Slide 1

CONGENITAL DIAPHRAGMATIC HERNIA Maj Asrar Ahmad MBBS, FCPS Slide 2 Anatomy 95 % 5 % Slide 3 Embryology Slide 4 Pathophysiology Slide 5 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 Slide 6 Incidence 1 :2500-5000 live births 100 cases in the U.S. annually 8 0 % Left side survival remains around ~65% Slide 7 Diagnosis Antenatal: U/S at ~20 weeks gestation ~60% Polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratio Slide 8 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 Slide 9 Slide 10 Initial Management Oxygenate but avoid barotrauma Intubate Sedate NGT for decompression Slide 11 Medical Management Medical emergency not surgical Pulmonary vasodilators Inotropes High frequency oscillatory ventilation ECMO Surfactant Antenatal steroids? Slide 12 ECMO Slide 13 Slide 14 Surgical Management Slide 15 Slide 16 Slide 17 Slide 18 Developing Therapies Fetal surgery PLUG fetal surgery Growth factors Slide 19 Slide 20