A 1-year-old boy presented with 5 weeks of periorbital ecchymosis, without associated trauma. Contrast-enhanced head and face CT reveals extensive osseous metastases involving facial bones and skull with spiculated periosteal reaction along the orbital walls (Fig. 1, arrows). Subsequent evaluation with iodine-123 metaiodobenzylguanidine (123I-MIBG) (Fig. 2) confirms a right paraspinal neuroblastoma (arrow) with widespread tumoral involvement of cortical bone and bone marrow.
Almost 60% of patients with neuroblastoma will have metastases at diagnosis. Periorbital metastases cause proptosis or orbital ecchymosis, resulting in the clinical raccoon-eye appearance. Ophthalmic involvement, reported in 10% to 43% of cases of neuroblastoma, may also manifest as opsoclonus, Horner syndrome, ocular motility defects or blindness [1].
123I-MIBG imaging, with its high sensitivity and specificity for neuroblastoma, will confirm the diagnosis, facilitate accurate disease staging and allow differentiation from orbital trauma, including that from child abuse, which can also present with raccoon-eye appearance [2].
References
Smith SJ, Diehl NN, Smith BD et al (2010) Incidence, ocular manifestations, and survival in children with neuroblastoma: a population-based study. Am J Ophthalmol 149:677–682
Bohdiewicz PJ, Gallegos E, Fink-Bennett D (1995) Raccoon eyes and the MIBG super scan: scintigraphic signs of neuroblastoma in a case of suspected child abuse. Pediatr Radiol 25:S90–S92
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Stanescu, L.A., Parisi, M.T. ‘Raccoon eyes’ revisited. Pediatr Radiol 40 (Suppl 1), 170 (2010). https://doi.org/10.1007/s00247-010-1865-6
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DOI: https://doi.org/10.1007/s00247-010-1865-6