title: Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.
abstract: OBJECTIVE
  Case report and literature review for congenital pulmonary lymphangiectasis (CPL) CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL.
  
  
  CONCLUSIONS
  Review of pathology, clinical course, and genetics of CPL.
  
  
  CONCLUSIONS
  This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.
authors.affiliation: The Center for Fetal Diagnosis and Treatment, Department of Surgery at The Children's Hospital of Philadelphia/University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4399, USA. wilsonrd@email.chop.edu
authors.forename: R Douglas
authors.initials: RD
authors.lastname: Wilson
authors.forename: Bruce
authors.initials: B
authors.lastname: Pawel
authors.forename: Michael
authors.initials: M
authors.lastname: Bebbington
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
authors.forename: Foong-Yen
authors.initials: FY
authors.lastname: Lim
authors.forename: David
authors.initials: D
authors.lastname: Stamilio
authors.forename: Angela
authors.initials: A
authors.lastname: Silber
authors.forename: Elaine
authors.initials: E
authors.lastname: Zakii
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
mesh_terms: D000328:Adult; D002916:Chylothorax / Q000151:congenital*; D003922:Diabetes Mellitus, Type 1 / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000473:pathology*; D008171:Lung Diseases / Q000151:congenital* / Q000473:pathology; D008200:Lymphangiectasis / Q000151:congenital* / Q000473:pathology; D008297:Male; D010996:Pleural Effusion / Q000151:congenital* / Q000196:embryology / Q000628:therapy; D011247:Pregnancy; D011254:Pregnancy in Diabetics