Antao B, Samuel M, Kiely E, Spitz L, Malone M
Department of Paediatric Surgery, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Fetal Pediatr Pathol. 2006 May-Jun;25(3):137-45. doi: 10.1080/15513810600908230.
Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality. We present three cases of congenital alveolar capillary dysplasia with associated gastrointestinal abnormalities. Three full-term neonates presented with pulmonary hypertension needing ventilatory support by oscillation. Of the three, two neonates subsequently needed extracorporeal membrane oxygenation. Abdominal distension associated with bilious aspirates was the gastrointestinal manifestation. One child had duodenal atresia and anorectal anomaly, one with intestinal malrotation and the other with a rare combination of intestinal malrotaion and total colonic Hirschsprung's disease. All three infants succumbed to pulmonary hypertension at mean age 34 days. The etiopathogenesis and pathology of this condition are discussed with a comprehensive review of the literature.
先天性肺泡毛细血管发育不良是导致不可逆性肺动脉高压的罕见病因，死亡率达100%。我们报告三例伴有胃肠道异常的先天性肺泡毛细血管发育不良病例。三名足月儿出现肺动脉高压，需要通过振荡通气给予支持。其中两名新生儿随后需要体外膜肺氧合。伴有胆汁样吸出物的腹胀是胃肠道表现。一名患儿有十二指肠闭锁和肛门直肠畸形，一名有肠旋转不良，另一名有肠旋转不良与全结肠 Hirschsprung 病的罕见组合。所有三名婴儿均在平均34日龄时死于肺动脉高压。本文结合文献综述对该病的病因病机及病理进行了讨论。
