Temporal Evolution of Hypertrophic Olivary Degeneration in a Pediatric Patient: A Case Report and Review of Literature

Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration seen after interruption of the dentato-rubro-olivary tract also known as the Guillain-Mollaret triangle (GMT). It is associated with hypertrophic changes of the inferior olive. Commonly reported in adults, this lesion presents with ataxia and oculopalatal myoclonus. Up to date, few cases have been published in the literature that refer to pediatric cases. This diagnosis is particularly important in the setting of brainstem tumor surgery as it should not be confused for tumor recurrence or metastasis, in turn avoiding unwarranted surgical intervention. We present the case of a 15-year-old male who underwent resection of a left superior cerebellar peduncle (SCP) pilocytic astrocytoma. On follow-up, magnetic resonance imaging (MRI) demonstrated evidence of mild residual tumor as well as progressive engorgement of the inferior olivary nucleus (ION). The patient was clinically asymptomatic and has since been observed expectantly without any issues. We were able to pinpoint the most probable location of injury in our patient’s GMT. HOD remains a somewhat obscure entity. Its presentation may be early and not accompanied by significant neurologic findings, in contrast to what has been previously reported. Particularly in neoplastic cases, it may represent a diagnostic challenge and could be easily confused for tumor recurrence. A multidisciplinary approach for this entity, as with other pathologies, is of particular importance. Its proper recognition will result in the best outcomes for the patient.