Spontaneous acute subdural hematoma and chronic epidural hematoma in a child with F XIII deficiency.

Factor XIII (F XIII) deficiency is a rare autosomal recessive congenital disorder that can cause spontaneous subdural or epidural hematomas. Due to its low incidence, F XIII deficiency may well be under-diagnosed. A 7-year-old girl with no history of medical problems presented with progressive headache of 3 days. Cerebral computed tomography (CT) scans revealed a large right acute parietooccipital subdural hematoma with a significant midline shift. After an emergent parietooccipital craniotomy and evacuation of the subdural hematoma, a screening test for factor XIII was performed. The results of the test were abnormal. She had full recovery and was discharged with a follow-up treatment of monthly transfusion of fresh frozen plasma as the replacement and prophylactic therapy. Ten months later, she was referred to our center with headache after a minor head trauma. Her medical history revealed that she had not received fresh frozen plasma for the last 2 months. CT scan showed a chronic right parietal epidural hematoma beneath the craniotomy flap. The present case indicates that although its incidence is very rare, F XIII deficiency can cause acute or chronic subdural and epidural hematomas. Therefore, in acute or chronic subdural and epidural hematomas with no underlying cause, the presence of a potential F XIII deficiency should be suspected as a cause of hemorrhagic diathesis.