Transcript CONGENITAL DIAPHRAMATIC HERNIA ( C D H )

CONGENITAL DIAPHRAGMATIC
HERNIA ( C D H )
Dr JACOB MATHEW
DEPT. OF PAED. SURGERY
Dr AHMED ABANAMY HOSPITAL
NORMAL DIAPHRAGM
CDH
Development of diaphragm
4th 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
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 : 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}
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
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