YY Chee, S Chim, P Beh, KY Wong

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.

肺泡血管發育不良（ACD）是罕見的和被認為是新生兒肺動脈高壓的致命型。我們報導一名患有中度臍膨出於產後10小時併發呼吸窘迫的男嬰，儘管提供最大的通氣和吸入性氧化一氮，但他的病情迅速惡化。ACD是按屍體解剖結果來確定的，ACD與臍膨出和腸扭轉的關係從未經報導，即使同時存在其它呼吸衰竭的風險因素，我們也建議把ACD納入難治性新生兒持續肺動脈高壓的鑑別診斷。