Fasciotomy for Spontaneous Acute Compartment Syndrome in a Patient with Acquired Hemophilia A-A Case Report

筋膜切開術於後天型A型血友病(Acquired hemophilia A)所誘發急性腔室症候群中的角色－病例報告

10.7096/TJTSPS.201003.0047

陳建良(Chien-Lian Chen)；謝榮賢(Jung-Hsien Hsien)；湯月碧(Yueh-Bih Tang)；陳明庭(Ming-Ting Chen)

compartment syndrome ； acquired hemophilia A ； lower extremity ； fasciotomy ； Factor VⅢ

臺灣整形外科醫學會雜誌

19卷1期（2010 / 03 / 01）

47 - 52

英文

背景： 急性腔室症候群是可能導致截肢的急症且大部份病人有外傷病史。如病人無外傷史或有出血性疾病時，常誤診或延遲診斷而導致嚴重出血或神經血管障礙等併發症。身為臨床醫師的我們應該熟稔造成腔室症候群的各式病因，給予包含手術及穩定腔室壓力的治療，以減少併發症發生。 目的及目標： 我們報告一位有後天型A型血友病病人因主動出血導致下肢腔室症候群的病例。病人腿部腔室壓力高達40毫米汞柱並緊急接受筋膜切開術，術後出現傷口大量出血及休克的併發症。經給予大量輸血及補充凝血因子來矯正他的凝血因子病變與易出血的體質後，傷口不再出血且經補皮後成功閉合。 結果： 有後天性A型血友病病人若出現下肢腔室症候群時治療困難且常被誤診。在這些病人若腿部腔室壓力在補充凝血因子或矯正凝血異常後仍不穩定或持續升高時應該仔細並考量進行筋膜切開術以免產生嚴重併發症。合併血液科醫師所建議的人工合成凝血因子與免疫抑制劑治療後,病患凝血功能逐漸改善並於一個月後出院。 結論： 後天性A型血友病患因自然的出血導致下肢腔室症候群的狀況極為少見。往往緊急執行筋膜切開術會造成持續出血並休克，而需要大量輸血並補充凝血因子。若能早期診斷並早期矯正凝血因子異常或許可以降低併發症且改善預後。

Background: The acute compartment syndrome is a limb-threatening condition and mostly associated with traumatic insults. Without an obvious history of injury nor in those with bleeding diathesis, it is often missed and delayed diagnosed, but frequently complicated by massive bleeding and neurovascular compromise. Materials and Methods: We present a rare case of acute compartment syndrome of the lower extremity due to spontaneous bleeding from acquired hemophilia A. The patient underwent fasciotomy and the postoperative course was complicated with massive bleeding from the wound bed. Blood components and factor VⅢ equivalents were administrated, and the wound was closed by a split-thickness skin graft thereafter. Results: Compartment syndrome of the legs in patients with acquired hemophilia A is hard to management and often misdiagnosed. Fasciotomy should be undertaken selectively and cautiously when the compartment pressure remains unstable or persistently high. Conclusion: Compartment syndrome from acquired hemophilia A is rarely reported. Constant bleeding from the fasciotomy site is a major complication, mandating frequent transfusions of blood and blood products. Early diagnosis and factor replacement may reduce the morbidity and result in better outcome.