congenital diaphragmatic hernia ( c d h ) dr jacob mathew dept. of paed. surgery dr ahmed abanamy...
TRANSCRIPT
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