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Prenat Diagn. 2006 Nov;26(11):1058-61.

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

Author information

1
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

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.

DISCUSSION:

Review of pathology, clinical course, and genetics of CPL.

CONCLUSION:

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.

PMID:
16941717
DOI:
10.1002/pd.1555
[Indexed for MEDLINE]

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