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系统性毛细血管渗漏综合征并发下肢骨筋膜室综合征:一例报告

Systemic capillary leak syndrome complicated by lower extremity compartment syndrome: a case report.

作者信息

Ishihara Asahi, Sagishima Katsuyuki, Ejima Tadashi, Kuwahara Manami, Hirata Naoyuki

机构信息

Department of Critical Care Medicine, Kumamoto University Hospital, Kumamoto, Japan.

Department of Anesthesiology, Kumamoto University Hospital, Kumamoto, 860-8556, Japan.

出版信息

JA Clin Rep. 2025 Jun 9;11(1):31. doi: 10.1186/s40981-025-00795-8.

Abstract

BACKGROUND

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.

CASE PRESENTATION

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.

CONCLUSION

This case highlights the risk of serious complications such as compartment syndrome in patients with SCLS.

摘要

背景

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。

病例介绍

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。

结论

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d68d/12149068/c848bcf129a5/40981_2025_795_Fig1_HTML.jpg

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