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Nitza Goldenberg-Cohen, Judith Luckman, Rawan Omary, Daniel Rappoport, Hana Leiba, Shalom Michowiz, Yoav Vardizer, Helen Toledano; Primary tumors underlying unilateral orbital proptosis in children. Invest. Ophthalmol. Vis. Sci. 2017;58(8):2418.
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© ARVO (1962-2015); The Authors (2016-present)
To describe tumors inducing proptosis: the etiologies, management and outcomes.
A retrospective study in three tertiary centers. Review of files of children under 18 years of age at diagnosis, who presented with unilateral non-infectious, non-inflammatory and non-thyroid-related proptosis. Demographic data, etiology, diagnosis, eye exam at diagnosis and end of follow-up, treatment, outcome and imaging studies were summarized.
Nineteen children presented with unilateral proptosis, 12 girls and 7 boys. Etiologies were optic nerve glioma (ONG, 11); rhabdomyosarcoma (RMS, 2); arteriovenous malformation AVM (2); capillary hemangioma (1); juvenile xanthogranuloma (1) frontal bone osteoma (1) and localized hypertrophic neuropathy of the supra-orbital nerve (1).All but two of the children with ONG presented with substantially decreased visual acuity. Treatment varied between chemotherapy (3), combination chemo and radiation (1), chemo and resection (3), resection only (1), chemo and anti-VEGF (1), MEK inhibitor only (1) and observation only (1). Both visual and cosmetic outcomes were poor in all cases. One patient with AVM was treated with oral corticosteroids and maintained good VA, while the other underwent catheterization and had a fluctuating VA depending on intraocular pressure (IOP) and orbital drainage. One patient with RMS was treated with resection and radiation therapy and had good visual and cosmetic outcomes, while the other patient had tumor spreading to the face, received chemotherapy and died 9 months after diagnosis. The patients with juvenile xanthogranuloma (JXG) (1), frontal bone osteoma (1) and localized hypertrophic neuropathy of the supra-orbital nerve (1) underwent resection with good visual and cosmetic results. Similar outcome was seen in the patient with compound capillary hemangioma (1) who was treated with laser and systemic propranolol.
Proptosis is the presenting sign of an orbital tumor when inflammation and infection have been excluded. ONG was the most common etiology. All etiologies demand treatment, and may cause severe morbidity and mortality. All children with unilateral presentation, preserved vision of the fellow eye. Among benign etiologies, visual outcome of gliomas was the worst. The combination of proptosis and visual loss is an uncommon presentation and suggestive of orbital tumor in the pediatric population.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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