Pediatric Surgery

ddx of double bubble sign

• ddx of double bubble sign:

• duodenal atresia

• malrotation

• annular pancreas

• MUST DO UGI (or LGI) contrast study to

R/O malrotation (surgical emergency)

gastroschisis Omphalocele

General considerations

• Daily maintenance fluids: 100ml/kg/d 0 – 10 kg 4ml/kg/h first 10 kg

50ml/kg/d 10 – 20 kg 2ml/kg/h second 10 kg

25ml/kg/d >20kg 1ml/kg/h each add. kg

• Central venous access

– External jugular, facial, saphenous veins

• Blood volume / replacement

– Total blood volume = 85ml/kg

– Transfusion = 10ml/kg (similar to 500ml in adult)

Pyloric stenosis

• Most common cause of vomiting in infancy

• Typically 4-6 weeks old, male, first born

• Projectile, nonbilious emesis with near complete obstruction

• Metabolic alkalosis, ↓K+, Cl-

• Urine pH ↑ initially, then ↓ as H+ ions excreted for Na in distal tubule

• PE – classic “olive” in RUQ, visible gastric waves

• US can make diagnosis in 95% of cases when PE unclear

• Must first fluid resuscitate (D5W, 0.45NS, +2-4meq/kg K+ at a rate of 150-175ml/h over 24hrs)

• Freder- Ramstedt pyloromyotomy = seromuscular layers of the pylorus excised down to submucosal layer allowing mucosa to herniate through incision

• Oral feedings resumed post-op, gradually increasing strength and volume, usually discharge in 48 hours

Lesions of the NeckThyroglossal duct cyst

• Thyroid develops at base of tongue (foreamen caecum), migrates

anterior and caudal

• Remnants of the thyroid migration may persist in midline of neck

• Usually apparent in children age 2-4yo when baby fat subsides

• Moves up and down with swallowing and tongue protrusion

• May become infected, needs I&D, then resection

• May be ectopic thyroid tissue, must feel normal thyroid first or do scan

• Resection (Sistrunk operation) involves removal of tissue, tract up to

base of tongue, midportion of hyoid bone, ligation of foramen cecum

Lesions of the NeckBrachial cleft anomalies

• Usually remnant of second brachial cleft

• Presents as fistula tract form pharynx to anterior border of SCM

• Typically drains clear fluid

• Tract extends superiorly from skin through carotid bifurcation to posterior

lateral pharynx at base of tonsillar fossa

• Surgical treatment only cure, must excise entire tract

• Step ladder incisions better cosmetically, use probe or dye to follow

Lesions of the NeckCystic hygroma

• Results from sequestration or obstruction of development of lymph channels

• Most common sites are posterior triangle of neck, axilla, groin, mediastinum

• Usually multiple cysts containing lymph fluid, cause local distortion

• May be present at birth, usually appear in first two years of life

• Can become infected with Staph or Strep → percutaneous drain

• Radical excision not advised in this benign lesion

• Conservative resection with unroofing of cysts indicated, may go back and

do re-excision to preserve vital structures

Congenital Diaphragmatic Hernia

(Bochdalek)

• Occurs when pleuroperitoneal canal does not close resulting in posteriolateral defect, commonly on left side (Morgagni - anterolateral)

• Abdominal cavity small, poorly developed, scaphoid

• Lungs hypoplastic bilaterally, decreased weight, volume and vasculature

• Can be detected by US as early as 15 weeks gestation

• Infants born hypoxic, hypercarbic, acidotic, persistent pulmonary HTN

• Immediate surgery usually not performed, placed on ventilator

• ECMO (extracorporeal membrane oxygenation) often needed to support infant 1-3 weeks

• Surgical approach through abdomen, 3/4 require synthetic patch

• May need to make abdominal wall defect or silo temporarily

Congenital Lobar Emphysema

• Results from intrinsic poor bronchial cartilage development

• Causes trapping of air and hyperexpansion of affected lung tissue

• Occurs in upper lobes the most, then middle, then lower lobes

– LUL (40%) > RML (35%) > RUL (20%) > lower lobes (<1%)

• Affected lobe over inflates and compresses normal lung

• Symptoms range from mild respiratory distress to complete failure

• CXR diagnostic showing hyperlucent affected side, compressed

adjacent lobes and shifted mediastinum to unaffected side

• 14% of children have associated cardiac defects

• Treatment is resection of affected lobe

Other Respiratory Disorders

• Pulmonary sequestration– Extralobar = Nonaerated, systemic blood supply, above left

diaphragm, usually asymptomatic, no tx necessary

– Intralobar = Within lung parenchyma, usually left side, also nonaerated, arterial supply from aorta below diaphragm, often causes infection, venous drainage to pulmonary vein, may need resection of entire L lower lobe to cure

• Bronchogenic cyst– Hamartomas, occur anywhere in respiratory tract, usually simple

cysts with respiratory epithelium, cartilage, smooth muscle

– May be asymptomatic, cause infection or airway compression

– CXR or CT shows dense mass; tx is simple cyst resection

Foreign Bodies

• Respiratory

– Usually toddler age, peanut most common object, hyperlucency

of affected lobe seen

– Most often ends up in R main stem bronchus or R lower lobe

– Mild respiratory distress or unilateral wheezing seen

– Remove with bronchoscopy

• Esophagus

– Toddlers, most commonly coins, may require contrast swallow

– Lodges in one of three places where the esophagus narrows –

cricipharyngeus, aortic arch, GE junction

– May cause drooling (inability to swallow), often asymptomatic

– Usually requires UGI endoscopy for removal

Esophageal atresia

Tracheoesophageal fistula

• Occurs when esophagus and tracheal fail to divide into

separate tubes at around 5 weeks gestation

• Catagorized by Gross-Vogt into five types

– A = atresia without fistula

– B = atresia with TEF between proximal esophagus and trachea

– C = atresia with TEF between distal esophagus and trachea

– D = atresia with TEF between both proximal & distal esophagus

– E = TEF without atresia (H type)

• 85% are type C, allows air in stomach and aspiration

• Congenital anomalies common (VATER)

Vertebral Anorectal TracheoEsophageal Renal

Esophageal atresia

Tracheoesophageal fistula cont.

• Clinical manifestations → regurgitation and drooling,

feedings followed by choking or coughing, abdomen

either distended (TEF present) or scaphoid (no TEF)

• Diagnosis may be made with prenetal US, or by a tube

coiled in the upper pouch

• Contrast media may be given to determine the type of

atresia/TEF

• Need to evaluate the condition of the lungs for

pneumonia/atelectasis, also echo heart for defects

Esophageal atresia

Tracheoesophageal fistula cont.

• Initial tx consists of: 1)warmer, 2) 30° head elevated,

3) IV access. 4)AB, 5)sump drainage proximal pouch

• Primary surgical correction

– Performed if baby stable, R retroperitoneal approach

– Divide TEF, close tracheal opening, mobilize esophageal ends

– Single layer primary anastomosis, drain, no gastrostomy

• Delayed surgical repair

– If baby unstable, premature, multiple anomalies

– First place gastrostomy until baby mature, stable

– Do standard primary repair

Esophageal atresia

Tracheoesophageal fistula cont.

• Post-operative course

– Parenteral nutrition for one week

– Contrast swallowing study to check for leak

– Continue drainage if leak present (will heal especially if done retropleural)

• Prognosis – 90% survive if stable, >60% if not

• Strictures develop in 10-20% of cases (especially if a leak)

– May occur months to years later

– Usually treatable with multiple dilatations

• “Recurrent” TEF

– Usually due to missed TEF or leak

• Reflux

– May develop clinical symptoms as child

– Tx with anti-reflux procedure (Nissen) but dysmotility may persist

Duodenal malformations

• Incomplete obstruction – web,anular pancreas, midgut volvulus

• Complete obstruction – atresia

– Polyhydramnios usually 3rd trimester

– Bilious vomiting (85%)

– No abdominal distention

– Classic “double bubble” seen

– One third have Down’s syndrome (trisomy 21)

– Tx = NG/OG tube, IVF, doudenoduodenostomy after evaluating for other abnormalities

• Tx anular pancreas = do not divide, duodenojejunostomy

• Tx web = excise through vertical incision, close horizontally

Midgut volvulus

• Midgut in umbilical cord 6-12 weeks

• Normally rotates 270° around SMA when returning into abdominal cavity

• If incomplete, cecum stays in epigastrum, but Ladd’s bands still form between the cecum and lateral abdominal wall crossing duodenum

• SMA is in a narrow pedicle and a volvulus may occur around the mesentery with jejunal obstruction and ischemia (clockwise)

• Must be ruled out in infant with bilious vomiting → UGI series

– Shows displacement of duodenal-jejunal junction to right

– May show corkscrew pattern of duodenum or complete obstruction

• Early surgical intervention mandatory due to risk of entire midgut necrosis

– Bowel untwisted counter-clockwise

– Ladd’s bands lysed

– Appendectomy (cecum usually ends up in LLQ)

– Frankly necrotic bowel resected or second-look operation done

Meconium ileus

• Occurs from meconium impaction in distal ileum

• Etiology usually cystic fibrosis causing a lack of

pancreatic enzymes

• Presents with bilious vomiting late, failure to pass

meconium

• Films show dilated loops of SB, no air/fluid levels

• Classic sign is “ground glass” appearance in terminal

ileum

• Tx non-operatively with dilute Gastrografin enema

• May need to repeat every 12 hours for several days

Necrotizing enterocolitis (NEC)

• Disease found mostly in stressed premature infants

• Common factors include intestinal ischemia, bacterial colonization of the gut, enteric feedings of synthetic formula, hypoalbuninemia

• First sign usually feeding intolerance (vomiting, large residuals)

• Invasion of the mucosa by gas-forming organisms causes “pneumatosis intestinalis” or hepato/portal gas

• Initial treatment = NPO, NG, IVAB

• Indications for surgery 1) free air 2) diffuse peritonitis 3) refractory acidosis, especially with hepato/portal air

• Resection of gangrenous bowel done with stomas, may need second looks

• If infant (<1500 grams) desperately ill → consider local drainage at bedside

• TPN continued for 2 weeks minimum

• 20% develop strictures (need to evaluate before restoring continuity of bowel), up to 80% survival now

Intussusception

• Usually in infants 8-12 weeks old, male

• Commonly occurs after viral illness 2 hypertrophy of Peyer’s

patches in terminal ileum

• May occur due to tumors, polyps, Meckel’s diverticulum

• Symptoms include cramping abdominal pain, vomiting, lethargy

• May pass bloody mucous “currant jelly”

• Classic finding is elongated mass in RUQ

• Tx is air enema using pressure less than 120 mmHG (60-90%

successful)

• If unsuccessful→ surgery and manual reduction, appy

• Occasionally need to resect gangrenous bowel, or cause of leading

point

Meckel’s diverticulum

• Remnant of ophalomesenteric (vitelline) duct

• On antimesenteric border 2 feet from ileocecal valve

• May mimic appendicitis; bleed if gastric mucosa present;

cause intussusception

• Can use technetium scan to diagnosis if gastric mucosa

present

• Tx is wedge resection if base is narrow, sleeve resection

for larger base

Imperforate anus

• Results from failure of decent of the urorectal septum

• “High” ends above levator ani muscle, “low” descends

partially through it

• Low lesions have fistula to perineum; midline of

scrotum/penis in males, posterior forchette in females

• Low lesions (40% in males, 70% in females) approached

through perineal incision, usually pts continent

• High lesions require colostomy, then pull-through

procedure at 2 months

• Tx : posterior lateral anorectoplasty

Hirshsprung’s disease

• Absence of ganglion cells in myenteric plexus of intestine, usually rectum or rectosigmoid

• Cause functional obstruction, variable degree

• Often failure to pass meconium in first 24 hours

• Barium enema shows transition zone (lg to sm), unreliable in infants

• Suction biopsy usually adequate to demonstrate lack of ganglion cells (may need full thickness)

• Also shows increase cholinesterase staining, hypertrophied nerve bundles

• Three pull-through procedures used with similar results

1) Swenson – rectum resected, colo-anal anatomosis

2) Duhamel – colon anastomosed to posterior rectum above anus

3) Soave – rectal mucosa resected and colon brought through sleeve

Omphalocele

• Abdominal wall defect at umbilicus

• Herniated bowel covered with peritoneum

• Frequently (60-70%) associated with other anomalies

• Tx: primary closure of abdominal wall, mechanical

stretching, may need silo

• Silo used to steadily apply pressure over 7 days to

reduce hernia contents

• 20-30% mortality due to associated cardiac and

chromosomal abnormalities

Gastroschisis

• Full thickness abdominal wall defect just right of

umbilicus

• Results form intrauterine rupture of umbilical cord

• Herniated bowel not covered with peritoneum

• Rarely associated with other anomalies

• Intestine usually thickened, edematous, discolored,

covered with exudate

• Transport in bowel bag to keep moist & normothermic

• Tx: emergent primary closure of abdominal wall,

mechanical stretching, may need silo

• Survival rate 90%

Wilm’s tumor (nephroblastoma)

• Most common renal malignancy of childhood

• Peak incidence between 1-5 years

• Typical X-ray finding: linear calcifications

• Tx: resection, chemo, radiation

• Clamp renal vein prior to mobilization to prevent spread

• May do resection of adjacent organ if needed

• Actinomycin D and vincristine useful

• 80-97% cure rate, depending on stage

• Long term follow-up required due to ↑ second

malignancies

Neuroblastoma

• Arises from sympathetic nervous tissue (neural crest cells)

• 3rd most common malignancy in children (#1 brain, #2 leukemia)

• Most common tumor in children < 1 yr

• Originates in adrenals, posterior mediastinum, pelvis, neck, etc.

• X-ray finding: fine-stippled calcification

• Best localizing test: MIBG scan

• Usually elevated catacholamines, VMA and HVMA in urine

• Tx: Resection, chemo, radiation

• Best chance of cure is complete surgical resection

• Better prognosis the younger the patient (85% if < 1 yr)

• Spontaneous regression can occur

Biliary tract abnormalities

• Choledochal cysts

– 5 types, type 1 most common, fusiform common duct dilation

– Classic triad includes pain, RUQ mass, jaundice

– Untreated leads to cirrhosis, portal htn, cholangitis, cancer

– Tx: resection of cyst, choledocho – jejunostomy or duodenostomy

• Biliary atresia

– May involve common duct, hepatic ducts, GB, cystic duct, etc.

– Causes jaundice, growth retardation, portal htn

– Kasai procedure (hepatoportoenterostomy) best tx

Kasai procedure