Cerebrospinal fluid venous fistula causing spontaneous intracranial hypotension resulting in venous sinus thrombosis and coma.
Timothy G White, Justin Turpin, Miriam Shao et al.
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Case in brief
A patient developed progressive cerebral venous sinus thrombosis and coma. Investigation showed low intracranial pressure consistent with spontaneous intracranial hypotension (SIH); invasive monitoring and imaging confirmed the pressure abnormality. Dynamic myelography identified a cerebrospinal fluid (CSF) venous fistula at T2/3. Transvenous embolization was followed by resolution of symptoms and almost immediate improvement in both the thrombosis and intracranial hypotension.
What made this case unusual
The CSF venous fistula-associated SIH presented with progressive venous sinus thrombosis and coma, rather than the headache-focused presentation described in most documented cases.
Diagnostic / clinical pearl
In a patient with cerebral venous thrombosis and unexplained low intracranial pressure, consider SIH from a CSF venous fistula; dynamic myelography can identify the fistula.
Why it matters
Recognizing this underlying cause may enable targeted fistula treatment; in this case, embolization was followed by rapid improvement in thrombosis, intracranial hypotension, and symptoms.