Alveolar capillary dysplasia - Report of a case of prolonged life without extracorporeal membrane oxygenation (ECMO) and review of the literature

We describe an unusual infant with the diagnosis of alveolar capillary dysp lasia who had a relatively prolonged life without extracorporeal membrane o xygenation (ECMO). We have used this case as a springboard for a thorough r eview of the literature. This was a full-term female infant who presented w ith a picture of persistent pulmonary hypertension of the newborn. She was treated as such, with various ventilatory modes, alkalinizing agents, surfa ctant therapy, tolazoline, prostacyclin and nitric oxide. Because of the pr olonged clinical course the possibility of alveolar capillary dysplasia was raised. The parents refused ECMO. Despite all efforts she progressively de teriorated and died at 22 days of age. Macro- and microscopic examination o f the lung at autopsy were diagnostic of alveolar capillary dysplasia. A de tailed review of 39 cases published in the literature with comments regardi ng incidence, etiology, pathophysiology, clinical picture, diagnosis and tr eatment is presented. (C) 2000 Elsevier Science Ireland Ltd. All rights res erved.