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老年获得性甲型血友病患者腿部骨筋膜室综合征的治疗:一例报告。

Treatment of a compartment syndrome in the leg in an elderly patient with acquired hemophilia type A: A case report.

作者信息

Fonseca Andres Felipe, Herrera Gilberto Antonio, Gallego Alejandro, Llinas Paulo J

机构信息

Department of Orthopedics and Traumatology, Hospital Universitario Fundación Valle del Lili, Cali, Colombia.

Centro de investigaciones clínicas, Hospital Universitario Fundación Valle del Lili, Cali, Colombia.

出版信息

Int J Surg Case Rep. 2023 Oct 28;112:108999. doi: 10.1016/j.ijscr.2023.108999.

Abstract

INTRODUCTION

Acquired hemophilia type A is a rare autoimmune disease characterized by bleeding episodes ranging from mild to severe, leading to significant morbidity and mortality. One of the various presentations is compartment syndrome secondary to a spontaneous hematoma.

CASE PRESENTATION

A 95-year-old woman arrived at the emergency department with a one-week history of progressive pain in the right leg, without a previous history of trauma. Upon physical examination, she presented tenderness, edema, and bruising on the posterior and lateral aspects of the right leg, without neurovascular deficits. Ultrasound revealed a collection in the posterior compartment of the leg, consistent with a hematoma. Due to worsening clinical condition, urgent drainage and fasciotomy of the posterior and lateral compartments of the leg was performed. After 2 years of follow-up, the patient has shown favorable progression.

DISCUSSION

Hematoma drainage and fasciotomy are the primary management approaches for compartment syndrome secondary to hematoma presence. This etiology should be suspected in patients diagnosed with hemophilia who present to the emergency department with acute limb pain of unclear origin. Treatment should focus on relieving compartment pressure, supplementing the deficient factor, and eradicating inhibitors. None of the globally reported cases present a scenario with such an elderly patient exhibiting positive outcomes.

CONCLUSION

Compartment syndrome secondary to a spontaneous hematoma in a patient with acquired hemophilia is a diagnosis associated with significant morbidity and mortality, necessitating urgent management to ensure appropriate progression. This requires a multidisciplinary approach to drain the hematoma and rectify the hematologic condition.

摘要

引言

获得性甲型血友病是一种罕见的自身免疫性疾病,其特征为出血发作程度从轻度到重度不等,可导致严重的发病率和死亡率。各种表现形式之一是自发性血肿继发的骨筋膜室综合征。

病例报告

一名95岁女性因右腿进行性疼痛一周就诊于急诊科,既往无外伤史。体格检查时,她右腿后侧和外侧有压痛、水肿及瘀斑,无神经血管功能缺损。超声显示小腿后骨筋膜室内有一液性暗区,符合血肿表现。由于临床状况恶化,遂对小腿后外侧骨筋膜室进行紧急引流和筋膜切开术。经过2年随访,患者病情进展良好。

讨论

血肿引流和筋膜切开术是血肿继发骨筋膜室综合征的主要治疗方法。对于诊断为血友病且因不明原因急性肢体疼痛就诊于急诊科的患者,应怀疑这种病因。治疗应侧重于减轻骨筋膜室压力、补充缺乏的凝血因子及消除抑制物。全球报道的病例中均未出现过老年患者有如此良好预后的情况。

结论

获得性血友病患者自发性血肿继发的骨筋膜室综合征是一种与显著发病率和死亡率相关的诊断,需要紧急处理以确保病情适当进展。这需要多学科方法来引流血肿并纠正血液学状况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2cd4/10667864/1a518d2b2328/gr1.jpg

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