• Sandipan Pati, Dimitris Kombogiorgas, Ahmad Anwar, and Rupert F Price.
• Department of Neurosurgery, University Hospital of North Staffordshire, ST4 7LN Hartshill, Stoke on Trent, United Kingdom.
• Surg Neurol. 2009 Jun 1; 71 (6): 689-92, discussion 692.

BackgroundAlthough 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 DescriptionWe 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.ConclusionsSpontaneous 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.

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