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CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

CONGENITAL DIAPHRAGMATIC HERNIA ( C D H ). Dr JACOB MATHEW DEPT. OF PAED. SURGERY Dr AHMED ABANAMY HOSPITAL. NORMAL DIAPHRAGM. C D H. Development of diaphragm 4 th to 8 th week. Development of Lung. Correlation of diaphragmatic defect and lung development. INCIDENCE. 1 : 2000 – 3000

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CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

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  1. CONGENITAL DIAPHRAGMATIC HERNIA ( C D H ) Dr JACOB MATHEW DEPT. OF PAED. SURGERY Dr AHMED ABANAMY HOSPITAL

  2. NORMAL DIAPHRAGM

  3. C D H

  4. Development of diaphragm4th to 8th week

  5. Development of Lung

  6. Correlation of diaphragmatic defect and lung development

  7. INCIDENCE • 1 : 2000 – 3000 • MALE = FEMALE • 80% Left • 20% Right • B/L Rare • Risk of recurrence in first degree relative is 2%

  8. CAUSE • We do not know • Exposure to phenmetriazine, thalidomide, quinine, nitrofen and Vitamin A deficiency • Genetic influences • Associated with chromosomal deletion(XO) chromosomal duplication-Trisomy 21,18,13 • Most cases non-syndromatic, isolated

  9. PATHO-PHYSIOLOGY

  10. Patho-physio - contd

  11. Patho-physio - contd: Pulmonary hypertension

  12. Patho-physio -contd • Known stimulators of pulmonary hypertension • Hypoxia • Hypothermia • Stress • Acidosis

  13. Patho-physiocontd : persistence of (R) to (L) shunting

  14. DIAGNOSISPrenatal diagnosis – U/S

  15. DIAGNOSIS Prenatal diagnosis -MRI

  16. Post-natal diagnosis • Respiratory symptoms at birth • Respiratory symptoms within 24 hours • Poor respiratory efforts, gasping • Cyanosis, decreased peripheral perfusion • Scaphoid abdomen • Asymmetric funnel chest • Bowel sounds in the chest • 10% - 20% late presentation

  17. INVESTIGATIONFor diagnosis

  18. Investigation - contd

  19. Investigation – associated anomalies • Echocardiogram – Cardiac defect • Cranial U/S - Neural tube defects • Abdomen U/S - Renal anomalies

  20. PROGNOSTIC FACTORS - Prenatal • Lung to head ratio (LHR) : >1.4 –Better prognosis, < 1 – very poor prognosis • Liver position • Position of stomach • Prenatal diagnosis • Polyhydramnios • (R) sided defect

  21. Prognostic factors -Prenatal contd • ASSOCIATED ANOMALIES : Chromosomal anomalies and serious cardiac defects have a negative impact while defects like solitary kidney, mal-rotation have no bearing on the prognosis.

  22. PROGNOSTIC FACTORS - Postnatal • PHYSIOLOGICAL PARAMETERS : Blood gas analysis : PO2( N : 50-80 ), PCO2( N : 35-45 ), pH( N : 7.25-7.45 ) • PROGNOSTIC INDICES : Calculated from ventilator parameters and blood gas analysis • V.I = RR × MAP × PaCO2 { < 1000 } • MVI = RR × PIP × PaCO2 ÷ 1000 { < 40 ; > 80 } • O.I = MAP × FiO2 ÷PaO2 {<0.06;0.175}

  23. TREATMENTAim • Prevention is better than cure • Treat the defect • Reverse the pulmonary hypertension

  24. Prenatal intervention -open fetal surgery

  25. Prenatal intervention – contdfetoscopic surgery

  26. Postnatal intervention • Surfactant • Nitric oxide • Sildenafil • Extracorporeal membrane oxygenation • Delayed surgery • Conventional ventilation • High frequency oscillatory ventilation

  27. POSTNATAL - SURFACTANT • Primary surfactant deficiency unlikely • CDH study group reports an overall potential for worse outcome in surfactant treated patients

  28. POSTNATAL – NITRIC OXIDE • Expected to have a dramatic effect on pulmonary hypertension in CDH • A recent Cochrane review found no clear data to support the use of inhaled nitric oxide in infants who have CDH

  29. POSTNATAL - SILDENAFIL • Decreases pulmonary vascular resistance • Maybe of some unique benefit but insufficient data exists to support it’s use currently

  30. POSTNATAL – E.C.MO

  31. POSTNATAL – E.C.M.O

  32. POSTNATAL – E.C.M.O • Rescue therapy after corrective surgery • Improved survival in CDH patients who had a predicted mortality of > 80 % • Now used more for pre-operative stabilisation • A Cochrane review concluded that ECMO offers short term benefits but overall effect of using ECMO remains unclear

  33. POSTNATAL -Delayed Surgery • Once considered a surgical emergency • Delay in surgery is not harmful hence there is no compelling reason to perform emergent surgery at birth • Now stabilization and delay of surgical repair is widely accepted

  34. POSTNATAL - VENTILATION • Hyperventilation and induced alkalosis were treatment norms in late 80’s and 90’s • “Gentle ventilation” pioneered by Wung and colleagues • Avoid hyperventilation and limit inflation pressure to < 25 cm of water • Survival rates improved from 40% to 89%

  35. POSTNATAL – High frequency oscillatory ventilation • High survival rates in CDH have been achieved by some centers • Lung protective ventilation must be provided to optimize CDH survival

  36. TREATMENT PROTOCOL –Prenatal • Investigate for associated anomalies • Ante-natal counseling • Normal delivery close to term

  37. TREATMENT PROTOCOL –in our hospital • Naso-gastric tube • Pre-ductal arterial line • I/V fluids • AVOID HYPOTHERMIA, HYPOPERFUSION,HYPOGLYCEMIA AND HYPOCALCEMIA • Endo-tracheal intubation and “gentle ventilation” • Sedation

  38. TREATMENT PROTOCOL –in our hospital • Investigations for anomalies • Delayed surgery • Post – op ventilation • Discharge

  39. SURGICAL ASPECTS

  40. SURGICAL ASPECTS

  41. SURGICAL ASPECTS - VATS

  42. DISCHARGE

  43. MORGAGNI HERNIA

  44. Eventration of the diaphragm

  45. KHALLAS -- SHUKARAN

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