Spontaneous intracranial hypotension complicated by refractory subdural hematomas in a patient with coagulation factor XIII deficiency

Abstract Spontaneous intracranial hypotension (SIH) is often secondary to an occult cerebrospinal fluid (CSF) leak in the neuroaxis. It is sometimes associated with chronic subdural hematomas (SDH). The authors present the case of a 61 year-old male who presented with SIH from an occult CSF leak and complicated by refractory SDH. The patient underwent treatment for SIH with multiple epidural blood patches, hydration and bed rest. In addition, he underwent evacuation of his SDH twice, once via burr hole drainage and the second via craniotomy. Once the diagnosis of coagulation factor XIII (CFXIII) deficiency was made, the patient was treated with recombinant factor XIII followed by an epidural blood patch. He subsequently made a complete recovery. In this report, the authors propose management strategies for treating patients with SIH complicated by refractory SDH and coagulopathies by reviewing the literature.