Spontaneous extra-axial intracranial hemorrhage followed by thrombosis in congenital afibrinogenemia: perioperative management of this rare combination

Spontaneous intracerebral hemorrhage must be included in the differential diagnosis in patients with known afibrinogenemia presenting with symptoms suggesting raised intracranial pressure. Immediately after surgery, intracranial pressure monitoring of patients is mandatory to pick up further intracranial bleeding early. Fibrinogen replacement therapy is recommended before surgery, but its use as a long-term prophylaxis against hemorrhage should be weighed against the risk of thrombosis.