Restoration of Foveal Anatomy and Function Following Chemoreduction for Bilateral Advanced Retinoblastoma With Total Retinal Detachment

It is common for children with retinoblastoma to have extensive subretinal fluid, often involving the fovea and compromising visual acuity.¹ Following chemoreduction, retinal detachment generally resolves over several months. In 1 study of eyes with total retinal detachment from retinoblastoma, complete retinal reattachment was found in 76% of cases using chemoreduction.² However, the microarchitecture of the attached retina has not yet been evaluated. In this case, we report the long-term foveal microarchitectural findings using optical coherence tomography (OCT) in a child with advanced retinoblastoma and total retinal detachment in both eyes.

In 1998, an 11-month-old girl was noted by her parents to have poor visual acuity. Ocular examination disclosed visual acuity of no fix or follow in each eye. Bilateral leukocoria with total retinal detachment behind the clear lens was found (Figure 1). The retinal detachment was 12 mm in elevation by ultrasonography in each eye. Bilateral exophytic extramacular retinoblastoma was confirmed, measuring ultrasonographically 18 mm in base and 12 mm in thickness in the right eye and 15 mm in base and 13 mm in thickness in the left eye (Figure 1). Chemoreduction was used with vincristine sulfate, etoposide, and carboplatin for 6 cycles under protocol, followed by thermotherapy consolidation of tumors and thermotherapy and cryotherapy consolidation of active subretinal seeds.³ The tumors and seeds were completely controlled. The retinal detachment completely reattached over 2 months. The last portion of retina to reattach was the fovea in each eye.

In 2004, the visual acuity was 20/50 OD and 20/70 OS. Complete tumor control was achieved (Figure 2). Despite the previous prolonged retinal detachment, OCT disclosed normal foveal microarchitecture without subretinal fluid or intraretinal edema. By OCT, the right foveal depression was blunted but there was no evident preretinal membrane. The left foveal depression appeared normal (Figure 2).

Current management of retinoblastoma includes enucleation, chemoreduction plus focal consolidation, external beam radiotherapy, and focal therapies.¹ Children with bilateral retinoblastoma are most often treated with chemoreduction followed by thermotherapy or cryotherapy consolidation of each tumor. Overall, long-term tumor control following chemoreduction is satisfactory, but eyes with extensive subretinal fluid and vitreous or subretinal seeds fare poorest.³ New information on visual results in successfully treated patients reveals that approximately 50% of children ultimately manifest 20/40 visual acuity or better.⁴ These visual results are more favorable than those previous to chemoreduction. Those at risk for poor vision display macular tumor involvement or macular subretinal fluid.

In this case, 6-year follow-up of a child with previous extensive and prolonged total retinal detachment showed remarkable restoration of the retinal anatomy and function. Despite the fact that the fovea was detached for at least 2 months and likely much longer, this patient regained impressive visual acuity and maintained foveal microarchitecture with a relatively normal appearance on OCT.⁵ These findings provide encouragement that the pediatric retina might, in some cases, tolerate prolonged retinal detachment. This further underscores the importance of conservative treatment for retinoblastoma.

Correspondence: Dr C. Shields, Ocular Oncology Service, Wills Eye Hospital, 840 Walnut St, Philadelphia, PA 19107 (carol.shields@shieldsoncology.com).

Financial Disclosure: None.

Funding/Support: This study was supported by the Eye Tumor Research Foundation, Philadelphia, Pa (Dr C. Shields); the Macula Foundation, New York, NY (Dr C. Shields); the Rosenthal Award of the Macula Society, Barcelona, Spain (Dr C. Shields); and the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J. Shields).