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      Pregnancy management for a woman with extensive vulvar and pelvic malformations caused by Klippel–Trénaunay syndrome

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          Abstract

          Klippel–Trénaunay syndrome (KTS) is a rare congenital disorder defined by a triad of capillary malformation, venous malformation, and soft tissue or bone hypertrophy most commonly affecting unilateral lower limbs. Due to the rarity of KTS, evidence‐based guidelines for the management of pregnancy in people with KTS are still lacking. A 34‐year‐old woman (gravidity 1; parity 0) presented at 25 weeks of gestation with malformations of the right side of her body. The extent of the KTS affecting the vulva, pelvis, and right leg was remarkable. As the prenatal MRI showed massive vascular malformations of the pelvis and vulva, we performed an elective cesarean section to avoid severe perinatal hemorrhage during a vaginal delivery. Intraoperatively, we observed varices on the parietal peritoneum within the vesico‐uterine pouch and the isthmocervical transition of the uterus, which were not identifiable in the preoperative MRI. Although KTS patients have been discouraged from pregnancy in the past because of a high risk for complications, successful and uncomplicated pregnancies are possible. For this purpose, we believe a multidisciplinary strategy that is crucial.

          Abstract

          To lower the risk for complications in pregnancies for people with excessive KTS, a multidisciplinary strategy including prenatal consultations with radiology, anesthesia, hemostaseology, and pediatrics is crucial.

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          Most cited references14

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          Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies.

          Vascular anomalies represent a spectrum of disorders from a simple "birthmark" to life- threatening entities. Incorrect nomenclature and misdiagnoses are commonly experienced by patients with these anomalies. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Classification schemes provide a consistent terminology and serve as a guide for pathologists, clinicians, and researchers. One of the goals of the International Society for the Study of Vascular Anomalies (ISSVA) is to achieve a uniform classification. The last classification (1997) stratified vascular lesions into vascular malformations and proliferative vascular lesions (tumors). However, additional disease entities have since been identified that are complex and less easily classified by generic headings, such as capillary malformation, venous malformation, lymphatic malformation, etc. We hereby present the updated official ISSVA classification of vascular anomalies. The general biological scheme of the classification is retained. The section on tumors has been expanded and lists the main recognized vascular tumors, classified as benign, locally aggressive or borderline, and malignant. A list of well-defined diseases is included under each generic heading in the "Simple Vascular Malformations" section. A short definition is added for eponyms. Two new sections were created: one dealing with the malformations of individually named vessels (previously referred to as "truncular" malformations); the second groups lesions of uncertain or debated nature (tumor versus malformation). The known genetic defects underlying vascular anomalies are included in an appendix. This classification is meant to be a framework, acknowledging that it will require modification as new scientific information becomes available.
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            Klippel-Trenaunay Syndrome

            Dr John (2019)
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              Vascular malformations syndromes: an update.

              To provide an update of vascular malformation syndromes by reviewing the most recent articles on the topic and following the new International Society for the Study of Vascular Anomalies (ISSVA) 2018 classification.
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                Author and article information

                Contributors
                konstantin_hofmann@yahoo.de
                Journal
                Clin Case Rep
                Clin Case Rep
                10.1002/(ISSN)2050-0904
                CCR3
                Clinical Case Reports
                John Wiley and Sons Inc. (Hoboken )
                2050-0904
                25 July 2022
                July 2022
                : 10
                : 7 ( doiID: 10.1002/ccr3.v10.7 )
                : e6130
                Affiliations
                [ 1 ] Department of Obstetrics and Gynecology University Medical Center of the Johannes Gutenberg‐University Mainz Mainz Germany
                [ 2 ] Department of Diagnostic and Interventional Radiology University Medical Center of the Johannes Gutenberg‐University Mainz Mainz Germany
                Author notes
                [*] [* ] Correspondence

                Konstantin Hofmann, Department of Obstetrics and Gynecology, University Medical Center of the Johannes Gutenberg‐University Mainz, Langenbeckstraße 1, 55131 Mainz, Germany.

                Email: konstantin_hofmann@ 123456yahoo.de

                Author information
                https://orcid.org/0000-0002-4099-1596
                Article
                CCR36130 CCR3-2022-04-0819.R1
                10.1002/ccr3.6130
                9309614
                35898753
                cea811be-ffab-40ae-a1a5-dcb4c04ac0b9
                © 2022 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 30 May 2022
                : 11 April 2022
                : 13 July 2022
                Page count
                Figures: 5, Tables: 0, Pages: 6, Words: 2614
                Categories
                Case Report
                Case Report
                Custom metadata
                2.0
                July 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.1.7 mode:remove_FC converted:25.07.2022

                c‐section,klippel–trénaunay syndrome,postpartum hemorrhage,pregnancy,vascular malformations

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