1314 Congenital Diaphragmatic Hernia Associated with Esophageal Atresia: A Challenging Combination for the Neonatologist and the Pediatric Surgeon

Abstract

Introduction: Congenital diaphragmatic hernia(CDH) together with esophageal atresia (EA) areassociated with different malformations in up to71%. In epidemiologic studies the prevalence ofCDH was 0.13, of EA 0.27, of both combined 0.005per 1000 births.

Patients: In our single institution center for CDH wetreated 425 CDH patients. Associated malformationsoccurred in 39.5%, left sided hernia in 81.6%, rightsided hernia in 16.1%, both sided in 2.3% of allpatients. 5 patients suffered from EA together withCDH.

Results: Of the patients treated since 1996 we had5 patients with CDH and EA. One patient had atrisomy 18, one patient had severe lung hypoplasia, both died soon after birth. Chronic lung disease ledto death at the age of three years in one patient. Two patients survived. Ventilation and perfusiondifficulties of hypoplastic lungs occured in everypatient due to the tracheoesophagel fistula. In allpatients except the trisomy 18 patient we closed thetracheoesophageal fistula quickly after birth. Wepreferred temporary banding of the distal esophagusand early closure of the diaphragmatic defect from anabdominal access. In one case a severe hypoxemiaoccurred directly after operation. We decided towithdraw an ECMO rescue treatment. Esophagealatresia was reconstructed by thoracotomy in a laterstage.

Conclusion: The combination of EA and CDHis a challenging interdisciplinary problem. Thehigh mortality is due to the combination of thesemalformations. Rescue treatment by ECMO hasto be discussed because there exists a high risk oflater pulmonary morbidity.

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