Illustrated operative management of spontaneous bleeding and compartment syndrome of the lower extremity in a patient with acquired hemophilia A: a case report

Abstract

IntroductionSpontaneous 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.Case presentationWe 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.ConclusionsAwareness, 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.

Atraumatic compartment syndrome of the leg caused by undiagnosed acquired hemophilia A

“…3 An incidence of only 1/500,000 are symptomatic and it is usually diagnosed by unexplained bleeding after surgical procedures. 4 A very similar presentation of atraumatic compartment syndrome of the leg was described by Jentzsch et al, 5 with prompt recognition of the undiagnosed coagulopathy. The patient underwent initial fasciotomy with continued bleeding post operatively leading to the diagnosis of hemophilia.…”

Review article: A comprehensive review of unusual causes of acute limb compartment syndrome

“…Pro‐ and anti‐coagulant clotting abnormalities are also associated with ALCS, including spontaneous haemorrhage from haemophilia, factor deficiencies and haematological malignancy. 11 , 18 , 19 , 20 , 21 , 22 Platelet deficiency or dysfunction also causes ALCS, and has been reported with human immunodeficiency virus. 23 , 24 Extreme thrombocytosis may also cause spontaneous haemorrhage leading to compartment syndrome.…”

Acute Compartment Syndrome of the Upper Extremity in Acquired Hemophilia A

“…We could not wait until bypass therapy was effective before performing a fasciotomy. We needed to reduce the compartment pressure of the upper extremity as quickly as possible because, as in previous reports [15][16][17][18] , the pressures had risen markedly by the time we measured them. In fact, there was a risk that severe bleeding because of AHA could lead to more serious ACS.…”