Associations of Refractory Spontaneous Intracranial Hypotension and Chronic Subdural Hematoma with Factor XIII Deficiency: A Case Report.

The co-occurrence of spontaneous intracranial hypotension (SIH) due to thoracic dural tear and factor XIII deficiency (FXIIID) is rare and may result in chronic subdural hematoma (CSDH). CSDH often recurs and is difficult to treat, despite appropriate treatment. However, there is no definitive knowledge on the optimal timing of factor XIII (FXIII) supplementation or therapeutic interventions, such as epidural blood patch (EBP) and burr hole drainage (BHD). We present a case of refractory SIH and CSDH associated with FXIIID. Considering an ineffective initial EBP, we performed a second EBP after observing sufficiently high FXIII activity; SIH was cured subsequently. The patient experienced SIH recurrence after 14 months and was treated with a combination of EBP and BHD, with sufficient FXIII supplementation. CSDH disappeared and did not recur for more than 12 months. During the treatment of SIH and CSDH associated with FXIIID, surgical treatment such as EBP appeared ineffective due to low FXIII activity. This necessitates intravenous supplementation of FXIII to maintain sufficient FXIII activity. Regular monitoring of FXIII activity is also necessary to prevent CSDH recurrence.