Diaphragmatic hernia
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Transcript of Diaphragmatic hernia
DIAPHRAGMATIC HERNIA
SUDARSHAN PANDEYINTERN , KUSMS
DEVELOPMENT OF DIAPHRAGM
DEVELOPMENT OF LUNGSFactor affecting Lung
Development• Lung liquid• Amniotic fluid volume• Intra thoracic space
availability• Fetal breathing
DEFINITION• A diaphragmatic hernia is defined as a communication
between the abdominal and thoracic cavities with or without abdominal contents in thorax.
TYPES
ETIOLOGY • Congenital• Traumatic
ANATOMICALLY• Hiatal (esophageal hiatus)• Paraesophageal • Retrosternal (Morgagni)• Posterolateral ( Bochdalek)
CONGENITAL DIAPHRAGMATIC HERNIA
CONGENITAL DIAPHRAGMATIC HERNIA
EPIDEMIOLOGY
• INCIDENCE: 1/2000- 1/5000 live births
• M:F – 1:2• Left side more common
(85%)• B/L <5%• Sporadic( most cases) • Familial ( autosomal
recessive, multifactorial)
ASSOCIATED ANOMALIES• CNS lesions• Omphalocele• Esophageal atresia• Cardiovascular lesions• Part of trisomy 21, trisomy
13, trisomy 18, fryns,
SIGNS AND SYMPTOMS• Respiratory distress (tachypnea,
grunting, cyanosis, use of accessory muscles – cardinal sign
• Scaphoid abdomen • Increased chest wall diameter• Bowel sound heard in chest
with decreased/absent breath sounds on side of hernia
• Shifting of apex beat contralateral side of hernia
DELAYED PRESENTATION• Often right side• Regurgitation• Vomiting( d/t intestinal
obstruction)• Incarcination of intestine• ischemia sepsis
and shock
DIAGNOSIS • Prenatal USG at 16-24 WOG:
polyhydramnios, chest mass, mediastinal shift, gastric bubble in thoracic cavity
• After delivery: Chest X Ray with nasogastric tube
• Barium study• Echocardiography • Amniocentesis
X RAY UPPER GI STUDY
PROGNOSTIC IMAGING FEATURE
• Lung to head size ratio(LHR)• LHR<1 : POOR SURVIVAL• LHR>1.4: INCREASED
SURVIVAL• Liver in thoracic cavity: poor
prognosis
LHR= LUNG AREA/HC
Differential Diagnosis• Cystic lung lesion( pulmonary sequestration, cystic adenoid
malformation Pulmonary sequestration• Eventration of Diaphragm• Bronchogenic cyst• Neurogenic Tumors• Primary Lung Sarcoma
TREATMENT
INITIAL MANAGEMENT• Aggressive respiratory support
( rapid endotracheal intubation, sedation
• NG tube insertion and urinary catheterization needed
• Pre- and postductal oxygenation and arterial pressure have to be monitored continuously by umbilical arterial catheter placement
• Prolonged Bag and mask contraindicated
GOAL• Pre ductal SaO2>= 85%• PIP<25 cm of water• Permissive hypercapnia
PaCO2 (45- 60 mm Hg)
VENTILATORY STRATEGIES• Conventional mechanical
ventillation• HFOV• ECMO
GOAL• Oxygenation without
barotrauma• PIP=<25 cm of water• Rate 30-60 breathes/min
CMV vs HFOV
Nitric oxide• Selective pulmonary
vasodilator
ECMO(Extracorporeal Membrane Oxygenation)
• Lower limit of weight required>=2000g
• Can be venoarterial and venovenous
SURGICAL REPAIR• Mostly after 48 hrs after
stabilization and resolution of pulmonary hypertension
• Delayed in newborn on HFOV• Most common approach :
subcostal approach• Laparoscopic and thoracoscopic
repair• Native tissue vs patch(GORE-
TEX, porous polytetrafluroethylene patch)
Relative indicators for stability• Requirement of
conventional ventilation only
• Low PIP• FiO2<50%
RECENT ADVANCES
• Liquid ventilation : Tracheal installation of perfluorocarbon (PFC) to replace nitrogen as a carriage for oxygen and carbon dioxide.
• Fetal surgery: Human Tracheal occlusion (fetoscopic endoluminal tracheal occlusion, FETO)
COMPLICATION
• GERD (50%)• Intestinal Obstruction (20%)• Recurrent diaphragmatic hernia (5-20%)• Delayed growth in 1st 2 years of life• Neurocognitive defect (more common in
infants requiring ECMO)• Pectus excavatum• Scoliosis
PROGNOSIS
POOR PROGNOSIS
• Overall survival of CDH 67%• Spontaneous fetal demise: 7-10%
• Associated major anomalies• Symptoms before 24 hrs of age• Severe pulmonary hypoplasia• Need for ECMO
REFERENCES
• Nelson- Text Book of Paediatrics-19th edition• Langman’s Medical Embryology- 11th edition
THANK YOU