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 MALE = FEMALE 80% Left 20% Right B/L Rare Risk of recurrence in first degree relative is 2%

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

PATHO-PHYSIOLOGY

Patho-physio - contd

Patho-physio - contd: Pulmonary hypertension

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

Patho-physio contd : persistence of (R) to (L) shunting

DIAGNOSIS Prenatal diagnosis – U/S

DIAGNOSIS Prenatal diagnosis -MRI

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

INVESTIGATION For diagnosis

Investigation - contd

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

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

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.

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

TREATMENT Aim Prevention is better than cure Treat the defect Reverse the pulmonary hypertension

Prenatal intervention - open fetal surgery

Prenatal intervention – contd fetoscopic surgery

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

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

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

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

POSTNATAL – E.C.MO

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

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

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%

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

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

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

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

SURGICAL ASPECTS

SURGICAL ASPECTS - VATS

DISCHARGE

MORGAGNI HERNIA

Eventration of the diaphragm

KHALLAS -- SHUKARAN