title: Illustrated operative management of spontaneous bleeding and compartment syndrome of the lower extremity in a patient with acquired hemophilia A: a case report.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Trauma Surgery, Department of Surgery, University Hospital Zürich, Rämistrasse 100, 8091 Zürich, Switzerland. thorsten.jentzsch@usz.ch.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Jentzsch
authors.forename: Brigitte
authors.initials: B
authors.lastname: Brand-Staufer
authors.forename: Frank P
authors.initials: FP
authors.lastname: Schäfer
authors.forename: Guido A
authors.initials: GA
authors.lastname: Wanner
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Simmen
mesh_terms: D000369:Aged, 80 and over; D003161:Compartment Syndromes / Q000209:etiology / Q000601:surgery*; D015942:Factor VIIa / Q000627:therapeutic use*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D006470:Hemorrhage / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D035002:Lower Extremity; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa