM. Mahfood S. McHenry MC. Goormastic M. Stewart RW. Golding LA. Taylor PC. J. Maxwell Chamberlain memorial paper. Sternal wound complications after isolated coronary artery bypass grafting: early and late mortality, morbidity, and cost of care. Ann Thorac Surg. 49(2):179-86; 1990 Feb.

Francel TJ. Kouchoukos NT. A rational approach to wound difficulties after sternotomy: the problem. Ann Thorac Surg. 72(4):1411-8, 2001 Oct.

Francel TJ. Kouchoukos NT. A rational approach to wound difficulties after sternotomy: reconstruction and long-term results. Ann Thorac Surg. 72(4):1419-29, 2001 Oct.

Mediastinal Hemorrhage

· Caused by trauma, aortic dissection, aneurysm rupture, or surgical procedures · May result in mediastinal tamponade, which is more insidious than pericardial tamponade · Meticulous hemostasis and adequate chest tube drainage will prevent this syndrome · Spontaneous mediastinal hemorrhage can result from mediastinal masses, altered coagulation status, and severe hypertension

Superior Vena Cava Syndrome (SVC Syndrome) SVC Obstruction and Collateral a) Obstruction below azygous vein · Azygous - hemiazygous, lumbar veins to IVC b) Obstruction above azygous vein · Venous collateral in neck to azygous to SVC c) Obstruction includes azygous vein · Internal mammary, paraspinous, esophageal and subcutaneous vein

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to IVC d) Cerebral decompression through a single jugular vein via midline intracranial venous sinuses Pathogenesis a) Extrinsic compression of SVC · Gradual SVC obstruction b) Invasion of SVC · Obstruction develops rapidly c) Thrombosis of SVC · Acute obstruction d) Venous hypertension and lymphatic obstruction - all empty into the subclavian veins

Abner, A. Approach to the patient who presents with superior vena cava obstruction [review]. Chest 1993;103 (4 Suppl):394S-397S.

Jackson, JE and Brooks, DM. Stenting of superior vena caval obstruction [review]. Thorax 1995;50 Suppl 1:S31-6.

Jahangri, M and Goldstraw, P. The role of mediastinoscopy in superior vena caval obstruction. Annals of Thoracic Surgery 1995;59:453-5.

Lochridge, SK; Knibbe, WP; and Doty, DB. Obstruction of the superior vena cava. Surgery 1979;85:14-24.

Causes a) Benign 10% · Inflammatory - histoplasmosis, idiopathic fibrosing mediastinitis · Iatrogenic - pacemaker electrode, hyperalimentation or other CV line b) Malignant 90% · Bronchogenic, epidermoid 65-80% · Small cell 12-30% · Lymphoma 12-20% Symptoms and Signs · Swelling face, neck, arms · Shortness of breath, orthopnea, cough and chest pain suggest upper airway obstruction · Hoarseness, stridor, tongue swelling, nasal congestion · Headaches, syncope and lethargy are caused by cerebral edema from venous hypertension · Symptoms worse lying down, bending forward · Symptoms of cerebral or laryngeal edema is associated witha reduced life expectancy of about 6 weeks, demanding urgent intervention · Caval obstruction may be the life-limiting problem of patients with underlying malignancy Diagnosis a) Chest x-ray · R. hilar mass - bronchogenic carcinoma · Anterior mediastinal mass - lymphoma · Calcification - histoplasmosis b) Simultaneous bilateral arm venogram · Defines obstruction and collateral circulation · Identifies thrombus c) Computerized axial tomography · Assessment of mediastinum · Determine patency of jugular veins · Directed needle biopsy

Radiation Therapy · Since most cases due to malignancy, nearly all patients receive radiation · 80-90% relieved of SVC Syndrome · 50% of patients relapse · Relapse occurs in benign disease as well; although collaterals develop, thrombosis will continue to propogate and occlude these

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collaterals over time Medical Therapy · Chemotherapy for lymphomas and small cell carcinoma · Diuretics and corticosteroids reduce cerebral edema · Anticoagulants in selected cases to prevent clot propagation · Thrombolytic therapy for selected acute thrombosis

Surgery · Severe SVC Syndrome associated with thrombosis of caval tributaries and inadequate collateral circulation · SVC bypass with composite autogenous vein grafts or PTFE 6-12 months after onset in benign causes or for palliation in malignant causes with severe or acute onset SVC syndrome

Primary Mediastinal Tumors Location · Lesions are predictable to some degree predictable · Most common tumors are neurogenic (20%), thymomas (20%), primary cysts (20%), lymphomas (13%), and germ-cell tumors (10%) · Most are located in anterosuperior compartment (54%), followed by posterior (26%) and middle (20%) tumors Mediastinal Tumors

Tumors and Cysts by Location Anterior Middle Posterior Thymoma Germ cell tumor Lymphoma Hemangioma Parathyroid adenoma Thymic cyst Lipoma Aberrant thyroid Lymphangioma

Enterogenous cyst Mesothelial cyst Lymphoma Thoracic duct cyst Granuloma Hamartoma

Neurogenic origin Neurenteric cyst Lymphoma

· A significant portion (25-40%) of mediastinal tumors are malignant · Anterosuperior tumors are more likely to be malignant, as are tumors of patients between the ages of 10 and 40 · Neurogenic tumors and non-Hodgkin's lymphomas are the most common tumors in children

Schlumberger Classification (TABLE, 8-4). • Tumors of nervous tissues • Tumors of hemopoietic

tissue and lymphoreticular tissue.

• Tumors of mesenchymal derivatives.

• Cysts of the mediastinum • Miscellaneous space

consuming lesions

Clinical Presentation · About two-thirds of patients will have symptoms at the time of diagnosis · The absence of symptoms is a reasonably good indicator that a diagnosed tumor is benign · Most common symptoms include chest pain, cough, and fever · Signs of mechanical compression or invasion of mediastinal structures are more common with malignant tumors · Paraneoplastic syndromes are not uncommon and include Cushing's syndrome, thyrotoxicosis, hypertension, hypercalcemia,

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hypoglycemia, diarrhea, and gynecomastia Diagnosis · CXR will localize the tumor and give information on calcification and relative density of the tumor · CT scanning identifies chest wall invasion, multiple masses, and extension into spinal column · MRI is more accurate for vascular involvement and intracardiac pathology · Echocardiography is useful for patients with middle compartment tumors to localize between intracardiac and pericardial tumors · Guided needle biopsy can make a diagnosis of malignancy in 80-90% of patients · Mediastinoscopy/mediastinotomy may be necessary to make a diagnosis and establish resectability

Mediastinal Thymoma Features · Represents 20% of all mediastinal masses in adults · Peak incidence is in 3rd to 5th decades of life; rare in children · About half are of mixed cell type, followed by epithelial (28%) and lymphocytic (20%) types · Between 15 and 65% of thymomas are benign · Frequently associated with paraneoplastic syndrome, most commonly myasthenia gravis · Myasthenia gravis is diagnosed in 30-50% of patients with a thymoma, and 15% of myasthenia patients will have a thymoma · Autoimmune reaction directed against the postsynaptic nicotinic receptors results in skeletal muscle fatigability and weakness, especially in axial muscles

Blumberg D. Port JL. Weksler B. et al. Thymoma: a multivariate analysis of factors predicting survival. Ann Thorac Surg. 60(4):908-13; discussion 914, 1995 Oct.

Bril V. Kojic J. Ilse WK. Cooper JD. Long-term clinical outcome after transcervical thymectomy for myasthenia gravis. Ann Thorac Surg. 65(6):1520-2, 1998 Jun.

Ferguson MK. Transcervical thymectomy. Semin Thorac Cardiovasc Surg. 11(1):59-64, 1999 Jan.

Graeber GM. Tamim W. Current status of the diagnosis and treatment of thymoma. Semin Thorac Cardiovasc Surg. 12(4):268-77, 2000 Oct.

Moore KH. McKenzie PR. Kennedy CW. McCaughan BC. Thymoma: trends over time. Ann Thorac Surg. 72(1):203-7, 2001 Jul.

Regnard JF. Magdeleinat P. Dromer C. et al. Prognostic factors and long-term results after thymoma resection: a series of 307 patients. J Thorac Cardiovasc Surg. 112(2):376-84, 1996 Aug.

Wilkins EW Jr. Grillo HC. Scannell JG. Moncure AC. Mathisen DJ. J. Maxwell Chamberlain Memorial Paper. Role of staging in prognosis and management of thymoma. Ann Thorac Surg. 51(6):888-92, 1991 Jun.

Operative Technique · Remove all anterior mediastinal tissue and any invasive disease, including involved lung, pleura, pericardium, and SVC/innominate vein · Thymic blood supply arises from the internal mammary arteries · Patients with stage IIa or higher disease should receive postoperative radiation · Chemotherapy is indicated for stage III or IV disease · Debulking may be appropriate for stage IV disease, although there is no evidence for increased survival · At 5 years after resection, 25-30% of patients will have complete resolution of myasthenia symptoms and 30-50% will be improved · Prognosis is dependent on stage of tumor, not on presence of myasthenia gravis

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Thymic Carcinoid

· Most occur in males and about two-thirds are symptomatic · Originate from Kulchitsky cells in the thymus, but are not associated with myasthenia gravis or the carcinoid syndrome · May cause other paraneoplastic syndromes, however, most commonly Cushing's syndrome (33%) · Presence of such syndromes is a very poor prognostic factor · Up to 75% will develop local recurrence or metastases · Low overall cure rate and mean survival is 3 years

de Montpreville VT. Macchiarini P. Dulmet E. Thymic neuroendocrine carcinoma (carcinoid): a clinic-pathologic study of fourteen cases. J Thorac Cardiovasc Surg. 111(1):134-41, 1996 Jan.

Economopoulos GC. Lewis JW Jr. Lee MW. Silverman NA. Carcinoid tumors of the thymus. Ann Thorac Surg. 50(1):58-61, 1990 Jul.

Fukai I. Masaoka A. Fujii Y. et al.. Thymic neuroendocrine tumor (thymic carcinoid): a clinicopathologic study in 15 patients. Ann Thorac Surg. 67(1):208-11, 1999 Jan.

Mediastinal Lymphomas (HL) The WHO classification divides HL into 2 main types: A. Lymphocyte-predominant Hodgkin lymphoma (LPHL) B. Classical Hodgkin lymphoma (CHL). CHL is divided into 4 subtypes: • Nodular sclerosis CHL (NSCHL) • Mixed cellularity CHL (MCCHL) • Lymphocyte-depleted CHL (LDCHL) • Lymphocyte-rich CHL (LRCHL).

Ricci C. Rendina EA. Venuta F. et al. Surgical approach to isolated mediastinal lymphoma. J Thorac Cardiovasc Surg. 99(4):691-5, 1990 Apr.

Yellin A. Pak HY. Burke JS. Benfield JR. Surgical management of lymphomas involving the chest. Ann Thorac Surg. 44(4):363-9, 1987 Oct.

Between 40 and 70% of lymphoma patients will have mediastinal involvement during their disease course · Only 5-10% of lymphoma patients will have isolated mediastinal disease, and are usually symptomatic · Characteristic Hodgkin's lymphoma symptoms are chest pain after alcohol consumption and cyclic Pel-Ebstein fevers · Nodular sclerosing and lymphocyte predominance forms of Hodgkin's lymphoma are the most common to cause mediastinal involvement · Up to 40% of patients with lymphoblastic non-Hodgkin's lymphoma will have mediastinal disease · Surgery is indicated if fine-needle aspiration is inconclusive or to evaluate residual mass after chemotherapy · Surgical options include cervical mediastinoscopy, parasternal mediastinotomy, and thoracoscopy Mediastinal Germ Cell Tumors (GCTs) Classification of Mediastinal GCTs • Mature teratoma • Immature teratoma • Teratomas with malignant component: Type I: with an associated GCT (seminoma, etc.) Type II: with another epithelial malignancy (squamous, adeno-carcinoma, etc.) Type III: with sarcomatous component (rhabdomyosarcoma, osteosarcoma, etc.) Type IV: a combination of any of the above • Seminoma

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• Yolk sac tumor (endodermal sinus tumor) • Embryonal carcinoma • Choriocarcinoma Combined GCT without teratomatous component . Comprise 15-25% of anterior mediastinal masses · Most common in children and young adults · Includes teratomas, teratocarcinomas, seminomas, embryonal cell carcinomas, choriocarcinomas, and endodermal cell or yolk-sac tumors · Identical to germ cell tumors originating in the gonads, but are not metastatic lesions from primary gonadal tumors · About 60% are benign and 40% are malignant

Bacha EA. Chapelier AR. Macchiarini P. Fadel E. Dartevelle PG. Surgery for invasive primary mediastinal tumors. Ann Thorac Surg. 66(1):234-9, 1998 Jul.

Knapp RH. Hurt RD. Payne WS. Farrow GM. Lewis BD. Hahn RG. Muhm JR. Earle JD. Malignant germ cell tumors of the mediastinum. J Thorac Cardiovasc Surg. 89(1):82-9, 1985 Jan.

WR. CD. Kesler KA. Nichols CR. Mahomed Y. Einhorn LH. Miller ME. Brown JW. Primary mediastinal nonseminomatous germ cell tumors. Results of a multimodality approach. J Thorac Cardiovasc Surg. 99(2):210-7, 1990 Feb.

A. Predominantly Benign Tumors · Teratomas are complex, multiple tissue element tumors · Symptoms are related to mechanical effects · Simplest form is the dermoid cyst, which consists of mostly dermal and epidermal tissue · More complex teratomas may have well-differentiated bone, cartilage, nerve, or glandular tissue · Malignant tumors are differentiated upon histologic identification of embryonic tissue

B. Malignant Tumors · Male predominance and most patients are symptomatic · 40% are seminomas and 60% are nonseminomas (embryonal cell, choriocarcinoma, yolk-sac, and teratocarcinoma)

Seminomas & Nonseminomasof the Mediastinum Seminomas Non-seminomas AFP/B-HCG rare 90% Associated syndromes

none Klinefelter's, trisomy 8, 5q deletion

Radiosensitivity High Insensitive Metastatic behavior

Remain intrathoracic

Frequently disseminated

Treatment Radiation Cis-platinum chemotherapy

Remission Over 80% CR in 55-60%, PR in 30-35%

5-year survival 50-80% 50-60% Remission CR=complete PR=partial

· Initial surgical intervention typically only for diagnosis due to high radiosensitivity of seminomas and frequent metastatic disease in non-seminomas · Surgical resection after induction of chemotherapy may have a role in non-seminomatous tumors

Mediastinal Endocrine Tumors

Intrathoracic Thryoid Hall TS. Caslowitz P. Popper C.

Smith GW. Substernal goiter versus intrathoracic aberrant thyroid: a critical difference. Ann Thorac Surg. · 80% are substernal extensions of a cervical goiter

· True intrathoracic thyroid (derives blood supply from thoracic

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vessels) comprises only 1% of all mediastinal tumors · More common in women and in the 6th to 7th decades, most are adenomas · Usually presents with tracheal or esophageal compression; thyrotoxicosis is uncommon · I-131 scanning should be done to identify presence of functioning cervical thyroid tissue before resecting these tumors · Resect substernal extensions through a cervical incision and true intrathoracic lesions through the chest

46(6):684-5, 1988 Dec. Mitchell JD. Donnelly RJ.

Retrosternal thyroid. Semin Thorac Cardiovasc Surg. 4(1):34-8, 1992 Jan.

Protopapas AD. Nicholson AG. Vini L. Harmer CL. Goldstraw P. Thoracic metastasectomy in thyroid malignancies. Ann Thorac Surg. 72(6):1906-8, 2001 Dec.

Parathyroid · Most are adenomas and are found by the superior pole of the thymus due to common embryogenesis from the third branchial cL. · Symptoms are usually due to hyperparathyroid syndrome · Parathyroid cysts are not usually hormonally active Mediastinal Primary Cysts

Bronchogenic Cysts

· Most common primary cysts in the mediastinum (5%) · Arise from ventral foregut and are usually located in the subcarinal or R. paratracheal region/a> · Two-thirds are asymptomatic; symptoms include tracheobronchial or esophageal compression and infection from tracheobronchial communication · Complete excision is recommended, even if asymptomatic, to prevent late complications

Demmy TL, Krasna MJ,

Detterbeck FC, Kline GG, Kohman LJ, DeCamp MM Jr, Wain JC. Multicenter VATS experience with mediastinal tumors. Ann Thorac Surg. 1998 Jul;66(1):187-92.

Hazelrigg SR, Landreneau RJ, Mack MJ, Acuff TE. Thoracoscopic resection of mediastinal cysts. Ann Thorac Surg. 1993 Sep;56(3):659-60.

Kelemen JJ 3rd, Naunheim KS. Minimally invasive approaches to mediastinal neoplasms. Semin Thorac Cardiovasc Surg. 2000 Oct;12(4):301-6.

Urschel JD, Horan TA. Mediastinoscopic treatment of mediastinal cysts. Ann Thorac Surg. 1994 Dec;58(6):1698-700.

Esophageal/Enteric Cysts · Comprise 3-5% of mediastinal tumors · More common in children and tend to occur in the lower third of the esophagus · Dysphagia is the most common symptom · CT scanning is essential in patients with vertebral anomalies to evaluate for possible spinal cord involvement (neuroenteric cyst) · Avoid endoscopic biopsy, as this may cause cyst perforation and infection · Complete excision is indicated; a thoracoscopic approach can be used for uninfected cysts

Pleuropericardial Cysts · Uncommon, classically occur at the pericardiophrenic angles, 70-80% on the R. side · Usually asymptomatic and may communicate with the pericardium · Guided needle aspiration is the initial therapy of choice · Surgical excision is indicated if the cyst recurs or if the diagnosis is in doubt

Mediastinal Neurogenic Tumors

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Etiology and Diagnosis · Most posterior mediastinal masses are of neurogenic origin · 95% of these tumors in adults are benign and are usually asymptomatic · In children, most neurogenic tumors are malignant · Classified according to cell origin; most arise from intercostal nerve or sympathetic chain Neurogenic Tumors Intercostal nerve Sympathetic ganglia Paraganglia cells Neurofibroma Neurilemoma Neurofibrosarcoma

Ganglioma Ganglioneuroblastoma Neuroblastoma

Paraganglioma (pheochromocytoma)

· Neurilemomas are the most common and originate from Schwann's cells · These are encapsulated tumors which stain with S-100 protein immunostain · Two primary types: Antoni A (organized pallisading pattern) and Antoni B (loose reticular pattern) · Neurofibromas originate from peripheral nerve · Form a pseudocapsule and have more variability with the S-100 stain · Both types of tumors are associated with von Recklinghausen's disease, although more commonly neurofibromas · Chest CT is sufficient for diagnosis of most of these tumors, and MRI should be used when an intraspinal component is present

Bousamra M 2nd, Haasler GB, Patterson GA, Roper CL. A comparative study of thoracoscopic vs open removal of benign neurogenic mediastinal tumors. Chest. 1996 Jun;109(6):1461-5.

Reeder LB. Neurogenic tumors of the mediastinum. Semin Thorac Cardiovasc Surg. 2000 Oct;12(4):261-7.

Operative Indications · Benign tumors (neurofibroma, neurilemoma, ganglioneuroma) can be effectively treated with local excision · Combined thoracic and neurosurgical approach is indicated for tumors with intraspinal extension · Recurrence is rare for benign tumors · Local recurrence is common for malignant tumors and overall prognosis is poor

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9. 9 Mostafa, Ezzeldin A; Elnahas, Yasser. Ain Shams Lecture Notes on Cardiovascular and Thoracic Surgery Volume 1: Fundamentals of Cardiac Surgery, 2e, 196 pages Elnasr Publishing Co for Digital Education. 2016 ISBN: 978.977.90.5817.7

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