Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
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Auteurs : Paul Singh [États-Unis] ; Matthew Connell [États-Unis]Source :
- Case Reports in Obstetrics and Gynecology [ 2090-6684 ] ; 2013.
Abstract
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DOI: 10.1155/2013/186173
PubMed: 23533860
PubMed Central: 3600248
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature</title><author><name sortKey="Singh, Paul" sort="Singh, Paul" uniqKey="Singh P" first="Paul" last="Singh">Paul Singh</name><affiliation wicri:level="1"><nlm:aff id="I1">Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064</wicri:regionArea><wicri:noRegion>MO 64064</wicri:noRegion></affiliation></author><author><name sortKey="Connell, Matthew" sort="Connell, Matthew" uniqKey="Connell M" first="Matthew" last="Connell">Matthew Connell</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064</wicri:regionArea><wicri:noRegion>MO 64064</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">23533860</idno><idno type="pmc">3600248</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600248</idno><idno type="RBID">PMC:3600248</idno><idno type="doi">10.1155/2013/186173</idno><date when="2013">2013</date><idno type="wicri:Area/Pmc/Corpus">003C89</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">003C89</idno><idno type="wicri:Area/Pmc/Curation">003C88</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">003C88</idno><idno type="wicri:Area/Pmc/Checkpoint">002051</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">002051</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature</title><author><name sortKey="Singh, Paul" sort="Singh, Paul" uniqKey="Singh P" first="Paul" last="Singh">Paul Singh</name><affiliation wicri:level="1"><nlm:aff id="I1">Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064</wicri:regionArea><wicri:noRegion>MO 64064</wicri:noRegion></affiliation></author><author><name sortKey="Connell, Matthew" sort="Connell, Matthew" uniqKey="Connell M" first="Matthew" last="Connell">Matthew Connell</name><affiliation wicri:level="1"><nlm:aff id="I2">Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</nlm:aff><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064</wicri:regionArea><wicri:noRegion>MO 64064</wicri:noRegion></affiliation></author></analytic><series><title level="j">Case Reports in Obstetrics and Gynecology</title><idno type="ISSN">2090-6684</idno><idno type="eISSN">2090-6692</idno><imprint><date when="2013">2013</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p><italic>Introduction</italic>. 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. <italic>Case</italic>. 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. <italic>Discussion</italic>. 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.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Milroy, W" uniqKey="Milroy W">W Milroy</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Nonne, M" uniqKey="Nonne M">M Nonne</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Greenlee, R" uniqKey="Greenlee R">R Greenlee</name></author><author><name sortKey="Hoyme, H" uniqKey="Hoyme H">H Hoyme</name></author><author><name sortKey="Witte, M" uniqKey="Witte M">M Witte</name></author><author><name sortKey="Crowe, P" uniqKey="Crowe P">P Crowe</name></author><author><name sortKey="Witte, C" uniqKey="Witte C">C Witte</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hurwitz, Pa" uniqKey="Hurwitz P">PA Hurwitz</name></author><author><name sortKey="Pinals, Dj" uniqKey="Pinals D">DJ 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Case Rep Obstet Gynecol</journal-id><journal-id journal-id-type="iso-abbrev">Case Rep Obstet Gynecol</journal-id><journal-id journal-id-type="publisher-id">CRIM.OBGYN</journal-id><journal-title-group><journal-title>Case Reports in Obstetrics and Gynecology</journal-title></journal-title-group><issn pub-type="ppub">2090-6684</issn><issn pub-type="epub">2090-6692</issn><publisher><publisher-name>Hindawi Publishing Corporation</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">23533860</article-id><article-id pub-id-type="pmc">3600248</article-id><article-id pub-id-type="doi">10.1155/2013/186173</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject></subj-group></article-categories><title-group><article-title>Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature</article-title></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid" authenticated="false">0000-0003-3895-7804</contrib-id><name><surname>Singh</surname><given-names>Paul</given-names></name><xref ref-type="aff" rid="I1"><sup>1</sup></xref><xref ref-type="corresp" rid="cor1">*</xref></contrib><contrib contrib-type="author"><name><surname>Connell</surname><given-names>Matthew</given-names></name><xref ref-type="aff" rid="I2"><sup>2</sup></xref></contrib></contrib-group><aff id="I1"><sup>1</sup>Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</aff><aff id="I2"><sup>2</sup>Department of Obstetrics and Gynecology, University of Missouri School of Medicine, 2301 Holmes Street, Kansas City, MO 64064, USA</aff><author-notes><corresp id="cor1">*Paul Singh: <email>paulsingh49@yahoo.com</email></corresp><fn fn-type="other"><p>Academic Editors: E. Cosmi, C.-C. Liang, and O. Picone</p></fn></author-notes><pub-date pub-type="ppub"><year>2013</year></pub-date><pub-date pub-type="epub"><day>28</day><month>2</month><year>2013</year></pub-date><volume>2013</volume><elocation-id>186173</elocation-id><history><date date-type="received"><day>6</day><month>1</month><year>2013</year></date><date date-type="accepted"><day>7</day><month>2</month><year>2013</year></date></history><permissions><copyright-statement>Copyright © 2013 P. Singh and M. Connell.</copyright-statement><copyright-year>2013</copyright-year><license xlink:href="https://creativecommons.org/licenses/by/3.0/"><license-p>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.</license-p></license></permissions><abstract><p><italic>Introduction</italic>. 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. <italic>Case</italic>. 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. <italic>Discussion</italic>. 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.</p></abstract></article-meta></front><floats-group><fig id="fig1" orientation="portrait" position="float"><label>Figure 1</label><caption><p>Note the swelling present along the dorsal aspect the left foot and throughout the distal left lower extremity.</p></caption><graphic xlink:href="CRIM.OBGYN2013-186173.001"></graphic></fig><fig id="fig2" orientation="portrait" position="float"><label>Figure 2</label><caption><p>Note the swelling along the distal right lower extremity.</p></caption><graphic xlink:href="CRIM.OBGYN2013-186173.002"></graphic></fig><fig id="fig3" orientation="portrait" position="float"><label>Figure 3</label><caption><p>Cross sectional view of the abdomen showing abdominal ascites.</p></caption><graphic xlink:href="CRIM.OBGYN2013-186173.003"></graphic></fig><fig id="fig4" orientation="portrait" position="float"><label>Figure 4</label><caption><p>Cross sectional view of the fetal head showing skin edema.</p></caption><graphic xlink:href="CRIM.OBGYN2013-186173.004"></graphic></fig></floats-group></pmc><affiliations><list><country><li>États-Unis</li></country></list><tree><country name="États-Unis"><noRegion><name sortKey="Singh, Paul" sort="Singh, Paul" uniqKey="Singh P" first="Paul" last="Singh">Paul Singh</name></noRegion><name sortKey="Connell, Matthew" sort="Connell, Matthew" uniqKey="Connell M" first="Matthew" last="Connell">Matthew Connell</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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