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P.D. Gandhi, J.C. Fleming, B.G. Haik, M.W. Wilson; Ophthalmic Complications Following Treatment of Primary Orbital and/or Paranasal Sinus Rhabdomyosarcoma . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5400.
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Purpose: To identify the type and frequency of ocular and orbital complications observed following treatment for primary rhabdomyosarcoma of the orbit and/or paranasal sinuses. Methods: A retrospective chart review was performed to identify all patients treated at one institution from 1972 to the present with biopsy–proven rhabdomyosarcoma of the orbit and/or paranasal sinuses. All patients were treated with a combination of chemotherapy and/or radiation. Pre–treatment and follow–up data were extracted from the patients’ medical records. Results: A total of 21 patients (12 male, 9 female) of median age 7.0 years (range 1.0 – 14.5 years) with primary orbital (19) or sinus (2) rhabdomyosarcoma (17 embryonal, 3 alveolar, 1 parameningeal) were treated and followed for a median period of 9.0 years (range 0.5 – 18.5 years). Pre–treatment ophthalmic co–morbidities were pain (4), dystopia or proptosis (15), ophthalmoplegia (10), eyelid swelling (13), and vision loss worse than 20/200 (3). Only one patient received chemotherapy and wide excision of an eyebrow lesion without radiation, and had no other ophthalmic complications. Twenty patients were treated with both chemotherapy and radiation (total dose 35.0 – 59.4 Gy) for their orbital or sinus tumors. In this group, the following ophthalmic complications were observed in the follow–up period: corneal dryness (16), conjunctival injection (8), cataract (9), orbito–facial bony hypoplasia (7), opportunistic infections (5), enophthalmos from orbital fat atrophy (4), persistent eyelid erythema or cellulitis (4), trichiasis (3), central corneal opacity (4), band keratopathy (2), phthisis bulbi (2), permanent blindness (4), draining periorbital fistula (3), radiation retinopathy (2), eyelid ptosis (2), and strabismus (1). Additionally, one patient with severe exposure keratopathy required a complete tarsorrhaphy, one developed socket contracture following enucleation and tumor debulking, and two had total orbital exenteration for progressive disease. Conclusions: Over a 32–year period, the combination of chemotherapy and radiation has been the most commonly used treatment modality for primary orbital and/or sinus rhabdomyosarcoma. The most frequently observed treatment–induced ophthalmic complications were corneal dryness, conjunctival injection, and cataract. Fortunately, these could be managed with minimal patient morbidity. Surprisingly, vision–threatening complications were infrequently encountered.
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