Noguchi Miho, Akita Sadanori, Nagai Fumio, Nomura Tadashi, Morishita Tsuyoshi, Yuzuriha Shunsuke
Department of Plastic and Reconstructive Surgery, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
Department of Bioregulation and Pharmacological Medicine, Fukushima Medical University, Fukushima 960-1247, Japan.
J Clin Med. 2025 Jul 7;14(13):4808. doi: 10.3390/jcm14134808.
Klippel-Trenaunay syndrome (KTS) is a congenital vascular malformation syndrome characterized by low-flow vascular anomalies, including venous malformation (VM) and lymphatic involvement. These anomalies may lead to limb asymmetry due to soft tissue and/or bone overgrowth. Compression therapy using elastic garments is considered a conservative and minimally invasive first-line treatment option for KTS. However, the benefits of compression therapy for low-flow vascular malformations, particularly limb VMs, have not been sufficiently evaluated. This prospective, multi-center study assessed the efficacy and safety of compression therapy for KTS with VM. After measuring the affected limb, a custom-made elastic garment providing 30 mmHg of compression was manufactured (THUASNE, France). A total of 20 patients (7 male, 13 female; mean age: 10.9 years) underwent compression therapy for 26 weeks at four nationwide institutions in Japan. The primary outcome was the change in lower limb circumference. Secondary outcomes included pain, modified Rankin Scale (mRS) score, body water content, vital signs, changes in garment elasticity, and adverse events. All 20 patients completed the study. At the study endpoint, the circumference ratio of the affected to unaffected limbs was significantly reduced at the superior end of the tibial tuberosity ( = 0.02) and the thinnest part of the ankle ( < 0.001). The elastic force of the garment declined by approximately 50% over 26 weeks. No serious adverse events related to the intervention were reported. Compression therapy using a custom-made elastic garment appears to be a safe and effective approach for managing limb overgrowth in patients with KTS and VM. To maintain the therapeutic effect, garment replacement is recommended at least every six months.
克-特综合征（KTS）是一种先天性血管畸形综合征，其特征为低流量血管异常，包括静脉畸形（VM）和淋巴管受累。这些异常可能由于软组织和/或骨骼过度生长而导致肢体不对称。使用弹力衣进行压迫治疗被认为是KTS保守且微创的一线治疗选择。然而，压迫治疗对低流量血管畸形，尤其是肢体VM的益处尚未得到充分评估。这项前瞻性、多中心研究评估了压迫治疗对伴有VM的KTS的疗效和安全性。在测量患侧肢体后，制作了一款提供30 mmHg压力的定制弹力衣（法国图阿斯内公司）。在日本全国的四家机构中，共有20例患者（7例男性，13例女性；平均年龄：10.9岁）接受了为期26周的压迫治疗。主要结局是下肢周长的变化。次要结局包括疼痛、改良Rankin量表（mRS）评分、身体含水量、生命体征、弹力衣弹性变化以及不良事件。所有20例患者均完成了研究。在研究终点，胫骨结节上端（P = 0.02）和踝关节最细处（P < 0.001）患侧与未患侧肢体的周长比显著降低。弹力衣的弹力在26周内下降了约50%。未报告与干预相关的严重不良事件。使用定制弹力衣进行压迫治疗似乎是管理伴有VM的KTS患者肢体过度生长的一种安全有效的方法。为维持治疗效果，建议至少每六个月更换一次弹力衣。
