2020 Volume 42 Issue 3 Pages 171-175
Congenital afibrinogenemia, which is one of the coagulation disorders, is an autosomal recessive inherited hemorrhagic disease. Incidence of this disease is extremely rare. We report a case of afibrinogenemia-related intracerebral hemorrhage and discuss about characteristics and management of this clinical situation. A 46-year-old male patient was followed up for congenital afibrinogenemia at our department of hematology. He was taken to the emergency department of a local hospital by ambulance because he had headaches and numbness of the whole body from 2 days ago. Although CT scan showed intracranial hemorrhage, he was thought necessary for intensive care, because of congenital afibrinogenemia, and was transferred to our hospital. CT scan showed a subcortical hematoma in the right parietal region and subarachnoid hematoma in the left fronto-temporal region. Result of his blood test shown as the following: PT-INR ≥ 10, APTT ≥ 360 s, fibrinogen < 30 mg/dL. He developed consciousness level reduction and appeared anisocoria with left hemiparesis. He underwent craniectomy and external decompression on the right frontotemporal area, and hematoma removal for the subcortical hemorrhage with fresh frozen plasma (FFP) transfusion. He had no postoperative hemorrhage in the perioperative period with FFP transfusion and fibrinogen administration. There are currently no definitive guidelines for the perioperative management of patients with congenital afibrinogenemia in a case of intracerebral hemorrhage. We found that it is necessary to control the fibrinogen value >50 mg/dL in the case of surgery for intracerebral hemorrhage, as other major surgery.
