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Diaphragmatic hernia
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- 3. DEVELOPMENT OF LUNGS Factoraffecting Lung Development • Lung liquid • Amniotic fluid volume • Intra thoracic space availability • Fetal breathing
- 4. DEFINITION • A diaphragmatichernia is defined as a communication between the abdominal and thoracic cavities with or without abdominal contents in thorax.
- 5. TYPES ETIOLOGY • Congenital • Traumatic ANATOMICALLY •Hiatal (esophageal hiatus) • Paraesophageal • Retrosternal (Morgagni) • Posterolateral ( Bochdalek) CONGENITAL DIAPHRAGMATIC HERNIA
- 6. 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,
- 7. 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
- 8. DIAGNOSIS • Prenatal USGat 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
- 9. X RAY UPPERGI STUDY
- 10. 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
- 11. Differential Diagnosis • Cysticlung lesion( pulmonary sequestration, cystic adenoid malformation Pulmonary sequestration • Eventration of Diaphragm • Bronchogenic cyst • Neurogenic Tumors • Primary Lung Sarcoma
- 12. TREATMENT INITIAL MANAGEMENT • Aggressiverespiratory 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)
- 13. VENTILATORY STRATEGIES • Conventionalmechanical ventillation • HFOV • ECMO GOAL • Oxygenation without barotrauma • PIP=<25 cm of water • Rate 30-60 breathes/min
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- 15. Nitric oxide • Selectivepulmonary vasodilator ECMO(Extracorporeal Membrane Oxygenation) • Lower limit of weight required>=2000g • Can be venoarterial and venovenous
- 16. SURGICAL REPAIR • Mostlyafter 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%
- 18. RECENT ADVANCES • Liquidventilation : 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)
- 19. 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
- 20. PROGNOSIS POOR PROGNOSIS • Overallsurvival of CDH 67% • Spontaneous fetal demise: 7-10% • Associated major anomalies • Symptoms before 24 hrs of age • Severe pulmonary hypoplasia • Need for ECMO
- 21. REFERENCES • Nelson- TextBook of Paediatrics-19th edition • Langman’s Medical Embryology- 11th edition
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