Diaphragmatic hernia

diaphragmatic hernia

1. DIAPHRAGMATIC HERNIA SUDARSHAN PANDEY INTERN , KUSMS

2. DEVELOPMENT OF DIAPHRAGM

3. DEVELOPMENT OF LUNGS Factor affecting Lung Development Lung liquid Amniotic fluid volume Intra thoracic space availability Fetal breathing

4. DEFINITION A diaphragmatic hernia 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 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

9. X RAY UPPER GI 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 Cystic lung lesion( pulmonary sequestration, cystic adenoid malformation Pulmonary sequestration Eventration of Diaphragm Bronchogenic cyst Neurogenic Tumors Primary Lung Sarcoma

12. 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)

13. VENTILATORY STRATEGIES Conventional mechanical ventillation HFOV ECMO GOAL Oxygenation without barotrauma PIP=<25 cm of water Rate 30-60 breathes/min

14. CMV vs HFOV

15. Nitric oxide Selective pulmonary vasodilator ECMO(Extracorporeal Membrane Oxygenation) Lower limit of weight required>=2000g Can be venoarterial and venovenous

16. 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%

17. 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)

18. COMPLICATION GERD (50%) Intestinal Obstruction (20%) Recurrent diaphragmatic hernia (5-20%) Delayed growth in 1 st 2 years of life Neurocognitive defect (more common in infants requiring ECMO) Pectus excavatum Scoliosis

19. 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

20. REFERENCES Nelson- Text Book of Paediatrics-19 th edition Langman’s Medical Embryology- 11 th edition

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