Klippel-Trenaunay综合征患者股骨颈骨折1例报告

Fabio Seiji Mazzi Yamaguchi, Leandro Ejnisman, Julio Parente, D. Bussius, B. A. Rudelli, Helder de Souza Miyhara, Henrique Melo de Campos Gurgel
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摘要

klipppel - trenaunay综合征(KTS)是一种罕见的先天性疾病,其特征是具有葡萄酒色斑的毛细血管畸形,下肢静脉曲张或静脉畸形,以及骨和软组织肥大。在这个病例报告中,我们提出了一名47岁的女性,她在22岁时被诊断为左下肢KTS。她因KTS引起的左髋关节骨关节炎,住进了巴西圣保罗医院髋关节组门诊。患者从站立位置坠落后发生左股骨颈Garden 4骨折。基于影像学、体格检查和血管评估,我们最初的手术计划是全髋关节置换术(THA),通过后路骨移植物和髋臼笼。然而,由于大量失血,血流动力学不稳定,以及髋臼附近存在大血管,我们决定放弃最初的THA计划,转而选择切除关节成形术。对于骨科医生来说,KTS是一个挑战,需要多学科的评估。接受THA的KTS患者需要对畸形进行放射学研究,以选择合适的手术方法,尽管出血的风险很高。
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Femur Neck Fracture in a Patient with Klippel-Trenaunay Syndrome- Case Report
Klippel-Trenaunay Syndrome (KTS) is a rare congenital disorder characterized by a triad of capillary malformation with port-wine stain, varicose veins, or venous malformation of the lower limbs, and bony and soft tissue hypertrophy. In this case report, we present the case of a 47-year-old woman who was diagnosed with KTS in her left lower limb at the age of 22. She was admitted to the Hip Group Ambulatory at the Hospital das Clinicas of Sao Paulo, Brazil, due to osteoarthritis in her left hip joint caused by KTS. The patient suffered a Garden 4 left femoral neck fracture after falling from a standing position. Based on imaging, physical examination, and vascular evaluation, our initial surgical plan was Total Hip Arthroplasty (THA) with bone graft and acetabular cage via the posterior approach. However, due to significant blood loss, hemodynamic instability, and the presence of large vessels close to the acetabulum, we decided to abort the initial plan of THA and instead opted for Resection Arthroplasty of Girdlestone. KTS is a challenge for orthopedic surgeons and requires a multidisciplinary evaluation. Patients with KTS who undergo THA require a radiologic study of malformations to choose an appropriate surgical approach, despite the high risk of bleeding.
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