Multimodal imaging and functional correlations identify unusual cases of macular retinal pigment epithelium hypopigmentation occurring without functional loss.

PURPOSE: Patients with unusual macular retinal pigment epithelium (RPE) hypopigmentation are described and analyzed using retinal multimodal imaging. METHODS: We report three cases of patients with unilateral (2) or bilateral (1) macular lesions discovered incidentally on fundoscopy. A comprehensive ophthalmic examination including visual acuity, fundoscopy, spectral-domain optical coherence tomography (SD-OCT), short-wavelength light and near-infrared autofluorescence, fluorescein angiography, microperimetry, multifocal electroretinogram, adaptive optics (AO), and OCT-angiography (OCT-A) has been performed. RESULTS: Visual acuity was 20/20 in both eyes of all patients. The lesion appeared hyperautofluorescent on short-wavelength light and hypoautofluorescent on near-infrared light. Fluorescein angiography revealed a sharply demarcated macular hyperfluorescence without any leakage, suggesting a window defect. Interestingly, SD-OCT revealed only a choroidal hyperreflectivity in relation to the lesions without any abnormality of the outer retinal layers. Microperimetry was normal except for 1 patient with bilateral lesion and subtle decrease in macular sensitivity. Mf ERG was normal in all three patients. AO showed a well-preserved cone mosaic, suggesting that the abnormality was localized under the photoreceptor layers. OCT-A revealed hyperreflectivity just below the RPE layer, corresponding to the macular lesion observed on fundoscopy and the choroidal hyperreflectivity seen on SD-OCT. CONCLUSIONS: Macular RPE hypopigmentation should be considered in case of an isolated macular lesion without functional visual impairment or anatomical defect on SD-OCT.

[Ranibizumab and exudative age-related macular degeneration: 5-year multicentric functional and anatomical results in real-life practice]. / Ranibizumab et dégénérescence maculaire liée à l'âge exsudative : analyse multicentrique à 5ans des résultats fonctionnels et anatomiques en pratique clinique réelle.

PURPOSE: The goal of this study was to evaluate five year functional and anatomical outcomes of wet AMD patients treated with ranibizumab according to a pro re nata (PRN) regimen in real-life practice. METHODS: A retrospective, multicentric chart review of 201 eyes of 201 patients who underwent their first ranibizumab intravitreal injection (IVT) between January 1, 2007 and December 31, 2008 was performed. Best-corrected visual acuity (BCVA), central macular thickness (CMT) on SD-OCT, number of IVT and follow-up visits were collected at baseline and during the entire follow-up period of 5 years. RESULTS: Mean BCVA at baseline was 52.3±16.5 letters. Mean BCVA change from baseline was respectively +2.8, +2.5, +1.8, -0.6 at 1, 2, 3, 4 years of follow-up. At year 5, 43% of eyes had a stable or improved letter score (≥0 letter gain), whereas 29% declined by 15 letters or more, with an overall significant mean decline of 2.8 letters (P<0.05). No correlation was observed between final visual outcome and age, baseline BCVA, type of neovascularization, naive status, number of IVT or number of follow-up visits. On SD-OCT, mean CMT was 293±96µm at baseline and was significantly reduced compared to baseline at each year end-point (P<0.005). The mean number of IVT was 15±10.4 at year 5, with 55% of eyes still being under active treatment. CONCLUSION: PRN ranibizumab in real-life practice improved or stabilized visual acuity over 4 years. During the 5th year, progressive decline of visual acuity was observed.

Ranibizumab for exudative age-related macular degeneration: A five year study of adherence to follow-up in a real-life setting.

PURPOSE: To analyze adherence to follow-up over 5 years in patients treated with intravitreal ranibizumab for exudative age-related macular degeneration (AMD) in a tertiary health care center. To investigate factors associated with failure to continue follow-up. METHODS: Retrospective chart review of all consecutive patients with exudative AMD, who received their first intravitreal ranibizumab injection at the Créteil Intercommunal University Hospital Eye Clinic between October 1, 2006 and March 31, 2007. Patient clinical characteristics at baseline and at the last follow-up visit were recorded. Distance from home to hospital was measured for each patient. A multiple-choice telephone survey was conducted for patients lost to follow-up to determine the main reasons for failure to continue follow-up. RESULTS: Two hundred and one patients were included. The rate of loss to follow-up over the 5-year period was 57% (115/201). Fifty-eight patients lost to follow-up completed the questionnaire. The main reasons reported by patients for follow-up discontinuation were long distance from home to hospital (51.7%, 30/58), subjective dissatisfaction with the benefits of intravitreal injections (34.5%, 20/58), and the excessive burden of periodic follow-up visits (24.1%, 14/58). Three factors were significantly associated with follow-up discontinuation: high age at baseline (82.2 vs. 76.5 years, P<0.001), poor best-corrected visual acuity (BCVA) at baseline (42.5 vs. 51.0 letters, P=0.020), and long distance from home to hospital (132 vs. 17.1km, P<0.001). CONCLUSION: In this study, adherence to follow-up over 5 years was poor. Age and BCVA at baseline and distance from home to hospital were independently associated with long-term adherence.

[Bitemporal hemianopia as presenting sign of severe ethambutol toxicity]. / Hémianopsie bitemporale révélatrice d'une atteinte toxique sévère à l'éthambutol.

INTRODUCTION: Optic neuropathy is a severe and well-known complication of ethambutol treatment. If not detected early, it may lead to profound and irreversible vision loss. CASE REPORT: We report the case of a 83-year-old female patient, referred for rapidly progressive, painless, bilateral visual loss, unimproved after bilateral cataract surgery. Automated Humphrey 24-2 visual field demonstrated bitemporal hemianopia associated with bilateral central scotoma. Brain MRI did not demonstrate any compressive lesion in the chiasmal region. However, on T2-weighted sequences, an area of elevated signal intensity appeared within the optic chiasm, enhancing after gadolinium injection. On detailed history, it was noted that the patient had been on ethambutol for the last 18months, for the treatment of a Mycobacterium avium-related pneumonitis. DISCUSSION: The incidence of ethambutol-related toxic optic neuropathy has dramatically decreased since the recommendations for regular follow-up of patients treated with ethambutol. This treatment is classically responsible for bilateral central or ceco-central scotoma due to optic neuropathy, although a few cases of bitemporal hemianopia have been reported in the literature, mimicking a compressive chiasmal lesion. However, none of these cases demonstrated a hypersignal in the optic chiasm on brain magnetic resonance imaging (MRI). CONCLUSION: Bitemporal hemianopia on visual field testing is very suggestive of a chiasmal lesion, which is generally due to a compressive, or more rarely inflammatory, lesion in the sellar region. Toxic chiasmal lesions are rare, but in the absence of any tumoral lesion in the sellar area, a detailed history must be obtained in order to rule out drug toxicity, so as to prevent irreversible visual loss.