| Recurrent and Extensive Vascular Malformations in a Patient With Bannayan-Riley-Ruvalcaba Syndrome. | |
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MedLine Citation:
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PMID: 21856113 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare, usually autosomal dominant, disease associated with the PTEN hamartoma tumor syndrome, and it is clinically diagnosed in the presence of the triad of macrocephaly, genital lentiginosis, and intestinal polyposis. Vascular malformations, including arteriolovenous shunts, arteriovenous anomalies, and arteriovenous fistulae, can also be seen in a subset of BRRS patients, varying from local overgrowth of small vessels to large aneurysms. The range of these vascular manifestations and the pathophysiology behind them are not completely understood. In this case study, we report on a patient with BRRS and recurrent vascular malformations of the left lower extremity. We also review the current literature, with a specific focus on the breadth of vascular malformations and reported care of these BRRS patients. Based on the recurrent and sometimes extensive nature of the vascular pathology, it is recommended that physicians monitor BRRS patients closely for early detection and treatment. |
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Authors:
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Maria Litzendorf; Kimberly Hoang; Patrick Vaccaro |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-8-17 |
Journal Detail:
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Title: Annals of vascular surgery Volume: - ISSN: 1615-5947 ISO Abbreviation: - Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-8-22 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8703941 Medline TA: Ann Vasc Surg Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Annals of Vascular Surgery Inc. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Division of Vascular Diseases and Surgery, The Ohio State University Medical Center, Columbus, OH. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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