Acquired Factor VIII Deficiency Presenting as Compartment Syndrome
Background: Compartment syndrome of the upper extremity is a surgical emergency that most often presents after an identifiable event, such as a crush injury, a closed fracture, a snake bite, reperfusion after embolectomy, an electrical injury, or a circumferential burn.
Case Presentation: We report an unusual case in which an older woman was diagnosed with acquired factor VIII deficiency after presenting with acute nontraumatic compartment syndrome in her right upper extremity. Emergency medicine, plastic surgery, internal medicine, hematology, and hand therapy specialists worked together to identify the pathology and successfully treat the patient.
Conclusions: An isolated elevated partial thromboplastin time level in a patient who is bleeding should raise suspicions for factor VIII deficiency and immediately trigger further evaluation. Once this diagnosis is suspected, multidisciplinary treatment is indicated for immediate and long-term successful outcomes.
Discussion
Forearm compartment syndrome is most often caused by fractures—distal radius in adults and supracondylar in children.2 This case initially presented as a diagnostic puzzle to the emergency department due to the patient’s lucid review of several days of nontraumatic injury.
The clinical hallmarks of compartment syndrome are the 5 Ps: pain, pallor, paresthesia, paralysis, and pulselessness. Patients will describe the pain as out of proportion to the nature of the injury; the compartments will be tense and swollen, they will have pain to passive muscle stretch, and sensation will progressively diminish. Distal pulses are the last to go, and permanent tissue damage can still occur when pulses are present.1
Compartment Syndrome
Compartment syndrome is generally a clinical diagnosis; however, in patients who are sedated or uncooperative, or if the clinical findings are equivocal, the examination can be supplemented with intercompartmental pressures using an arterial line transducer system.2 In general, a tissue pressure of 30 mm Hg or a 20- to 30-mm Hg difference between the diastolic and compartment pressures are indications for fasciotomy.1 The hand is treated with an open carpal tunnel release, interosseous muscle release through 2 dorsal hand incisions, and thenar and hypothenar muscle release. The forearm is treated through a curved volar incision that usually decompresses the dorsal compartment, as it did in our patient. If pressures are still high in the forearm, a longitudinal dorsal incision over the mobile wad is necessary. Wounds can be closed primarily days later, left open to close by secondary intention, or reconstructed with skin grafts.2 In our patient, compartment syndrome was isolated to her forearm and the carpal tunnel release was performed prophylactically since it did not add significant time or morbidity to the surgery.
Nontraumatic upper extremity compartment syndrome is rare. A 2021 review of acute nontraumatic upper extremity compartment syndrome found a bleeding disorder as the etiology in 3 cases published in the literature between 1993 and 2016.4 One of these cases was secondary to a known diagnosis of hemophilia A in a teenager.5 Ogrodnik and colleagues described a spontaneous hand hematoma secondary to previously undiagnosed acquired hemophilia A and Waldenström macroglobulinemia.4 Ilyas and colleagues described a spontaneous hematoma in the forearm dorsal compartment in a 67-year-old woman, which presented as compartment syndrome and elevated PTT and led to a diagnosis of acquired FVIII inhibitor. The authors recommended prompt hematology consultation to coordinate treatment once this diagnosis issuspected.6 Compartment syndrome also has been found to develop slowly over weeks in patients with acquired FVIII deficiency, suggesting a high index of suspicion and frequent examinations are needed when patients with known acquired hemophilia A present with a painful extremity.7
Nontraumatic compartment syndrome in the lower extremity in patients with previously undiagnosed acquired hemophilia A has also been described in the literature.8-11 Case reports describe the delay in diagnosis as the patients were originally seen by clinicians for lower extremity pain and swelling within days of presenting to the emergency room with compartment syndrome. Persistent bleeding and abnormal laboratory results prompted further tests and examinations.8,9,11 This underscores the need to be suspicious of this unusual pathology without a history of trauma.
