Intravitreal bevacizumab for choroidal neovascular membrane associated with Best's vitelliform dystrophy.

Best's vitelliform macular dystrophy is a hereditary form of progressive macular dystrophy that can be complicated by choroidal neovascularization. Authors report successful treatment of choroidal neovascularization with intravitreal bevacizumab in one such eye in an 'adult' Indian male with visual improvement. A 23-year-old male presented with diminution of vision in the right eye for the past sixteen months. Visual acuity was 20/400 in the that eye. After three consecutive intravitreal injections of bevacizumab (1.25 mg/0.05 ml), vision improved to 20/120. Seven months following the last injection of bevacizumab, fundus appeared stable and visual acuity was maintained. No drug-related ocular or systemic side effects were encountered. To the best of our knowledge (PubMed search), this is the first report of its kind in an adult Indian patient. Intravitreal bevacizumab appears to be a promising and cost-effective modality of treatment in such eyes with potential for improvement in vision. However, a long-term follow-up is warranted.

Best's disease, also known as Best's vitelliform macular dystrophy, is a hereditary form of progressive macular dystrophy that can be complicated by choroidal neovascularization. [1] Andrade et al. have reported regression of choroidal neovascularization, resolution of exudative manifestations and signifi cant visual improvement following a single treatment session of photodynamic therapy (PDT). [2] However, PDT may not always be an aff ordable treatment option in the developing world. Bevacizumab, an anti-vascular endothelial growth factor (VEGF), is a humanized, monoclonal antibody being commonly used as an 'off -label' drug for the management of choroidal neovascularization due to age related macular degeneration, [3] pathological myopia, [4] idiopathic parafoveal telengiectasia [5] and angioid streaks. [6] Recently, there has been a report of treatment of choroidal neovascularization related to Best's disease in a thirteen-year-old child with a single injection of intravitreal bevacizumab. [7] We hereby report a case of successful treatment of choroidal neovascularization with intravitreal bevacizumab in one such eye in a young adult Indian male associated with visual improvement. To the best of our knowledge (PubMed search), this is the fi rst report of its kind.

Case Report
A 23-year-old male presented with a history of diminution and distortion of vision in the right eye for the past 16 months. His best corrected visual acuity (BCVA) was 20/400; Ͻ N36 in right eye and 20/20; N6 in the left . Biomicroscopic examination of the anterior segment was unremarkable in both eyes. Fundus examination of the right eye revealed a large, hypopigmented, egg yolk-like subfoveal lesion with fresh subretinal hemorrhage, subretinal fl uid (SRF) and internal limiting membrane (ILM) striae [ Fig. 1a]. These clinical fi ndings were suggestive of choroidal neovascular membrane (CNVM) in the right eye. Left eye fundus examination also revealed a hypopigmented, egg yolk-like lesion at the fovea, characteristic of the vitelliform stage of Best's disease [ Fig. 1b]. Fundus fl uorescein angiography (FFA) revealed early hyperfluorescence with intense late leakage confi rmatory of CNVM in the right eye along with hypofl uorescence corresponding to the subretinal hemorrhage [ Fig. 1c]. Electro-oculogram (EOG) of both eyes revealed sub-normal responses; however recordings were unreliable due to lack of sustained fixation during the test. Optical coherence tomography (OCT) of the right eye confi rmed the presence of SRF, cystoid macular edema, disorganization of the retinal pigment epithelium (RPE)-choriocapillaris complex corresponding to the CNVM, vitelliform changes and subretinal blood [ Fig. 1d]. The treatment options of PDT and anti-VEGF agents were discussed with the patient. The patient opted for intravitreal bevacizumab over PDT and the same was administered (1.25 mg/0.05 ml) under strict aseptic precautions, aft er obtaining an informed (writt en) consent. Post injection period was uneventful.
At fi ve weeks follow up, BCVA in the right eye was still 20/400, N36 and the left eye was stable. Fundus examination of the right eye revealed regression of CNVM with marked resolution of subretinal hemorrhage and reduction of SRF at fovea [ Fig. 2a]. FFA revealed reduced leakage from CNVM [ Fig. 2b]. OCT revealed markedly reduced SRF and increased fibrosis of CNVM as compared to the previous visit [ Fig. 2c]. A second intravitreal injection of bevacizumab (1.25 mg/0.05 ml) was given at six weeks following the fi rst one. Post injection period was uneventful. Four weeks later, BCVA in the right eye was still maintained. Fundus examination revealed a scarred CNVM with ILM folds, resolution of subretinal fl uid and subretinal hemorrhage. FFA showed reduced yet persisting leakage from the CNVM in the right eye. Clinical fi ndings were confi rmed on OCT. Hence, a third injection of intravitreal bevacizumab was given. Post injection period was uneventful. The patient reported at eight weeks following the third injection. Right eye BCVA had improved to 20/120, N12. Anterior segment examination was normal in both eyes. Fundus examination of the right eye showed a scarred CNVM with ILM striae [Fig. 3a]. There was no evidence of SRF and subretinal hemorrhage had completely resolved. Clinical fi ndings were confi rmed on FFA [ Fig. 3b] and OCT [ Fig. 3c]. The eye remained stable at 12 weeks follow-up [ Fig. 4] and subsequently, at the last follow-up, seven months aft er the third injection of bevacizumab. During the entire course of treatment, we did not encounter any drug-related ocular or systemic side eff ects.

Discussion
Our report corroborates the results of similar treatment as reported by Leu et al. [7] in a 13-year-old boy, albeit in an adult Indian patient with three injections. We also feel that had the treatment been initiated at a more acute stage, the outcome would have been bett er. Intravitreal bevacizumab appears to be a promising, cost-eff ective modality of treatment with a potential for improvement in visual acuity, although with inherent risks associated with intravitreal injections. A longterm follow-up is warranted to address possible recurrences and determine the optimal number of re-treatments required in achieving a long-term stabilization of the aforesaid condition.

Samrat Chatt erjee, Deepshikha Agrawal
We report a case of fungal keratitis occurring in a patient with latt ice dystrophy. A 57-year-old farmer presented with a corneal ulcer following probable entry of paddy husk in the right eye, of one month duration. Corneal scraping revealed pigmented fungal filaments while culture grew Alternaria alternata. Treatment with 5% natamycin eye drops and 1% atropine healed the infection in four weeks. We would like to draw att ention to the fact that the cornea in latt ice dystrophy is prone to frequent erosions and is a compromised epithelial barrier to invasion by microorganisms. Patients must be made aware of this fact and should seek att ention at the earliest following any trivial trauma. Management of minor corneal abrasions in them should be directed at healing the epithelium with adequate lubricants and preventing infection with topical antibiotic prophylaxis. Latt ice dystrophy of the cornea is a bilateral, inherited, primary, localized corneal amyloidosis characterized by subepithelial opacities, stromal white dots, refractile fi lamentary lines and stromal haze giving rise to recurrent corneal erosions and irregularity of the epithelium with accompanying decrease in visual acuity. [1] Recurrent erosion and an unhealthy epithelium in latt ice dystrophy may predispose the cornea to microbial infections. [2] Few such cases have been reported in the literature with bacterial and viral infections. [2][3][4] We report an uncommon case of fungal keratitis in a patient with latt ice dystrophy.

Case Report
A 57-year-old farmer presented with complaints of pain, redness, watering and reduced vision in the right eye, of onemonth duration. There was a vague history of entry of paddy husk in the eye. He also gave a history of repeated episodes of foreign body sensation, pain and watering in both the eyes since fi ve years. His visual acuity in the right eye was counting fi ngers at 2 m while in the left eye was 20/20. There was nasal pterygium in both eyes. There were no signs of blepharitis or meibomitis. There was a para-central epithelial defect with dry, white, full-thickness stromal infi ltrate of 3 ϫ 2.5 mm diameter [ Fig. 1a]. There were multiple linear refractile lines consistent with latt ice-lines in the corneal stroma extending to the periphery [ Fig. 1b]. There was hypopyon, the pupil was