Opsoclonus in Children: Diagnosis, Etiology and Ophthalmologic Assessment of Patients at a Tertiary Children’s Hospital

Introduction: Opsoclonus is often associated with serious neurologic and paraneoplastic pathology. Pediatric ophthalmologists play an important role in its diagnosis. Methods: Retrospective chart review of patients seen for suspicion of opsoclonus. Results: 259 patients were identified for whom opsoclonus was suspected, of which 83 (32%) were found to be true opsoclonus. The ophthalmology consultation changed the course of workup in 44 of the 117 patients that received ophthalmologic evaluation (38%). 16 (9%) were found to have primary ophthalmic diagnoses. Of the 83 children with opsoclonus, 36 (43%) had paraneoplastic opsoclonus-myoclonus-ataxia syndrome (OMAS), 32 (39%) had non-paraneoplastic OMAS, 1 (1.2%) had optic pathway glioma, 5 (6.0%) had other neurologic diseases, 2 (2.4%) had hydrocephalus, 6 (7.2%) had benign neonatal opsoclonus, and 1(1.2%) had opsoclonus of unknown etiology. Most patients (78 patients; 94%) received brain MRIs, followed by MRI chest/abdomen/pelvis studies and urine catecholamines in 57 patients each (69%). Conclusions: Extensive workup is usually performed to rule out underlying neoplastic pathology and includes MRI brain, neck, chest, and abdomen, and urine catecholamine studies. Pediatric ophthalmologists can help to make critical ophthalmic diagnoses in a minority of cases. If involved early in the diagnostic course, this may spare children unnecessary testing.