Jentzsch Thorsten, Brand-Staufer Brigitte, Schäfer Frank P, Wanner Guido A, Simmen Hans-Peter
Division of Trauma Surgery, Department of Surgery, University Hospital Zürich, Rämistrasse 100, 8091 Zürich, Switzerland.
J Med Case Rep. 2014 Apr 30;8:132. doi: 10.1186/1752-1947-8-132.
Spontaneous bleeding resulting in compartment syndrome at the lower adult leg due to acquired hemophilia A is rare. There are no reports on operative management of this entity.
We present a case of atraumatic compartment syndrome of the lower leg due to acquired factor VIII deficiency, in an 83-year-old Caucasian man of European descent. He was treated surgically with a long and complicated postoperative course after presenting to a community hospital with a 2-day history of increasing pain and swelling in his left lower leg without a previous history of trauma.
Awareness, prompt diagnosis and effective treatment of compartment syndrome caused by a rare bleeding disorder, which is usually acquired by the elderly, is essential and may spare a patient from surgery or even limb loss, if early administration of recombinant factor VIIa is effective. The course of disease in a patient with operative management of spontaneous bleeding, compartment syndrome and acquired hemophilia A may be prolonged. However, an interdisciplinary approach with meticulous surgical treatment and bleeding management with recombinant factor VIIa as well as inhibitor eradication by immunosuppressive treatment can be successful and expensive.
获得性血友病A导致成人小腿部自发性出血并引发骨筋膜室综合征的情况较为罕见。目前尚无关于该病症手术治疗的报道。
我们报告一例83岁具有欧洲血统的白种男性因获得性凝血因子VIII缺乏导致小腿部非创伤性骨筋膜室综合征的病例。他因左小腿疼痛和肿胀加重2天就诊于一家社区医院,此前无创伤史,随后接受了手术治疗,术后病程漫长且复杂。
对于通常在老年人中获得性的罕见出血性疾病引起的骨筋膜室综合征,提高认识、及时诊断并进行有效治疗至关重要,若早期给予重组凝血因子VIIa有效,可使患者避免手术甚至截肢。自发性出血、骨筋膜室综合征和获得性血友病A患者接受手术治疗后的病程可能会延长。然而,采用精心的手术治疗、重组凝血因子VIIa进行出血管理以及免疫抑制治疗根除抑制剂的多学科方法可能会取得成功,但费用高昂。