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CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

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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 ... – PowerPoint PPT presentation

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Title: CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )


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-physio contd 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) gt1.4 Better
    prognosis, lt 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 lt 1000
  • MVI RR PIP PaCO2 1000 lt 40 gt 80
  • O.I MAP FiO2 PaO2
    lt0.060.175

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 its 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 gt 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 80s and 90s
  • Gentle ventilation pioneered by Wung and
    colleagues
  • Avoid hyperventilation and limit inflation
    pressure to lt 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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