Exploding retinoblastoma- Management of spontaneous scleral rupture in an infant with bilateral retinoblastoma.

PURPOSE: To describe a case of spontaneous globe rupture in an infant with bilateral retinoblastoma (RB) and the challenges faced during its management. METHODS: An infant with bilateral retinoblastoma on treatment, presented with spontaneous globe rupture in the eye with advanced disease, after an episode of conjunctivitis while on neoadjuvant chemotherapy with VEC (vincristine, etoposide and carboplatin) regimen, and was managed by urgent enucleation followed by chemotherapy and radiotherapy. RESULTS: Metastatic workup, post enucleation was negative, and she had good regression of lesions in the other eye with chemotherapy and maintains a Cardiff acuity of 6/24. Follow up of 2 years showed no recurrences. CONCLUSION: Spontaneous globe rupture is an exceptionally rare complication of retinoblastoma, this being the second reported case worldwide. Advanced disease stage, high intraocular pressure, chemotherapy induced immunosuppression, and superadded infection could all be the possible etiologies in this case. This condition carries a significant risk of life-threatening metastasis.