Idiopathic Intracranial Hypertension Following Non-aneurysmal Spontaneous Subarachnoid Hemorrhage in a Sickle Cell Disease Patient with Suspected Reversible Cerebral Vasoconstriction Syndrome
Leslie Beltran1, Prescott Cheong1, Hania Zafar1, Rachael Osaitile1, Shrenevas Nandam1, Daniel Davila-Williams2
1Baylor College of Medicine, 2Texas Children's Hospital
Objective:
To describe a pediatric patient with sickle cell disease presenting with non-aneurysmal spontaneous subarachnoid hemorrhage (SAH) suspected secondary to Reversible Cerebral Vasoconstriction Syndrome (RCVS), later complicated by Idiopathic Intracranial Hypertension (IIH).
Background:

We present a 6-year-old male with HbSS and mild persistent asthma was admitted for vaso-occlusive crisis and developed acute neurologic symptoms on hospital day 4, including severe headache, left eye pain, and hypertension (Pediatric NIH Stroke Scale: 3). Magnetic resonance imaging (MRI) stroke study showed a small right frontal subarachnoid hemorrhage (SAH) with adjacent thin subdural collection (SDH) and vessel imaging (CTA brain) showed findings suggestive of possible reversible cerebral vasoconstriction syndrome (RCVS). He was started on nimodipine and repeat imaging 7 days after admission revealed interval development of several small foci of ischemia in the posterior greater than anterior white matter, left parasagittal occipital, and parietal lobes with mild associated cortical swelling. FLAIR imaging showed a possible Ivy sign suggestive of cerebral vasculopathy, however dedicated vessel imaging was unrevealing.

He remained in the intensive care unit for close neuromonitoring for continued intractable headaches, while undergoing serial transcranial dopplers (TCD). He subsequently reported vertical diplopia 11 days after admission, and TCDs showed elevated pulsatile indices suggesting possible increased intracranial pressure. Ophthalmology confirmed bilateral grade III papilledema on their examination. Lumbar puncture obtained showed an opening pressure of > 55 cm H20 consistent with IIH, for which he was started on acetazolamide, with gradual improvement in headache and his papilledema.

Design/Methods:
NA
Results:
NA
Conclusions:
This case is notable as a novel report of spontaneous SAH in a pediatric sickle cell patient with delayed cerebral ischemia and subsequent IIH. While sickle cell-related vasculopathy is well-documented, its association with RCVS remains unclear. Post-inflammatory changes, possible venous outflow obstruction and CSF pressure disorders in sickle cell disease may contribute to IIH pathogenesis in such cases.
10.1212/WNL.0000000000217632
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