Journal of Neurosciences in Rural Practice
 
CASE REPORT
Year : 2019  |  Volume : 10  |  Issue : 2  |  Page : 355-359

Decompression hemicraniectomy for refractory intracranial hypertension in reversible cerebral vasoconstriction syndrome


Division of Neurocritical Care, Cerebrovascular Center, Cleveland Clinic Foundation, Cleveland, Ohio, USA

Correspondence Address:
Naresh Mullaguri
Division of Neurocritical Care, Cerebrovascular Center, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jnrp.jnrp_334_18

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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.


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