Otitic hydrocephalus and papilloedema-re-evaluating a treatment paradigm

Otitic hydrocephalus is increased intracranial pressure without ventricular dilation secondary to mastoiditis and cerebral venous sinus thrombosis (CVST). It is associated with significant visual morbidity, though more detailed data on visual outcomes is lacking. We sought to better characterize the management of increased intracranial pressure and visual outcomes in this population.

Retrospective chart review at a quaternary Children's Hospital of patients <18 years of age who were diagnosed with otitic hydrocephalus from January 2009 to July 2023. Data were collected on patient demographics, clinical course, imaging, and treatment outcomes.

Fifteen patients were identified with mastoiditis complicated by otitic hydrocephalus. The average age was 5.7 years (range 2-15). Eight patients were male (53%). Six patients (40%) had cranial nerve VI palsy and 14 (93%) developed papilloedema. Eleven patients (73%) developed progressively worsening papilloedema despite improving infection, clot burden, and acetazolamide; of these, three required ventriculostomy catheters. Eight were started on corticosteroids. Six had resolution of papilloedema without the need for shunt placement. Two patients had evidence of optic atrophy from increased ICP and visual loss prior to corticosteroid initiation. Both had stabilization of their condition without further visual loss.

This study highlights the importance of systemic corticosteroids as part of the treatment paradigm for otitic hydrocephalus to prevent vision loss in patients otherwise unresponsive to medical management. It also identifies the risk of papilloedema progression and visual morbidity even after the initiation of appropriate medical therapy. Visual outcomes were significantly improved and invasive neurosurgical procedures were avoided with use of corticosteroids.