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Alveolar capillary dysplasia with misalignment of the pulmonary veins: a rare but fatal cause of neonatal respiratory failure.

Abstract
Respiratory failure in the neonate that is refractory to maximal medical management is a frequent indication for extracorporeal life support (ECLS). Alveolar capillary dysplasia with misalignment of the pulmonary veins is an irreversible cause of respiratory failure that cannot be diagnosed on clinical grounds alone and would not be expected to respond to ECLS therapy. A recent experience with a patient prompted us to review the literature regarding this condition for the purpose of identifying factors suggestive of this diagnosis. This condition should be considered in neonates with presumed pulmonary hypertension who cannot be weaned from ECLS. If the diagnosis is made by antemortem open-lung biopsy, a costly, protracted, and unnecessary continued course of ECLS may be avoided.
AuthorsT D Kane, J M Greenberg, K E Bove, B W Warner
JournalPediatric surgery international (Pediatr Surg Int) Vol. 14 Issue 1-2 Pg. 89-91 (Nov 1998) ISSN: 0179-0358 [Print] Germany
PMID9880707 (Publication Type: Case Reports, Journal Article)
Topics
  • Capillaries (abnormalities)
  • Extracorporeal Membrane Oxygenation
  • Female
  • Humans
  • Infant, Newborn
  • Persistent Fetal Circulation Syndrome (complications)
  • Pulmonary Alveoli (blood supply)
  • Pulmonary Veins (abnormalities)
  • Respiratory Insufficiency (etiology, mortality, therapy)

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