Please use this identifier to cite or link to this item:
http://doi.org/10.25358/openscience-8798
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 |
ROR: | https://ror.org/023b0x485 |
DOI: | http://doi.org/10.25358/openscience-8798 |
Version: | Published version |
Publication type: | Zeitschriftenaufsatz |
License: | CC BY-NC-ND |
Information on rights of use: | https://creativecommons.org/licenses/by-nc-nd/4.0/ |
Journal: | Clinical case reports 10 7 |
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 |
Files in This Item:
File | Description | Size | Format | ||
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![]() | pregnancy_management_for_a_wo-20230210105331931.pdf | 2.58 MB | Adobe PDF | View/Open |