Abstract
Purpose: :
To determine the visual and systemic outcomes of children with ophthalmologic involvement at diagnosis of neuroblastoma and/or during the course of the disease.
Methods: :
A retrospective chart review at St. Jude Children’s Hospital identified 336 patients diagnosed with neuroblastoma from 1995 until present.
Results: :
Seventy patients had ophthalmic findings upon presentation and/or during the course of their disease (20.8%). Seven children had Horner syndrome (HS) at diagnosis and 12 patients developed post-operative HS. It persisted in all but one patient (5.3%) at the last exam. Seven patients had Opsoclonus-myoclonus-ataxia syndrome (OMA) at presentation. Only two of these twenty-six patients (7.7%) died of their disease. No visual loss was found in these children.Fifty-two patients had orbital metastases (either clinical or sub-clinical) at presentation and/or during recurrence of disease. Thirty-seven (71.2%) had proptosis/ecchymosis at diagnosis and/or recurrence. Thirty-one of the 52 (59.6%) patients died. Twenty-six of the 52 patients had recorded visual acuities; sixteen were 20/20-20/50, two were 20/60 to 20/100, three were between 20/200 and LP, and five patients had NLP vision.
Conclusions: :
Twenty percent of our patients with neuroblastoma developed ocular complications at some point in their disease. While HS and OMA were not associated with high mortality rates (7.7%) or ocular morbidity (0%), children with orbital metastases had higher rates of mortality (59.6%) and ocular morbidity (37%).HS and OMA in neuroblastoma are worrisome, but are not harbingers of a poor visual or systemic prognosis. However, orbital metastasis from neuroblastoma in our population was associated with significant morbidity and visual loss.
Keywords: neuro-ophthalmology: diagnosis • oncology • tumors