Christanto Abraham Gita Ramanda, Shodiq M Ali, Fatah Sahal, Wiryawan Wahyu
Department of Surgery, Faculty of Medicine Universitas Diponegoro, Dr. Kariadi General Hospital, Semarang, Indonesia.
Division of Thoracic, Cardiac and Vascular Surgery, Department of Surgery, Faculty of Medicine Universitas Diponegoro, Dr. Kariadi General Hospital, Semarang, Indonesia.
Int J Surg Case Rep. 2023 Jul;108:108491. doi: 10.1016/j.ijscr.2023.108491. Epub 2023 Jul 8.
Servelle-Martorell syndrome (SMS) is a rare congenital anomaly that is frequently mistaken for Klippel-Trenaunay syndrome (KTS) or Parkes-Weber syndrome (PWS). SMS usually involves venous dilatations, soft tissue hypertrophy, and bone hypotrophy, while KTS and PWS usually have bone hypertrophy. The management of SMS is primarily conservative, and surgery should be done selectively. This study aimed to report a case of SMS and its management to relieve a painful aneurysm on the right knee by excision.
A 16-year-old male patient presented with a slightly shorter right lower limb and multiple bluish swelling on his right lower limb. Supporting venography and angiography showed venous malformations, soft tissue hypertrophy, and bone hypotrophy on the right lower limb. The physical and supporting examinations led to the diagnosis of SMS. The patient was admitted because of severe pain in the right knee. Surgical excision of the venous malformation in the knee region was done to relieve the pain. The patient felt significantly reduced pain on one-month follow-up.
SMS has similar features to KTS and PWS. The excision surgery was indicated due to severe pain in the right knee.
SMS is a rare disease and important to be recognized as it is frequently mistaken as KTS or PWS. The management is primarily conservative and surgical management should only be done in severe aneurysmal complications and shunting. As venous malformations and pain can reoccur after surgical excision, regular follow-ups should be maintained.
塞尔韦勒 - 马托雷尔综合征（SMS）是一种罕见的先天性异常，常被误诊为克-特综合征（KTS）或帕克斯 - 韦伯综合征（PWS）。SMS通常涉及静脉扩张、软组织肥大和骨质萎缩，而KTS和PWS通常有骨质肥大。SMS的治疗主要是保守治疗，手术应选择性进行。本研究旨在报告一例SMS病例及其通过切除缓解右膝疼痛性动脉瘤的治疗情况。
一名16岁男性患者，右下肢稍短，右下肢有多处蓝色肿胀。辅助静脉造影和血管造影显示右下肢静脉畸形、软组织肥大和骨质萎缩。体格检查和辅助检查确诊为SMS。患者因右膝剧痛入院。对膝部区域的静脉畸形进行手术切除以缓解疼痛。术后1个月随访时患者疼痛明显减轻。
SMS与KTS和PWS有相似特征。因右膝剧痛而行切除手术。
SMS是一种罕见疾病，重要的是要认识到它常被误诊为KTS或PWS。治疗主要是保守治疗，手术治疗仅适用于严重的动脉瘤并发症和分流情况。由于手术切除后静脉畸形和疼痛可能复发，应定期进行随访。
