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Authors: Hofmann, Konstantin
Macchiella, Doris
Kloeckner, Roman
Hasenburg, Annette
Title: Pregnancy management for a woman with extensive vulvar and pelvic malformations caused by Klippel–Trénaunay syndrome
Online publication date: 10-Feb-2023
Year of first publication: 2022
Language: english
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.
DDC: 610 Medizin
610 Medical sciences
Institution: Johannes Gutenberg-Universität Mainz
Department: FB 04 Medizin
Place: Mainz
Version: Published version
Publication type: Zeitschriftenaufsatz
License: CC BY-NC-ND
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Journal: Clinical case reports
Pages or article number: e6130
Publisher: Wiley
Publisher place: Chichester
Issue date: 2022
ISSN: 2050-0904
Publisher DOI: 10.1002/ccr3.6130
Appears in collections:DFG-491381577-G

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