congenital diaphragmatic hernia maj asrar ahmad mbbs, fcps
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CONGENITAL DIAPHRAGMATIC
HERNIA
Maj Asrar AhmadMBBS, FCPS
Anatomy
95 %
5 %
Embryology
Pathophysiology
Pathophysiology Pulmonary hypoplasia“compression theory”
- modeled in fetal lambs- rationale for early surgery
“global embryopathy”- modeled in newborn rats- rationale for new therapeutic ideas
Pulmonary hypertension - causes persistent fetal circulation
Incidence 1:2500-5000 live births 1100 cases in the U.S.
annually 80 % Left side
survival remains around ~65%
Diagnosis Antenatal:
U/S at ~20 weeks gestation ~60%
Polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratio
Presentation Shortness of breath Scaphoid abdomen
Three general presentations:Severe respiratory distress at the time of birth.Respiratory deterioration hours after delivery
Benefit from correction of hypoxemia and pulmonary hypertension
Feeding difficulties, chronic respiratory disease, pneumonia
10-20 % intestinal obstruction
Initial Management Oxygenate but avoid barotrauma
Intubate
Sedate
NGT for decompression
Medical Management Medical emergency not surgical
Pulmonary vasodilators Inotropes High frequency oscillatory ventilation ECMO Surfactant Antenatal steroids?
ECMO
ECMO
Surgical Management
Surgical Management
Surgical Management
Surgical Management
Developing Therapies Fetal surgery
PLUG fetal surgery
Growth factors
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