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Case Reports in Obstetrics and Gynecology
Volume 2013, Article ID 186173, 4 pages
http://dx.doi.org/10.1155/2013/186173
Case Report

Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature

1Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA
2Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA

Received 6 January 2013; Accepted 7 February 2013

Academic Editors: E. Cosmi, C.-C. Liang, and O. Picone

Copyright © 2013 Paul Singh and Matthew Connell. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction. Primary congenital lymphedema is a rare disorder associated with insufficient development of lymphatic vessels. Usually most patients present with lower extremity edema seen sonographically. Rarely primary congenital lymphedema may be associated with severe lymphatic dysfunction resulting in hydrops fetalis. Case. A 27-year-old primigravida with a family history of leg swelling throughout multiple generations was diagnosed early in the third trimester with hydrops fetalis. Delivery was undertaken at 32 weeks for nonreassuring fetal status and the infant expired at approximately 45 minutes of life. Primary congenital lymphedema was confirmed via molecular testing of the vascular endothelial growth factor receptor-3 gene. Discussion. The diagnosis of PCL is suspected prenatally when ultrasound findings coincide with a positive family history of chronic lower limb lymphedema. Isolated PCL is rarely associated with significant complications. Rarely, however, widespread lymphatic dysplasia may occur, possibly resulting in nonimmune hydrops fetalis.