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CONGENITAL DIAPHRAGMATIC HERNIA Maj Asrar Ahmad MBBS, FCPS

CONGENITAL DIAPHRAGMATIC HERNIA Maj Asrar Ahmad MBBS, FCPS. Anatomy. 5 %. 95 %. Embryology. Pathophysiology. Pathophysiology. Pulmonary hypoplasia “ compression theory ” - modeled in fetal lambs - rationale for early surgery “ global embryopathy ” - modeled in newborn rats

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CONGENITAL DIAPHRAGMATIC HERNIA Maj Asrar Ahmad MBBS, FCPS

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  1. CONGENITAL DIAPHRAGMATIC HERNIAMaj Asrar AhmadMBBS, FCPS

  2. Anatomy 5 % 95 %

  3. Embryology

  4. Pathophysiology

  5. 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

  6. Incidence • 1:2500-5000 live births • 1100 cases in the U.S. annually • 80 % Left side survival remains around ~65%

  7. Diagnosis • Antenatal: • U/S at ~20 weeks gestation • ~60% • Polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratio

  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

  9. Initial Management • Oxygenate but avoid barotrauma • Intubate • Sedate • NGT for decompression

  10. Medical Management • Medical emergency not surgical • Pulmonary vasodilators • Inotropes • High frequency oscillatory ventilation • ECMO • Surfactant • Antenatal steroids?

  11. ECMO

  12. ECMO

  13. Surgical Management

  14. Surgical Management

  15. Surgical Management

  16. Surgical Management

  17. Developing Therapies • Fetal surgery • PLUG fetal surgery • Growth factors

  18. THANK YOU

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