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克-特综合征孕妇的产科管理:英国一例病例报告及文献综述

Obstetric management for pregnant women with Klippel-Trenaunay syndrome: A UK case report and review of the literature.

作者信息

Silva Correia Inês F, Hussain Munawar, Johnson Jo-Anne

机构信息

School of Medicine, Faculty of HEMS, Anglia Ruskin University, Chelmsford, UK.

Department of Obstetrics and Gynaecology, Southend University Hospital, Southend-on-Sea, UK.

出版信息

Int J Gynaecol Obstet. 2025 Feb;168(2):484-486. doi: 10.1002/ijgo.15889. Epub 2024 Sep 3.

Abstract

Klippel-Trenaunay Syndrome (KTS) is a rare congenital vascular disorder characterized by extensive capillary and venous malformations that pose unique challenges during pregnancy. This case report discusses the successful management of a 34-year-old pregnant woman with KTS who had two caesarean sections, resulting in the birth of two healthy babies. Despite the lack of evidence-based guidelines for obstetrical management in KTS, a multidisciplinary team collaborated to devise a high-risk thrombosis management plan, involving the use of compression stocking and low molecular weight heparin prophylaxis. The patient's elevated risk of thrombosis, exacerbated during pregnancy, informed the decision of caesarean sections, aligning with finding that in most KTS pregnancies, this method of delivery based on obstetric indications and arteriovenous malformations is chosen. This case highlights the importance of systematic and patient-centered care, advocating for comprehensive obstetric management guidelines to address the unique challenges posed by KTS during pregnancy. Further research is warranted to enhance our understanding and refine guidelines for individuals with vascular abnormalities linked to KTS.

摘要

克-特综合征(KTS)是一种罕见的先天性血管疾病,其特征是广泛的毛细血管和静脉畸形,这在孕期带来了独特的挑战。本病例报告讨论了一名34岁患有KTS的孕妇的成功管理,她进行了两次剖宫产,产下了两个健康的婴儿。尽管缺乏关于KTS产科管理的循证指南,但一个多学科团队合作制定了一项高风险血栓管理计划,包括使用弹力袜和低分子量肝素预防。患者血栓形成风险升高,在孕期加剧,这为剖宫产的决定提供了依据,这与大多数KTS妊娠中基于产科指征和动静脉畸形选择这种分娩方式的发现一致。本病例强调了系统和以患者为中心的护理的重要性,倡导制定全面的产科管理指南,以应对KTS在孕期带来的独特挑战。有必要进行进一步研究,以加深我们的理解并完善针对与KTS相关的血管异常个体的指南。

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本文引用的文献

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Eur J Obstet Gynecol Reprod Biol. 2023 Dec;291:96-98. doi: 10.1016/j.ejogrb.2023.10.009. Epub 2023 Oct 12.
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