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

R. Douglas Wilson; Bruce Pawel; Michael Bebbington; Mark P. Johnson; Foong Yen Lim; David Stamilio; Angela Silber; Elaine Zakii; Alan W. Flake

doi:10.1002/pd.1555

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

R. Douglas Wilson
, Bruce Pawel
, Michael Bebbington
, Mark P. Johnson
, Foong Yen Lim
, David Stamilio
, Angela Silber
, Elaine Zakii
, Alan W. Flake

Department of Obstetrics & Gynecology

Research output: Contribution to journal › Article › peer-review

26 Scopus citations

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.

Original language	English
Pages (from-to)	1058-1061
Number of pages	4
Journal	Prenatal Diagnosis
Volume	26
Issue number	11
DOIs	https://doi.org/10.1002/pd.1555
State	Published - Nov 2006

Keywords

Autosomal recessive
Congenital pulmonary lymphangiectasis
Perinatal mortality
Pleural effusion

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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. 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.",

keywords = "Autosomal recessive, Congenital pulmonary lymphangiectasis, Perinatal mortality, Pleural effusion",

author = "Wilson, \{R. Douglas\} and Bruce Pawel and Michael Bebbington and Johnson, \{Mark P.\} and Lim, \{Foong Yen\} and David Stamilio and Angela Silber and Elaine Zakii and Flake, \{Alan W.\}",

year = "2006",

month = nov,

doi = "10.1002/pd.1555",

language = "English",

volume = "26",

pages = "1058--1061",

journal = "Prenatal Diagnosis",

issn = "0197-3851",

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TY - JOUR

T1 - Congenital pulmonary lymphangiectasis sequence

T2 - A rare, heterogeneous, and lethal etiology for prenatal pleural effusion

AU - Wilson, R. Douglas

AU - Pawel, Bruce

AU - Bebbington, Michael

AU - Johnson, Mark P.

AU - Lim, Foong Yen

AU - Stamilio, David

AU - Silber, Angela

AU - Zakii, Elaine

AU - Flake, Alan W.

PY - 2006/11

Y1 - 2006/11

N2 - 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.

AB - 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.

KW - Autosomal recessive

KW - Congenital pulmonary lymphangiectasis

KW - Perinatal mortality

KW - Pleural effusion

UR - https://www.scopus.com/pages/publications/33751338706

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DO - 10.1002/pd.1555

M3 - Article

C2 - 16941717

AN - SCOPUS:33751338706

SN - 0197-3851

VL - 26

SP - 1058

EP - 1061

JO - Prenatal Diagnosis

JF - Prenatal Diagnosis

IS - 11

ER -

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

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Keywords

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