Session Title: Uveitis & Intraocular Tumours
Session Date/Time: Friday 27/09/2013 | 14:30-16:00
Paper Time: 15:42
Venue: Hall C (Level 1)
First Author: V.Prabhu INDIA
Co Author(s): D. Ambike A. Kulkarni
Retinoblastoma(RB) is most common intraocular malignancy in children. Retinoblastoma management has seen huge advances and a paradigm shift towards chemo reduction along with serially aggressive local therapy (SALT) and /or focal consolidation. Management of bilateral Retinoblastoma especially in advanced stages at presentation (common in developing countries) is a challenge. We report a case of bilateral Retinoblastoma; one eye enucleated; other eye salvaged with systemic chemoreduction and subtenons carboplatin (2 injections) showing partial regression of the tumor thus salvaging the only remaining eye .
One year old female child referred to our clinic by a pediatrician who noticed white reflex in the child’s left eye (LE) when the child was brought to him for fever and running nose by the parents. No history of premature birth, ocular trauma.
The child followed light in Right Eye (RE) and did not follow light in LE and objected to occluding RE. Evaluation under anesthesia showed RE endophytic growth i.e. yellowish white mass filling the vitreous cavity inferior half with diffuse vitreous seeds along with rim of SRF; LE- Circum Corneal Congestion, Corneal edema, full chamber hyphaema, no view of fundus. A diagnosis of RE-Group D and LE Group E intraocular retinoblastoma --ICIOR (International Classification of Intraocular Tumors )classification was made. LE enucleation was carried out using minimally manipulation surgical technique ,long optic nerve stump of 16mm was obtained and a ball implant was placed. Histopathological examination(HPE) confirmed diagnosis of RB and cut end of the optic nerve was free from tumor infiltration.In an attempt to salvage the only remaining eye and possibly vision the patient was referred to the oncologist for chemotherapy. Systemic chemotherapy with three drugs vincristine, etoposide and carboplatin was started alongwith subtenons carboplatin two injections at 3rd and 9th week of chemocycle in RE were administered. Injections were given a day prior to systemic chemo session. In each sitting 20mg (2ml) of carboplatin was given under aseptic precautions .
At 6 weeks post –chemo RE tumor started showing regression ,at 12weeks post chemo RE tumor size reduced to from 11.2 mm base to 5.08mm and height from 5.09mm to 1.65mm with complete regression of vitreous seeding and resorption of SRF. LE socket was healthy . Follow-up after 3 weeks and focal consolidation planned. Local delivery of drugs is associated with increased efficacy and decreased systemic risk. Subconjuctival carboplatin injections are now an accepted treatment for vitreous seeds. Various studies have shown the efficacy of periocular (subtenons) carboplatin which when injected deep posterior substenons space penetrates through the sclera and achieves effective concentrations in the vitreous cavity. Earlier studies have shown that periocular chemotherapy achieves as high as 70% eye salvage in patients with RB with diffuse vitreous seeds. No toxic effect on retinal function has been reported but optic neuropathy, aseptic orbital cellulitis and fibrosis of orbital soft tissues has been reported. The orbital cellulitis is self limiting.
Retinoblastoma management has seen a paradigm shift towards chemo reduction along with serially aggressive local therapy (SALT) and /or focal consolidation especially in advanced stages of Bilateral Retinoblastoma at presentation (common in developing countries) Subtenons carboplatin is adjunct to systemic chemoreduction for treating vitreous seeds, SRF and localized detachments and salvaging eyes. Multimodality treatment as systemic and local chemotherapy is essential to treat advanced RB. But further studies with larger number of patients are needed to prove its efficacy.