title: Omphalocele and alveolar capillary dysplasia: a new association.
abstract: OBJECTIVE
  First report of an infant with coexistent omphalocele and alveolar capillary dysplasia.
  
  
  METHODS
  Descriptive case report.
  
  
  METHODS
  Neonatal intensive care unit of a tertiary care children's hospital.
  
  
  METHODS
  We describe a term infant with omphalocele and respiratory insufficiency attributable to pulmonary hypertension. The patient was placed on extracorporeal membrane oxygenation, but the pulmonary hypertension persisted. After 10 days on extracorporeal membrane oxygenation, a lung biopsy was performed. It showed alveolar capillary dysplasia. Because of the lethal prognosis, extracorporeal membrane oxygenation was withdrawn and the patient expired.
  
  
  CONCLUSIONS
  This is the first description of an association between omphalocele and alveolar capillary dysplasia. In newborns with omphalocele who have severe respiratory insufficiency and pulmonary hypertension, alveolar capillary dysplasia should be considered.
authors.affiliation: Department of Neonatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Luella C
authors.initials: LC
authors.lastname: Gerrits
authors.forename: Amerik C
authors.initials: AC
authors.lastname: De Mol
authors.forename: Johan
authors.initials: J
authors.lastname: Bulten
authors.forename: Frans H
authors.initials: FH
authors.lastname: Van der Staak
authors.forename: Arno F J
authors.initials: AF
authors.lastname: Van Heijst
mesh_terms: D001997:Bronchopulmonary Dysplasia / Q000235:genetics; D002196:Capillaries / Q000002:abnormalities* / Q000473:pathology; D015897:Comorbidity; D015199:Extracorporeal Membrane Oxygenation; D017809:Fatal Outcome; D006554:Hernia, Umbilical / Q000473:pathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011650:Pulmonary Alveoli / Q000098:blood supply / Q000473:pathology*; D011667:Pulmonary Veins / Q000002:abnormalities*