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E. M. Bratton, M. E. Hoehn, E. B. Morris, N. C. Kerr; Presenting Ophthalmic Signs and Symptoms of Neuroblastoma. Invest. Ophthalmol. Vis. Sci. 2010;51(13):959.
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To determine the incidence of Horner's syndrome, proptosis, ecchymosis, and Opsoclonus-myoclonus-ataxia (OMA) syndrome at presentation of neuroblastoma, noting concurrent systemic findings.
We performed a retrospective chart review at St. Jude Children’s Research Hospital, identifying 336 patients diagnosed with neuroblastoma since 1995.
Of the children diagnosed with neuroblastoma, 45 children (13.4%) had ophthalmic findings at presentation. Seven children had Horner's syndrome [57% (n=4) had associated systemic signs]. Sixteen children had ecchymosis, most with systemic signs (n=15, 93.8%). Ecchymosis was usually bilateral (n=14, 87.5%) and sometimes associated with proptosis (n=5, 31.3%). Nineteen had proptosis, 89.5% of which also had systemic signs (n=17). Three children with proptosis also had ptosis of the involved eye. Two children with proptosis had papilledema. Seven had OMA (3 with associated systemic signs, 42.9%). In addition, one child had papilledema as the only sign at presentation. The majority of ophthalmic signs occurred with other systemic signs of neuroblastoma (86.7%).
Prior studies have not investigated the co-existence of ophthalmic and systemic signs in children presenting with neuroblastoma. Ophthalmic signs were noted at presentation in a minority of children with neuroblastoma. Of these, the vast majority (86.7%) had systemic findings.Ophthalmologists should be aware that most children who present with ophthalmic signs of neuroblastoma also have systemic findings. Therefore careful history and complete physical examination is warranted when ophthalmic signs are noted.
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