Pediatric anophthalmic sockets and orbital implants: outcomes with polymer-coated implants.

PURPOSE: To compare wrapped and polymer-coated hydroxyapatite implants in children undergoing primary enucleation with no adjuvant therapies. DESIGN: Retrospective, interventional cohort study. PARTICIPANTS: All children undergoing primary enucleation without adjuvant therapies between 1999 and 2009 at a tertiary pediatric cancer hospital. METHODS: Review and analysis of patient records. MAIN OUTCOME MEASURES: Implant exposure, extrusion and migration, socket contracture, and formation of pyogenic granuloma. RESULTS: Sixty consecutive patients undergoing primary enucleation with no adjuvant chemotherapy or radiation with follow-up of at least 12 months were included. Retinoblastoma was the diagnosis in 59 eyes (98.3%). Median follow-up was 3.6 years (range, 1.0-9.3 years). Two implant sizes were used: 20 mm in 47 patients (78.3%) and 18 mm in 13 patients (21.7%). Overall, 52 patients (86.7%) had an event-free recovery. Polymer-coated hydroxyapatite implants (43/60, 71.7%), when compared with wrapped ones (17/60, 28.3%), had a trend toward greater event-free recovery (odds ratio [OR], 1.6; 95% confidence interval [CI], 0.3-7.7) and lower exposure rate (OR, 2.1; 95% CI, 0.4-10.5). CONCLUSIONS: The use of polymer-coated hydroxyapatite implants is associated with favorable outcomes in the pediatric population. Despite observed complications, long-term implant retention is possible in most children. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Treatment of retinoblastoma: current status and future perspectives.

Treatment of retinoblastoma must be individualized. Most patients with unilateral, non-metastatic retinoblastoma can be cured with enucleation alone. In patients with histologic risk factors, adjuvant chemotherapy is recommended, with the addition of orbital radiation for patients with trans-scleral involvement or tumor present at the level of the cut end of the optic nerve. Patients with metastases require intensive chemotherapy and consolidation with autologous hematopoietic stem cell rescue. Patients with bilateral or multifocal disease represent a major challenge. Cure of the disease is the first priority, but the therapeutic approach also has to consider eye and vision preservation. The approach is conservative, and only eyes with very advanced disease are enucleated upfront. Patients are treated with chemotherapy and intensive focal treatments, with the aim of delaying or avoiding radiation therapy and enucleation. For patients with early intraocular stage (Reese-Ellsworth groups I-III and International Groups A-B), the two-drug combination of vincristine and carboplatin is recommended. Patients with more advanced intraocular disease (Reese-Ellsworth groups IV-V and International Groups C-D) require more intensive chemotherapy. Standard of care for these patients incorporates etoposide into the regimen. Effective agents with good intraocular penetration, such as topotecan, are being investigated. Because most failures are secondary to progression of the vitreous seeds, subconjunctival carboplatin is added in cases with poor response of the vitreous tumors. Patients must be monitored very closely, with examinations under anesthesia every 4 to 6 weeks, and focal treatments are applied during the procedure. These include cryotherapy for small anterior tumors, thermotherapy and laser photocoagulation for small posterior tumors, and brachytherapy for larger tumors. New treatment approaches under development include the refinement of periocular chemotherapy administration using slow-release devices, the use of suicide gene therapy with local delivery of the herpes simplex thyrosine kinase gene (followed by systemic administration of ganciclovir), and the development of small-molecule inhibitors of the MDMX-p53 interaction.

A rapidly growing choroidal melanoma.

PURPOSE: We describe a rapidly growing choroidal melanoma that failed to respond to consecutive globe-sparing treatments in an individual with ocular melanocytosis. DESIGN: Interventional case report. METHODS: A 53-year-old man with right ocular melanocytosis, decreased vision in the right eye, and right eye pain was referred for suspected choroidal melanoma. The lesion, which measured 2.4 mm x 6.6 mm x 6.0 mm, was treated with transpupillary thermotherapy on two occasions and with iodine-125 (I-125) plaque brachytherapy, both of which failed. Enucleation allowed histologic analysis. RESULTS: At enucleation, the choroidal melanoma in the right eye measured 13.6 mm x 16.5 mm x 16.9 mm, which demonstrated the most rapid growth and doubling time reported in a tumor of this type thus far. Pathologic analysis classified the tumor as a mixed-cell type. CONCLUSION: This case reiterates the association between ocular melanocytosis and choroidal melanoma, provides additional evidence that rapid growth and doubling time may be associated with transpupillary thermotherapy and I-125 plaque failure, and highlights the need to follow those tumors closely.