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      Klippel-Trenaunay Syndrome and Pregnancy

      case-report
      Author(s): , *
      Publication date (Electronic):
      Journal: Obstetrics and Gynecology International
      Publisher: Hindawi Publishing Corporation

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          Abstract

          Klippel-Trenaunay syndrome is a rare congenital vascular disorder, and only few cases have been described in pregnancy. We describe two cases, in one patient without complications, the other patient developed postpartum deep venous thrombosis.

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          Klippel-Trénaunay syndrome: spectrum and management.

          A. G. Jacob, D J Driscoll, W J Shaughnessy … (1998)
          To describe a series of 252 patients with Klippel-Trénaunay syndrome (KTS), a rare congenital malformation characterized by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity. We reviewed the clinical characteristics and findings in 136 female and 116 male patients with KTS who underwent assessment at Mayo Clinic Rochester between January 1956 and January 1995. In addition, management options are discussed. Capillary malformations (port-wine stains) were found in 246 patients (98%), varicosities or venous malformations in 182 (72%), and limb hypertrophy in 170 (67%). All three features of KTS were present in 159 patients (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, excision of vascular malformations, amputations, and debulking procedures. Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2.0 cm at skeletal maturity, which can be treated with epiphysiodesis in the growing child. If a functioning deep vein system is present, removal of symptomatic varicosities or localized superficial venous malformations in selected patients can yield good results.
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            Genetic aspects of the Klippel-Trenaunay syndrome.

            P Jorens, G E Aelvoet, Dave L Roelen (1992)
            An extensive search for a genetic pattern in Klippel-Trenaunay syndrome (KTS) revealed two other cases of KTS in the families of two of the 86 patients with this vascular syndrome who were questioned. Patients with KTS also had family members with other malformations: e.g. hemihypertrophy in one family, and a prevalence of 7/400 of naevi flammei in first-degree relatives of KTS patients was observed. We suggest that KTS can be inherited in a multifactorial way and a range of vascular malformations can be observed in the family members of patients with this syndrome.
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              Increased parental age and number of pregnancies in Klippel-Trenaunay-Weber syndrome.

              I. Lorda-Sanchez, L. Prieto, E Rodríguez-Pinilla … (1998)
              The Klippel-Trenaunay-Weber syndrome (KTWS) is generally thought to occur sporadically, following a somatic mutation model. However, in some cases, clinical manifestations of the syndrome have been found in family members, suggesting an autosomal dominant inheritance. Here we present an epidemiological analysis of a consecutive series of cases with KTWS identified in the Spanish Collaborative Study of Congenital Malformations (ECEMC). We found an increase in parental age and in the number of pregnancies, as well as familial occurrence of haemangiomas. These observations suggest a genetic contribution to the occurrence of KTWS.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Obstet Gynecol Int
                Journal ID (publisher-id): OGI
                Title: Obstetrics and Gynecology International
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1687-9589
                ISSN (Electronic): 1687-9597
                Publication date (Print): 2010
                Publication date (Electronic): 19 December 2010
                Volume: 2010
                Electronic Location Identifier: 706850
                Affiliations
                Department of Obstetrics and Gynaecology, Antwerp University Hospital UZA, Wilrijkstraat 10, 2650 Edegem, Belgium
                Author notes

                Academic Editor: John M. G. van Vugt

                Article
                DOI: 10.1155/2010/706850
                PMC ID: 3010666
                PubMed ID: 21209709
                SO-VID: 0e27ebd5-40e8-49c2-829a-cb99275f397b
                Copyright © Copyright © 2010 T. Güngor Gündoğan and Y. Jacquemyn.
                License:

                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.

                History
                Date received : 10 August 2010
                Date revision received : 30 October 2010
                Date accepted : 9 November 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Obstetrics & Gynecology
                Data availability:
                ScienceOpen disciplines: Obstetrics & Gynecology

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