| Kasabach-Merritt coagulopathy complicating Klippel-Trenaunay-Weber syndrome in pregnancy. | |
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MedLine Citation:
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PMID: 7724128 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Klippel-Trenaunay-Weber syndrome is a sporadic genetic syndrome characterized by localized hemangiomas, venous varicosities, and asymmetric osseous hypertrophy of the ipsilateral extremities. Most commonly seen in association with hemangiomas, Kasabach-Merritt syndrome is defined by the presence of thrombocytopenia and a consumptive coagulopathy. CASE: A 22-year-old primigravida with a prior diagnosis of Klippel-Trenaunay-Weber syndrome presented for genetic counseling and delivery management at 37 weeks' gestation. Large varicosities of the vulva required cesarean delivery. Multiple hemangiomas in the right lower quadrant of the abdomen necessitated the use of a left paramedian cutaneous incision. The patient subsequently developed Kasabach-Merritt syndrome and required the transfusion of blood products as well as heparin and aminocaproic acid therapy for her postoperative management. CONCLUSION: Klippel-Trenaunay-Weber syndrome in pregnancy is rare. The potential for a refractory coagulopathy presenting as Kasabach-Merritt syndrome should be considered in any patient who presents with extensive hemangiomas. |
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Authors:
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A G Neubert; M A Golden; N C Rose |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Obstetrics and gynecology Volume: 85 ISSN: 0029-7844 ISO Abbreviation: Obstet Gynecol Publication Date: 1995 May |
Date Detail:
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Created Date: 1995-05-22 Completed Date: 1995-05-22 Revised Date: 2009-10-26 |
Medline Journal Info:
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Nlm Unique ID: 0401101 Medline TA: Obstet Gynecol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 831-3 Citation Subset: AIM; IM |
Affiliation:
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Department of Obstetrics and Gynecology, University of Pennsylvania Medical Center, Philadelphia, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Apgar Score Cesarean Section Disseminated Intravascular Coagulation / etiology* Female Hemangioma, Cavernous* / complications, diagnosis Humans Infant, Newborn Klippel-Trenaunay-Weber Syndrome* / complications, diagnosis Postoperative Complications Pregnancy Pregnancy Complications, Cardiovascular* / diagnosis Syndrome Thrombocytopenia* / complications, diagnosis |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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