Name

Restauration de la microstructure fovéolaire et résultats fonctionnels dans une cohorte de 90 patients (104 yeux) atteints de rétinoblastome fovéolaire après traitement par chimiothérapie intraveineuse ou intra-artérielle

Foveolar-involvement of retinoblasotma tumors has been associated with poor visual outcome. Frequency of recovery of foveal microanatomy in eyes with foveolar-involving retinoblastoma has not yet been investigated. The purpose of this study was to determine the frequency for recovery of foveal microanatomy by optical coherence tomography (OCT) after intravenous chemotherapy (IVC) or intraarterial chemotherapy (IAC) for eyes with foveolar-involving retinoblastoma and to provide a correlation of the clinical features at presentation with visual outcome.

Retrospective comparative case series including 104 eyes (90 patients) with foveolar-involving retinoblastoma and OCT determination of foveal microanatomy after completion of treatment. All patients received chemotherapy and no eye received foveolar tumor consolidation with laser photocoagulation, thermotherapy, cryotherapy or plaque radiotherapy. A comparison of clinical features  between those with foveal recovery (FR) and those without (No FR) on OCT was performed. Snellen visual acuity was assessed.

Overall, 19 (18%) eyes had FR on OCT: 2 (2/19, 2%) with complete foveal restoration and normal microanatomy, 10 (10/19, 10%) with complete foveal restoration but abnormal microanatomy, and 7 (7/19, 6%) with partial recovery (hemifovea) only. A comparison of clinical features (FR vs No FR) revealed significant differences in that FR demonstrated greater mean age at presentation (13 months vs 7 months, p=0.003), less advanced eyes (group B: 6/19, 32% vs 6/85, 7%; p=0.008), and smaller mean tumor diameter (12.7mm vs 15.5mm, p=0.026). There was no differences regarding vitreous or subretinal seeds, extent of retinal detachment, and choice of primary therapy (IVC or IAC). Sub-analysis of visual outcome in 74 eyes (FR=14, No FR=60) revealed eyes with FR showing better mean visual acuity (20/160 vs 20/400, p=0.006), greater number with visual acuity ≥20/40 (3/14, 21% vs 0/60, 0%, p=0.006), and lesser number with visual acuity <20/200 (4/14, 29% vs 41/60, 68%, p=0.013). However, despite No FR, 32% (19/60) achieved visual acuity ≥20/200.

Visual prognosis in eyes with foveolar-involvement of retinoblastoma has generally been regarded as poor, but published data are limited. We were able to demonstrate that complete resolution of the tumor from the foveola was possible after IVC or IAC, allowing,in some cases, a complete microstructure recovery of the foveola and with return of central visual acuity. However, the absence of foveal recovery on OCT was not necessarily associated with a poor visual outcome, and functional vision was achieved in some cases. Factors that affect vision in eyes with chemotherapy-treated retinoblastoma include foveolar scar from previous tumor, previous subretinal fluid, complications of chemotherapy, and amblyopia.

Of 104 eyes with foveolar-involving retinoblastoma treated with chemotherapy, 18% demonstrated OCT-evidence of foveal microanatomic restoration, correlating significantly with better visual outcome. OCT can be useful for estimation of visual potential in preverbal children following chemotherapy for retinoblastoma