Karmacharya Robin Man, Vaidya Satish, Bhatt Swechha, Tamang Ashish, Shrestha Rohit Bhasink, Bhandari Niroj, Paudel Bijaya, Shah Manish, Nepal Gaurav
Unit Chief, Cardio Thoracic and Vascular Surgery Unit, Department of Surgery, Kathmandu University School of Medical Sciences, Dhulikhel, 45210, Nepal.
Cardio Thoracic and Vascular Surgery Unit, Department of Surgery, Kathmandu University School of Medical Sciences, Dhulikhel, 45210, Nepal.
Ann Med Surg (Lond). 2022 May 7;78:103732. doi: 10.1016/j.amsu.2022.103732. eCollection 2022 Jun.
Klippel Trenaunay Syndrome (KTS) is a rare congenital malformation with capillary and venous malformations and soft tissue/bony overgrowth with or without lymphatic malformation. Cutaneous vascular stain, varicosities and tissue hypertrophy represent its main clinical features. Besides, the patient can develop thromboembolic pathologies, recurrent bouts of infection, stasis eczema, limb length discrepancy and intolerable pain typical of intraosseous involvement.
Here, we report a case series of seven patients aged 10-45 who presented to our centre with clinical features suggestive of KTS. Out of them, six patients had involvement of unilateral lower limb, while only one had involvement of bilateral lower limb. They all had typical cutaneous vascular stains and underlying venous malformation, while one patient had developed complications with multiple ulcer formation.
An interdisciplinary team of vascular surgeons, dermatologists, interventional radiologists, orthopaedics, and physiotherapists managed the cases. We performed an individualized treatment as per the patient's presentation, which included a combination of supportive, medical, interventional radiologic, and surgical interventions. The follow-up outcomes of all the patients revealed significant resolution of symptoms.
Patients with KTS can have diverse presentations. Therefore, clinicians should ensure an individualized treatment with the involvement of a multidisciplinary team for proper management and prevention of complications.
克-特综合征（KTS）是一种罕见的先天性畸形，伴有毛细血管和静脉畸形以及软组织/骨骼过度生长，可有或无淋巴管畸形。皮肤血管痣、静脉曲张和组织肥大是其主要临床特征。此外，患者可能会出现血栓栓塞性病变、反复感染、淤积性皮炎、肢体长度差异以及典型的骨内受累引起的难以忍受的疼痛。
在此，我们报告了一组7例年龄在10至45岁之间的病例系列，这些患者因临床特征提示KTS前来我们中心就诊。其中，6例患者单侧下肢受累，只有1例双侧下肢受累。他们均有典型的皮肤血管痣和潜在的静脉畸形，其中1例患者出现了多处溃疡形成的并发症。
由血管外科医生、皮肤科医生、介入放射科医生、骨科医生和物理治疗师组成的多学科团队对这些病例进行了管理。我们根据患者的表现进行了个体化治疗，包括支持性、药物、介入放射学和手术干预的联合应用。所有患者的随访结果显示症状有显著缓解。
KTS患者的表现可能多种多样。因此，临床医生应确保在多学科团队的参与下进行个体化治疗，以妥善管理和预防并发症。
