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

Background: Although congenital afibrinogenemia can commonly present with hemorrhage from the umbilical cord at birth, or with spontaneous mucosal or intracranial hemorrhage in the neonatal period, life-threatening intracerebral hemorrhage in adults is infrequent. Case Description: We report a 32-year-old woman with congenital afibrinogenemia. Postoperatively, she developed bilateral pulmonary emboli despite the fact that her INR was elevated to 2.3. Highly purified fibrinogen concentrate infusion may have partly contributed to this complication. An inferior vena caval filter was used successfully to prevent further pulmonary emboli. Conclusions: 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.