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REVIEW article

Front. Surg.

Sec. Genitourinary Surgery and Interventions

Volume 12 - 2025 | doi: 10.3389/fsurg.2025.1532509

This article is part of the Research TopicInnovations and Challenges in Surgical EducationView all 12 articles

Genitourinary Involvement in Klippel-Trénaunay Syndrome: Pathophysiology, Evaluation, and Management

Provisionally accepted
Weilong  LinWeilong LinChong  XieChong XieHuaijie  WangHuaijie Wang*Weijia  YangWeijia YangPeihua  WangPeihua WangZhengtuan  GuoZhengtuan Guo
  • Xi'an International Medical Center Hospital, Xi’an, China

The final, formatted version of the article will be published soon.

Klippel-Trénaunay syndrome (KTS) typically involves a combination of capillary, lymphatic, and venous malformations of the lower extremities. Genitourinary involvement is frequently observed in patients with KTS. Bleeding and pelvic pain are the most common complications. This condition has been increasingly reported in recent years. However, most authors have only depicted extreme presentations or various combinations of clinical findings. The underlying pathophysiology of genitourinary involvement in KTS remains unclear. Genitourinary involvement in female patients with KTS has a more complicated pathophysiology than that in male patients with KTS. After understanding its pathophysiology, some patients can be successfully managed by using a staged plan. Therefore, recognizing the pathophysiology of genitourinary involvement is necessary for practitioners to evaluate and determine adequate management. Owing to the complexity and rarity of this condition, a prospective controlled study involving a large cohort of patients is impossible. Based on a literature review and our practice, we discuss the pathophysiology, evaluation, and treatment strategies for genitourinary involvement in KTS.

Keywords: Klippel-Trénaunay syndrome, Uterus, Bladder, Dysmenorrhea, Hematuria, Menorrhagia

Received: 22 Nov 2024; Accepted: 04 Aug 2025.

Copyright: © 2025 Lin, Xie, Wang, Yang, Wang and Guo. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Huaijie Wang, Xi'an International Medical Center Hospital, Xi’an, China

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