Retinoblastoma treatment in a Brazilian population. Presentation and long-term results.

INTRODUCTION: Retinoblastoma is a malignant tumor with a high cure potential when proper therapy is used. The purpose of this paper is to report the clinical features and outcomes of patients with retinoblastoma who were treated with a combination of local and systemic chemotherapy-based protocols. METHOD: We retrospectively studied patients treated with systemic chemotherapy plus local treatment between 2003 and 2015 with a follow-up ≥2 years. We correlated clinical and pathological characteristics with decimal visual acuity (VA) and death. RESULTS: Among 119 patients, 60% had unilateral disease (UNI), and 52% were male. The median presentation age was 19.5 months, 10% had a positive family history, and the most frequent sign was leukocoria (68.8%). Advanced disease was more frequent in eyes with UNI (98.4%) than in eyes with bilateral retinoblastoma (BIL: 55.3%). Enucleation was performed in 97% of UNI eyes and in 55.8% of BIL eyes. The overall globe salvage was 26.6%, 44.25% of BIL eyes. Bilateral enucleation was required in 5%. High-risk pathologic features occurred in 50% and 37% of eyes enucleated without and with neoadjuvant chemotherapy, respectively. High-risk features were related to the presence of goniosynechiae in the pathologic specimen and were more frequent in children younger than 10 months or older than 40 months. Extraocular disease was present in 5% of patients, and the death rate related to metastasis of the tumor was 8%. The final VA was ≥ 0.7 in 72.8% and ≥0.1 in 91% of BIL patients. CONCLUSIONS: Treatment of retinoblastoma with conservative systemic-based chemotherapy was associated with an excellent survival rate (92%). Albeit the low overall globe salvage rate, in BIL patients, approximately half the eyes were conserved, and a satisfactory functional visual result was achieved The evaluated protocol is an important treatment option, especially in developing countries.

Piggyback Photochromic Contact Lens for Visual Rehabilitation and Photophobia Management in Traumatic Aniridia.

ABSTRACT: We report herein a case of fitting with a photochromic silicone hydrogel contact lens under a rigid gas-permeable lens (piggyback system) for photophobia and low vision correction after traumatic aniridia and aphakia. A 40-year-old woman was referred to our practice for contact lens fitting in her right eye, which was left aphakic after an open globe injury. She also presented traumatic aniridia in the right eye, and her left eye had been previously eviscerated. A successful fitting was obtained with a photochromic silicone hydrogel (senofilcon A) contact lens, with a Dk/t of 121 × 10-9, under an aspheric design, +13.00 D rigid gas-permeable lens. The patient displayed visual acuity and contrast sensitivity improvement and reported decreased photophobia.