Direct Optic Nerve Infiltration by Cerebellar Vermian Medulloblastoma in a Pediatric Patient

Medulloblastoma in pediatric patients often leads to obstructive hydrocephalus with papilledema and optic neuropathy. Here we present a case of optic atrophy in the setting of direct optic nerve infiltration after papilledema from non-obstructive cerebellar vermian medulloblastoma.

Poster 91 Direct Optic Nerve Infiltration by Cerebellar Vermian Medulloblastoma in a Pediatric Patient Shehroz Rana 1, Rita Okumu 2, John Paddock 2, Cristiano Oliveira 3, Marc Joshua Dinkin 3 Weill Cornell Medical College Qatar, 2 Weill Cornell Medical College, 3 Weill Cornell Medical College, New York, United States of America 1 Introduction: Medulloblastoma in pediatric patients often leads to obstructive hydrocephalus with papilledema and optic neuropathy. Here we present a case of optic atrophy in the setting of direct optic nerve infiltration after papilledema from non-obstructive cerebellar vermian medulloblastoma. Description of Cases: A 14 year old boy with history of H. pylori developed nausea that did not improve with antibiotic therapy, followed by bilateral blurry vision. Examination showed decreased visual acuity to 20/40 OD and 20/100, bilateral Frisen grade 4 papilledema and bilateral sixth nerve palsies. Lumbar puncture showed elevated opening pressure of 55 cm H2O and few atypical hematopoietic progenitor cells in the CSF with inconclusive cytopathology. MRI brain revealed white matter signal changes in the anterior temporal lobes, tonsillar herniation, and small foci of superficial enhancement along the cerebellar vermis with trace susceptibility, initially favored to be possibly leptomeningeal or superficial cortical vasculature. Treatment with acetazolamide did not improve symptoms, and vision continued to decline. The cerebellar vermian lesion was biopsied and revealed medulloblastoma. Bilateral optic nerve sheath fenestration and shunt placement normalized ICP but did not improve visual function or papilledema. He was treated with whole brain radiation followed by Avastin, Irinotecan, and Temodar. Disc edema resolved after systemic therapy, raising the possibility of leptomeningeal infiltration of the optic nerves. Unfortunately, vision did not improve and stabilized at 20/300 OD and CF 3' OS. Conclusions, including unique features of the case: Optic disc edema in this case was not related to elevated intracranial pressure from obstructive hydrocephalus and did not resolve with normalization of ICP, but after treatment of the underlying medulloblastoma. This atypical presentation of medulloblastoma highlights possible alternative sources of optic disc edema besides elevated intracranial pressure such as suspected leptomeningeal optic nerve infiltration, which should be considered. Ophthalmic findings can be the presenting symptom of medulloblastoma, which may appear atypical on initial neuroimaging. References: Garrity, Herman, Dinapoli, Waller, Campbell, Isolated metastasis to optic nerve from medulloblastoma, Ophthalmology, 976, 207-210 Chan, Voss, Papilledema and Infiltrative Optic Neuropathy as Presenting Signs of Medulloblastoma, Neuro-Ophthalmology, 31, 11-13, 2007 Hirst, Miller, Jumar, Udvarhelyi, American Journal of Ophthalmology, 1980 Keywords: High intracranial pressure/headache, Neuroimaging, Tumors, Pediatric neuro-ophthalmology, Visual fields Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: Shehroz Rana, [e-mail] 2023 Annual Meeting Syllabus | 155