INADVERTENT INTRAOCULAR SURGERY IN CHILDREN WITH UNSUSPECTED RETINOBLASTOMA: A Study of 14 Cases.

PURPOSE: To study the clinical presentation and treatment outcome of patients who underwent inadvertent intraocular surgery before the diagnosis of retinoblastoma. DESIGN: Retrospective study of 14 patients who had undergone an inadvertent intraocular surgery before the diagnosis of retinoblastoma. RESULTS: The mean age at presentation to the ocular oncology clinic was 69 months. The most common initial misdiagnosis was endophthalmitis (n = 4). The most common inadvertent intraocular surgeries were pars plana vitrectomy (n = 6) with/without lensectomy and evisceration with/without previous pars plana vitrectomy (n = 5). The mean interval between intraocular procedure and initiation of treatment for retinoblastoma was 7 months. At presentation in the oncology clinic, the tumor was intraocular (n = 3), with extrascleral tumor extension (n = 11), and/or optic nerve tumor extension (n = 5). All patients were started on multimodal treatment including 12 cycles of high-dose systemic chemotherapy, enucleation or orbital exenteration, and orbital external beam radiotherapy. Over a mean follow-up period of 27 months, 8 (57%) patients died because of progressive disease despite initiation of treatment. CONCLUSION: Misdiagnosis and inadvertent surgical intervention in cases of retinoblastoma in combination with delayed initiation of appropriate treatment is associated with poor prognosis. High index of suspicion for retinoblastoma is needed to avoid misdiagnosis and mismanagement.

RETINOBLASTOMA IN INDIA: Clinical Presentation and Outcome in 1,457 Patients (2,074 Eyes).

PURPOSE: To study the clinical presentation, treatment, and outcome of patients with retinoblastoma (RB) in India. METHODS: Retrospective study of 1,457 patients with RB (2,074 eyes). RESULTS: The mean age at presentation of RB was 29 months (median, 24 months; range, <1-370 months). There were 812 (56%) men and 645 (44%) women with unilateral presentation of RB in 57% (n = 834) and bilateral in 43% (n = 623). Familial RB was present in 4% (n = 55). The most common presenting complaints included leukocoria (n = 1,100; 75%), proptosis (n = 91; 6%), strabismus (n = 77; 5%), and red eye (n = 68; 5%). Most (n = 1,889; 91%) tumors were intraocular in location, and 185 (n = 185; 9%) had extraocular tumor extension at presentation. The most common modalities of primary treatment-included systemic chemotherapy (n = 1,171; 60%) and enucleation (n = 674; 35%). At a mean follow-up period of 44 months (median, 30 months; range, 3-234 months), 92% (n = 1,206) were alive, and 108 (8%) patients died because of RB. Based on Kaplan-Meier analysis, the survival at 1, 3, 5, and 10 years was 94%, 91%, 90%, and 89%, respectively. CONCLUSION: The most common presenting signs of RB in Asian Indian population are leukocoria and proptosis. With appropriate treatment, the survival rate is favorable at 92%.