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  • By Shimon Rumelt, MD
    Oculoplastics/Orbit

    This retrospective study describes the clinical features and management of extensive ocular surface squamous neoplasia (OSSN) of the socket in three patients with ocular prosthesis. The malignancy was subtle, found in sockets with chronic discharge, and occurred following prosthesis wear of at least 13 years. Combined topical and injection IFNa2b could be an effective therapy.

    OSSN was detected in patients who were ages 60, 43, and 20 years and had worn ophthalmic prostheses for 54, 26 and 13 years, respectively. They all had chronic discharge and irritation, which two patients managed with intermittent topical corticosteroids. There were no predisposing factors of cigarette exposure, radiation exposure, eczema, systemic immune suppression or organ transplantation. The prosthesis fit well with nonirritative edges. At presentation, OSSN was subtle, vascular and multifocal, with the largest lesions or confluence of lesions measuring 20, 25 and 20 mm, respectively. The tumors involved the tarsal conjunctiva in all three cases and the bulbar and forniceal surfaces in two cases. This propensity for tarsal tumor location raises the possibility of the role of chronic irritation from eyelid movement over the artificial prosthesis surface.

    The patients were administered Interferon α 2b (IFNa2b) eye drops (1 million units/cc) four times daily until complete regression, followed by a twice-daily maintenance phase, and IFNa2b sublesional injection (5 million units/0.5cc to 8 million units/0.8 cc). Complete regression was achieved in two cases—at one month and 20 months—and partial regression in one case (at nine months). All patients continue on chronic maintenance IFNa2b topically. There were no recurrences, and IFNa2b injection side effects of nausea and chills were minor, lasting one day. No patient required surgical removal of tumors from the socket or exenteration.

    The cause of OSSN in the anophthalmic socket remains unknown. Several factors have been proposed, including chronic irritation from poorly fitting prosthesis leading to conjunctival inflammation and epithelial dysplasia with eventual transformation into carcinoma. The role of human papillomavirus remains speculative, as does the role of topical immune suppression with corticosteroid eye drops. The development of OSSN in this series could have been multifactorial with chronic localized corticosteroid-related immune suppression in an environment of chronic inflammation.

    The authors failed to mention the number and frequency of injections and the duration of the injection period. Although IFNa2b is a tempting alternative to exenteration, a larger number of patients and a longer follow-up are required to confirm these results. One conclusion that can be made is that OSSN can develop in patients wearing prosthesis, probably because of constant friction, and careful examination of the cul de sac is warranted.