Alveolar capillary dysplasia associated with omphalocoele

Abstract

Alveolar capillary dysplasia (ACD) is a rare and universally fatal form of persistent pulmonary hypertension of the newborn (PPHN). We report a case of a newborn male baby with a moderate sized omphalocoele who developed respiratory distress 10 hours after birth. He deteriorated and succumbed shortly afterwards despite maximal ventilatory support and inhaled nitric oxide. ACD was diagnosed at postmortem examination, and the association of ACD with omphalocoele and gut malrotation has not been reported. We suggest including ACD in the differential diagnosis of refractory PPHN in a newborn even with the presence of other risk factors for respiratory failure.