Congenital alveolar capillary dysplasia and associated gastrointestinal anomalies

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Capillaries / abnormalities*
  • Capillaries / pathology
  • Congenital Abnormalities / pathology*
  • Extracorporeal Membrane Oxygenation
  • Female
  • Gastrointestinal Tract / abnormalities*
  • Gastrointestinal Tract / pathology
  • Hirschsprung Disease / etiology
  • Hirschsprung Disease / pathology
  • Humans
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / pathology
  • Infant, Newborn
  • Intestinal Atresia / etiology
  • Intestinal Atresia / pathology
  • Male
  • Pulmonary Alveoli / abnormalities*
  • Pulmonary Alveoli / blood supply*
  • Pulmonary Alveoli / pathology
  • Pulmonary Circulation