World neurosurgery | 2019
Perioperative management of a patient with severe factor V deficiency presenting with chronic subdural hematoma: a clinical report.
Abstract
BACKGROUND\nSevere factor V deficiency is an extremely rare coagulation disorder. Patients with factor V activity below 5% usually become symptomatic in early childhood.\n\n\nMETHODS\nWe report the case of an 82 year old woman with incidentally diagnosed severe factor V deficiency, who developed a symptomatic chronic subdural hematoma, requiring burr-hole craniostomy. Successful management was achieved by a multidisciplinary approach. Preoperatively, factor V activity was increased from 2 % to 50 % by administration of 25 ml/kg body weight of fresh frozen plasma over 30 minutes under close cardiopulmonary monitoring on ICU. Straight afterwards, the patient was transferred to the operating room where surgery was performed under general anesthesia. Burr-hole craniostomy could be performed without perioperative complications. In the postoperative days there was no relevant recurrence of the subdural hematoma in the follow-up CT scans under frequent control of coagulation parameters. However, despite further transfusion of FFP, factor V activity did not increase above 16%.\n\n\nRESULTS\nThe patient was discharged without any neurological deficits. In a hemostaseologic follow-up two months after surgery, factor V activity below 1% was confirmed with evidence of a factor V inhibitor in the modified Bethesda assay. Most likely, the patient suffered from an acquired form of factor V deficiency with preformed antibodies that had been boosted by the initial treatment with FFP.\n\n\nCONCLUSION\nWe conclude that in this rare bleeding disorder, intracranial surgery was successfully managed due to a thoroughly planned perioperative therapeutic strategy. However, if there is time prior to surgery, a full check-up of the bleeding disorder is advisable.