Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.
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.
Wilson
R Douglas
RD
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
Pawel
Bruce
B
Bebbington
Michael
M
Johnson
Mark P
MP
Lim
Foong-Yen
FY
Stamilio
David
D
Silber
Angela
A
Zakii
Elaine
E
Flake
Alan W
AW
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