Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
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 result...
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Wiley
2013-01-01
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| Series: | Case Reports in Obstetrics and Gynecology |
| Online Access: | http://dx.doi.org/10.1155/2013/186173 |
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| author | Paul Singh Matthew Connell |
| author_facet | Paul Singh Matthew Connell |
| author_sort | Paul Singh |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-86ce1c719f904b749fa8e99d560b0f49 |
| institution | Kabale University |
| issn | 2090-6684 2090-6692 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Obstetrics and Gynecology |
| spelling | doaj-art-86ce1c719f904b749fa8e99d560b0f492025-02-03T01:21:17ZengWileyCase Reports in Obstetrics and Gynecology2090-66842090-66922013-01-01201310.1155/2013/186173186173Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the LiteraturePaul Singh0Matthew Connell1Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USADepartment of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USAIntroduction. 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.http://dx.doi.org/10.1155/2013/186173 |
| spellingShingle | Paul Singh Matthew Connell Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature Case Reports in Obstetrics and Gynecology |
| title | Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature |
| title_full | Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature |
| title_fullStr | Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature |
| title_full_unstemmed | Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature |
| title_short | Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature |
| title_sort | primary congenital lymphedema complicated by hydrops fetalis a case report and review of the literature |
| url | http://dx.doi.org/10.1155/2013/186173 |
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