CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

Dr JACOB MATHEWDEPT. OF PAED. SURGERY

Dr AHMED ABANAMY HOSPITAL

NORMAL DIAPHRAGM

C D H

Development of diaphragm4th to 8th 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

DIAGNOSISPrenatal 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

INVESTIGATIONFor 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 : 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}

TREATMENTAim

• Prevention is better than cure

• Treat the defect • Reverse the pulmonary hypertension

Prenatal intervention -open fetal surgery

Prenatal intervention – contdfetoscopic 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

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

SURGICAL ASPECTS - VATS

DISCHARGE

MORGAGNI HERNIA

Eventration of the diaphragm

KHALLAS -- SHUKARAN