Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder of dysregulation of cerebrovascular tone resulting in transient segmental vasoconstriction which resolves in 1-3 months.
Cerebral edema is an underrecognized complication in RCVS. It is likely multifactorial. This
edema can lead to
intracranial hypertension that can be refractory to medical management. Limited evidence exists regarding surgical management of
intracranial hypertension in RCVS. We present a 29-year-old Caucasian right-handed female patient with a medical history of
migraine, polysubstance abuse presented to the emergency department (ED) daily for 3 days with the chief complaint of recurrent
thunderclap headache. She declined neuroimaging and lumbar puncture. She was treated for
migraine with abortive medications with no improvement. During the third ED visit, she became lethargic with right-sided
homonymous hemianopia. Computerized tomography of the brain showed left parietal
intracerebral hemorrhage with intraventricular extension, cortical
subarachnoid hemorrhage, and diffuse
cerebral edema. Digital subtraction angiography showed multifocal moderate-to-severe segmental vasoconstriction suggestive of vasculopathy. Oral
verapamil was initiated. Continuous intracranial pressure monitoring showed uncontrolled
intracranial hypertension, despite maximal medical management with hyperosmolar
therapy, induced
coma, and
hypothermia. Decompressive hemicraniectomy with duraplasty was performed for refractory
intracranial hypertension. We provisionally diagnosed her with RCVS. She was discharged to inpatient rehabilitation with residual right
homonymous hemianopia. Transcranial Doppler study during follow-up showed improved mean flow velocities. She continued to have residual cognitive deficits with complete resolution of
headache.