Ishihara Asahi, Sagishima Katsuyuki, Ejima Tadashi, Kuwahara Manami, Hirata Naoyuki
Department of Critical Care Medicine, Kumamoto University Hospital, Kumamoto, Japan.
Department of Anesthesiology, Kumamoto University Hospital, Kumamoto, 860-8556, Japan.
JA Clin Rep. 2025 Jun 9;11(1):31. doi: 10.1186/s40981-025-00795-8.
Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by hypotension, hypoalbuminemia, and hemoconcentration, typically caused by increased vascular permeability due to endothelial dysfunction. We report a case of SCLS complicated by bilateral lower extremity compartment syndrome.
A 29-year-old man developed fever, cough, and rhinorrhea. He was restless, hypotensive, and had generalized edema with tense extremities. Laboratory findings included a hemoglobin level of 24.9 g/dL, hematocrit of 69.3%, albumin of 1.8 g/dL, and creatinine of 3.27 mg/dL. SCLS-induced shock was diagnosed with detection of monoclonal gammopathy of the IgG-λ type. Treatment consisted of fluid resuscitation, vasopressors, high-dose corticosteroids, and intravenous immunoglobulin. Although hemodynamic status improved, he developed bilateral lower-limb compartment syndrome, necessitating fasciotomy. Although the patient exhibited sensory deficits and impaired dorsiflexion and plantarflexion in both ankles, he was able to ambulate with a cane and was discharged on hospital day 50.
This case highlights the risk of serious complications such as compartment syndrome in patients with SCLS.
系统性毛细血管渗漏综合征（SCLS）是一种罕见的疾病，其特征为低血压、低白蛋白血症和血液浓缩，通常由内皮功能障碍导致的血管通透性增加引起。我们报告一例SCLS并发双侧下肢骨筋膜室综合征的病例。
一名29岁男性出现发热、咳嗽和流涕。他烦躁不安、血压降低，全身水肿，四肢紧张。实验室检查结果包括血红蛋白水平为24.9 g/dL、血细胞比容为69.3%、白蛋白为1.8 g/dL以及肌酐为3.27 mg/dL。通过检测IgG-λ型单克隆丙种球蛋白病诊断为SCLS诱发的休克。治疗包括液体复苏、血管升压药、大剂量皮质类固醇和静脉注射免疫球蛋白。尽管血流动力学状态有所改善，但他出现了双侧下肢骨筋膜室综合征，需要进行筋膜切开术。尽管患者双侧踝关节出现感觉障碍以及背屈和跖屈功能受损，但他能够拄拐行走，并于住院第50天出院。
该病例突出了SCLS患者发生骨筋膜室综合征等严重并发症的风险。
