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

Background

Opsoclonus is often associated with serious neurological and paraneoplastic pathology. Pediatric ophthalmologists play an important role in its diagnosis.

Methods

This study was a retrospective chart review of patients seen for suspicion of opsoclonus.

Results

A total of 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 evaluation in 44 of the 117 patients who received ophthalmologic evaluation (38%). Sixteen (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 nonparaneoplastic OMAS, one (1.2%) had optic pathway glioma, 5 (6.0%) had other neurological diseases, 2 (2.4%) had hydrocephalus, 6 (7.2%) had benign neonatal opsoclonus, and one (1.2%) had opsoclonus of unknown etiology. Most patients (78 patients; 94%) received brain magnetic resonance imaging (MRI), followed by MRI of the chest/abdomen/pelvis and urine catecholamines in 57 patients each (69%).

Conclusions

Extensive evaluation is usually performed to rule out underlying neoplastic pathology and includes MRI of the 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.