Intravitreal injection of ranibizumab for foveal-macular pattern dystrophy: case report.

In the recent years, anti-angiogenic medications have successfully treated other diseases associated with choroidal neovascularization. The anti-angiogenic therapy alone or combined with LASER and/or steroids has been effective in controlling ocular neovascularization, not only restricted to the treatment of typical membranes due to macular degeneration in the wet form. The discovery and subsequent use of these drugs has revolutionized medicine and ophthalmology. This report illustrates an example of successful treatment in a challenging pathology where it was found important visual and anatomical response after the use of ranibizumab.

Intravitreal injection of ranibizumab for foveal-macular pattern dystrophy: case report / Injeção intravítrea de ranibizumabe para distrofia foveal-macular em padrão: relato de caso

In the recent years, anti-angiogenic medications have successfully treated other diseases associated with choroidal neovascularization. The anti-angiogenic therapy alone or combined with LASER and/or steroids has been effective in controlling ocular neovascularization, not only restricted to the treatment of typical membranes due to macular degeneration in the wet form. The discovery and subsequent use of these drugs has revolutionized medicine and ophthalmology. This report illustrates an example of successful treatment in a challenging pathology where it was found important visual and anatomical response after the use of ranibizumab.

Nos últimos anos, os medicamentos antiangiogênicos têm tratado com sucesso outras doenças relacionadas com a neovascularização da coroide. A terapia antiangiogênica isoladamente ou combinada com LASER e/ou esteroides têm se mostrado eficaz no controle da neovascularização ocular, não se restringindo apenas ao tratamento das membranas típicas da degeneração macular na forma úmida. A descoberta e o posterior uso destas drogas vêm revolucionando a medicina e a oftalmologia. Este relato ilustra um exemplo de tratamento de sucesso numa patologia desafiadora onde se obteve importante resposta visual e anatômica após uso do ranibizumabe.

Vision First, a program to detect and treat eye diseases in young children: the first four years.

PURPOSE: The Vision First program began in the fall of 2002 as a community outreach initiative by the Cleveland Clinic Cole Eye Institute in partnership with the Cleveland Metropolitan School District. It was designed to provide free eye examinations to all prekindergarten, kindergarten, and first grade students enrolled in Cleveland City public schools in order to diagnose refractive errors, amblyopia, and strabismus, so that treatment is instituted and the best possible visual outcome attained. METHODS: Examinations are performed in 2 lanes of a specially outfitted recreational vehicle. All children undergo monocular visual acuity testing at distance and near, stereopsis testing, cover testing at distance and near, testing of versions, and external ocular inspection. If a child fails any part of this examination according to the guidelines set by the American Academy of Pediatrics, cycloplegic drops are instilled and an optometrist refracts the child on location and performs indirect ophthalmoscopy. Glasses are prescribed and follow-up with a pediatric ophthamologist is arranged. RESULTS: During the first 4 years of the program, 22,988 examinations were performed. Seven percent of children had errors of refraction that necessitated optical correction, about 2.1% had strabismus, and 1.7% had amblyopia. The cost per student per year was around $23 excluding glasses. CONCLUSIONS: About 10% of 5- and 6-year-old schoolchildren have eye problems that require either glasses or treatment for strabismus or amblyopia. The Vision First model brings eye care professionals to the schools and provides effective comprehensive screening of all children, as well as detailed examination and referral of those with significant eye problems.

Reopening of previously closed macular holes after cataract extraction.

PURPOSE: To evaluate the frequency of reopening of macular holes after cataract extraction. DESIGN: Retrospective, comparative, consecutive case series. METHODS: Two hundred and eleven eyes with idiopathic macular holes closed by vitrectomy were divided into four groups: Group 1: prior cataract extraction; Group 2: vitrectomy then cataract extraction; Group 3: vitrectomy only; and Group 4: vitrectomy and cataract extraction as a combined procedure. The main outcome measure of macular hole reopening was evaluated in relationship to multiple variables. RESULTS: Two hundred and eleven eyes were included: Group 1: 56 eyes; Group 2: 86 eyes; Group 3: 41 eyes; and Group 4: 28 eyes. Twenty-four macular holes reopened (11%) (mean follow-up 26.6 months, range, three to 118 months). The greatest number of macular hole reopenings, 17 (20%), were in Group 2. Cox multivariate analysis failed to demonstrate an association between duration of hole, serum use, internal limiting membrane peeling, or stage and reopening of a macular hole. Cox analysis showed a four-fold increased risk of reopening in Group 2 eyes (95% confidence interval [CI]: 1.7 to 11.2; P = .002). Eyes with cystoid macular edema after cataract extraction had a seven-fold increased risk of macular hole reopening (7.72; 95% CI: 2.79 to 21.3; P < .0005). Kaplan-Meier analysis showed increased rates of macular hole reopening in Group 2 eyes compared to the other 3 groups combined (log-rank P < .00005). CONCLUSIONS: Cataract extraction after successful vitrectomy for macular hole, when complicated by cystoid macular edema (CME), may increase the risk of macular hole reopening.

Management of macular hole and submacular hemorrhage in the same eye.

BACKGROUND: To report the management of patients with a macular hole and submacular hemorrhage in the same eye. METHODS: Case reports of two eyes of two patients undergoing pars plana vitrectomy (PPV), subretinal injection of tissue plasminogen activator (t-PA) and air-fluid exchange to displace a submacular hemorrhage. In one eye with a submacular hemorrhage due to age-related macular degeneration, a macular hole formed during subretinal t-PA injection. In another patient with a submacular hemorrhage due to a ruptured retinal arterial macroaneurysm (RAM), a sub-internal limiting membrane (ILM) hemorrhage was noted, and a macular hole was found after peeling the ILM, overlying the subretinal hemorrhage. RESULTS: In the first case, after 45 min was allowed for the subretinal clot to liquefy, the macular hole was noted to be closed. A partial air-fluid exchange was performed and the patient was positioned upright, to displace the submacular hemorrhage and tamponade the macular hole. Two weeks later, visual acuity had improved from 20/400 with eccentric viewing to 20/100, the macular hole was closed by optical coherence tomography, and the patient subsequently underwent two sessions of verteporfin photodynamic therapy (PDT) to treat choroidal neovascularization detected by fluorescein angiography. At last follow-up 7 months after surgery, vision was 20/200, the CNV was active angiographically, and another session of PDT was performed. In the second case, PPV was combined with phacoemulsification and intraocular lens implantation. An 80% air-fluid exchange was performed after injecting the subretinal t-PA, the air was exchanged for 14% perfluoropropane gas, and the patient was positioned upright. Visual acuity improved from 20/400 to 20/200 at last follow-up 4 months after surgery, with the RAM spontaneously sclerosed and the macular hole closed clinically and angiographically. CONCLUSIONS: Intraoperative evacuation of subretinal hemorrhage is not necessary in cases with coexisting macular hole and submacular hemorrhage. The submacular hemorrhage can be displaced using air or gas, and the bubble can be used to tamponade the macular hole.

Nonarteritic anterior ischemic optic neuropathy and 'visual field defects' following vitrectomy: could they be related?

BACKGROUND: Visual field defects after uncomplicated vitrectomy have been reported but poorly explained. We describe two cases of nonarteritic anterior ischemic optic neuropathy (NAION) observed following vitrectomy. We also reviewed the literature for cases of post-vitrectomy visual field defects for evidence of optic nerve damage. METHODS: Two patients developed optic disc edema and features of an optic neuropathy after uncomplicated vitrectomy for macular hole and epiretinal membrane. A systematic literature search was conducted to obtain prior reports of visual field defects or ischemic optic neuropathy following vitrectomy. Additional studies were identified from the bibliographies of the retrieved articles. RESULTS: The incidence of visual field defects following vitrectomy has varied from 1-71% across all studies. Overall, we found 160 (14.5%) cases of unexplained visual field defects following vitrectomy out of 1,104 patients. Of these, 31 eyes (19.4%) have shown some sign of optic nerve damage following vitrectomy, including pallor in 29 eyes, relative afferent pupillary defect in eight eyes, and intrapapillary hemorrhage in two eyes. CONCLUSIONS: NAION may develop following vitrectomy. "Visual field defects" following vitrectomy are common and many of the involved eyes demonstrate evidence of optic nerve damage, some of which may have represented NAION.

Bilateral macular lesions in a 10-year-old girl.

PURPOSE: To report a previously undescribed macular dystrophy. DESIGN: Single descriptive case report. METHODS: Presentation of clinical findings, including ophthalmoscopy, fluorescein angiography, full-field and multifocal electroretinography (ERG), optical coherence tomography (OCT), and visual fields. RESULTS: A 10 year-old Caucasian girl had peculiar reddish blotchy macular lesions bilaterally, associated with decreased visual acuity, and no family history of similar lesions. Fluorescein angiography was inconclusive and showed mottled hyperfluorescence in the macula. The full-field electroretinogram was normal, but multifocal ERG revealed diminished waveforms centrally. CONCLUSION: This is possibly a new macular dystrophy, the nature of which remains to be determined.

Mutation screen of the cone-specific gene, CLUL1, in 376 patients with age-related macular degeneration.

Clusterin is a secreted glycoprotein expressed ubiquitously in many tissues that appears to function as a molecular chaperone capable of protecting stressed proteins. It is upregulated in many different forms of neurodegeneration and is thought to represent a defense response against neuronal damage. Clusterin has been found to be a common protein identified in drusen preparations isolated from the retina of donor eyes of patients with age-related macular degeneration (AMD), the leading cause of blindness in the elderly population of developed countries. A retina-specific clusterin-like protein (CLUL1) showing nearly 25% identity to clusterin at the protein level was recently cloned and shown to be expressed specifically in cone photoreceptor cells. For these reasons, we investigated CLUL1 as a candidate gene for AMD. A mutation screen of the entire coding region of the CLUL1 gene in 376 unrelated patients with AMD uncovered three sequence variations, one isocoding change and two intronic changes. One intronic change appears significantly less frequent in patients with the more severe forms of AMD than in control subjects, suggesting that this variant may reduce the risk for AMD or may be linked to a nearby variant that may reduce AMD risk. Variant alleles of the CLUL1 gene were found; however, none are considered pathogenic. None of the variants identified are predicted to create or destroy splice donor or acceptor sites based on splice-site prediction software.

Innovations in eye surgery.

The field of ophthalmology has undergone revolutionary changes during the past few decades. Advancements in understanding the pathophysiology of eye diseases, superior surgical instrumentation and surgeon skills, and cotreatment with medical therapies have enhanced outcomes. The geriatric population, preferentially affected by these illnesses, has seen a meaningful visual benefit from these surgical innovations. Most importantly, these improvements have led to increases in quality-of-life measures and mental and physical well-being of aging patients.

Primary lymphoma of the central nervous system.

This article discusses primary lymphoma of the central nervous system, which is a variant of extranodal non-Hodgkin's lymphoma that arises from specific sites such as the brain, spinal cord, meninges, or eyes.

Surgery for hemorrhagic choroidal neovascular lesions of age-related macular degeneration: ophthalmic findings: SST report no. 13.

PURPOSE: To present best-corrected visual acuity (BCVA) findings and other clinical outcomes from eyes of patients enrolled in one of the Submacular Surgery Trials (SST) evaluating surgical removal versus observation of predominantly hemorrhagic subfoveal choroidal neovascularization (CNV) associated with age-related macular degeneration. DESIGN: Randomized clinical trial (SST Group B Trial). PARTICIPANTS: Eligible patients had subfoveal choroidal neovascular lesions greater than 3.5 disk areas (8.9 mm2) composed of at least 50% blood (either blood or CNV underlying the center of the foveal avascular zone) and BCVA of 20/100 to light perception in the study eye. INTERVENTION: Patients were assigned randomly at time of enrollment to observation or surgical removal of blood and any associated CNV. MAIN OUTCOME MEASURE: A successful outcome was defined a priori as either improvement in visual acuity (VA), no change in VA, or a decline in VA of no more than 1 line (7 letters) from baseline to the 24-month examination based on an intent-to-treat analysis. RESULTS: Of 336 patients enrolled, 168 were assigned to each treatment arm; treatment arms were balanced by baseline characteristics. Of 1501 expected examinations 3 months through 36 months after baseline, 1370 (91%) were performed. Loss of > or =2 lines (> or =8 letters) of VA occurred in 56% of surgery eyes, versus 59% of observation eyes examined at 24 months. Although severe loss of VA was not the primary outcome of interest, surgery more often prevented such loss: 36% in the observation arm versus 21% in the surgery arm at the 24-month examination (chi2 P = 0.004). Of initially phakic eyes, the cumulative percentage that had undergone cataract surgery by 24 months was 44% in the surgery arm, compared with 6% in the observation arm. Twenty-seven eyes (16%) in the surgical arm, compared with 3 eyes (2%) in the observation arm, had a rhegmatogenous retinal detachment (RD). CONCLUSIONS: Submacular surgery as performed in the SST Group B Trial did not increase the chance of stable or improved VA (the primary outcome of interest) and was associated with a high risk of rhegmatogenous RD, but did reduce the risk of severe VA loss in comparison with observation. This article contains additional online-only material available at http://www.ophsource.com/periodicals/ophtha.

Macular translocation with chorioscleral outfolding: 2-year results.

PURPOSE: To evaluate the results of macular translocation with radial chorioscleral outfolding in patients with subfoveal choroidal neovascularization (CNV) and to identify factors associated with decreased postoperative visual acuity. DESIGN: Retrospective interventional case series. METHODS: Twenty-seven consecutive patients with surgically treated subfoveal CNV were operated on and followed for more than 2 years. Macular translocation with radial chorioscleral outfolding was performed using titanium clips. Surgical outcomes including visual acuity, foveal displacement, surgically induced astigmatism, and complications were recorded. Factors associated with decreased visual acuity postoperatively were identified by multiple logistic regression analysis. RESULTS: Foveal displacement ranged from 349 to 3391 (median, 1576) microm. Surgically induced astigmatism ranged from 0 to 3.0 (median, 0.5) diopters. Visual acuity after surgery improved in 19 of the 27 patients (70.4%; median, 5 lines), but in 14, final vision ultimately decreased from the best postoperative level. Final visual acuity improved from the preoperative level in 11 patients (40.7%), remained unchanged in seven (25.9%), and decreased in nine (33.3%). In our series, the mean preoperative vision was 20/174, the best postoperative visual acuity was 20/74, and the mean final vision was 20/167. Factors associated with a decrease in postoperative visual acuity included the postoperative enlargement of CNV. CONCLUSION: Macular translocation with radial chorioscleral outfolding improved or stabilized the vision in 66.7% of patients with subfoveal CNV over the course of more than 2 years. Closing the CNV or preventing its further growth is required to maintain a better postoperative visual acuity.

A novel RDS/peripherin gene mutation associated with diverse macular phenotypes.

Pattern dystrophy is a heterogeneous group of retinal dystrophies of which butterfly-shaped pattern dystrophy (BPD) and adult-onset foveomacular dystrophy (AOFMD) are the two most common forms. BPD is characterized by a butterfly-shaped, irregular, depigmented lesion at the level of the retinal pigment epithelium. In contrast, AOFMD is characterized by the presence of slightly elevated, symmetric, solitary, round to oval, yellow lesions at the level of the retinal pigment epithelium. We identified three independent kindreds with pattern dystrophy, one with four patients affected with BPD and the other two with 14 affected patients with AOFMD. We performed complete ophthalmic examination, fluorescein angiography, linkage mapping, and mutational screening in the RDS/peripherin gene in the affected patients. Patients affected with BPD had a best-corrected vision of 20/20 to 20/25, whereas vision in the eyes of patients with AOFMD ranged from 20/20 to 20/400. In all three kindreds, sequence analysis identified an A-to-G change at nucleotide position 422 of the RDS/peripherin gene, predicting a novel Tyr-141-Cys substitution. A haplotype analysis revealed that these three kindreds shared an identical disease haplotype at the RDS/peripherin locus, indicating that the mutation reflects a founder effect. The sequence change that segregated with the disease phenotype was not observed in 200 control chromosomes. Our results identified a novel mutation in the RDS/ peripherin gene that can cause diverse macular phenotypes. Genetic and clinical investigation of pattern dystrophy may provide useful diagnostic tools and new treatment strategies for this disorder.

Removal of retained subfoveal perfluoro-n-octane liquid.

PURPOSE: To report a novel technique to remove retained submacular perfluorocarbon liquid. DESIGN: Retrospective cases series. METHODS: Two patients with retained subfoveal perfluorocarbon liquid were treated with this technique. With a three-port pars plana approach, a 39-gauge flexible cannula was used to perform a retinotomy adjacent to the subfoveal perfluorocarbon bubble. The tip of the cannula was inserted into the bubble, which was removed with active suction. RESULTS: Both patients underwent successful removal of the perfluorocarbon liquid without complications. In one patient, visual acuity improved from 20/70 to 20/30, with resolution of his preoperative central scotoma. The second patient required removal of subfoveal perfluorocarbon liquid at the time of silicone oil removal. The preoperative vision of finger counting improved to 20/60. CONCLUSIONS: Subretinal perfluorocarbon can be removed using a flexible 39-gauge cannula without inducing retinal detachment or making a large retinotomy. This technique may benefit patients with retained subfoveal perfluorocarbon liquid.

Age-related macular degeneration: evaluation and treatment.

Any patient age 50 or older with distorted vision or vision loss may have age-related macular degeneration and should be immediately referred to an ophthalmologist. Early diagnosis and treatment are essential to preserve the current level of vision. We outline risk factors, clinical signs, what happens to the retina, and what treatments are currently available, as well as recommendations about vitamin and mineral supplementation.

Peripheral retinal degenerations and the risk of retinal detachment.

PURPOSE: To review the degenerative diseases of the peripheral retina in relationship with the risk to develop a rhegmatogenous retinal detachment and to present recommendations for use in eyes at increased risk of developing a retinal detachment. DESIGN: Focused literature review and author's clinical experience. RESULTS: Retinal degenerations are common lesions involving the peripheral retina, and most of them are clinically insignificant. Lattice degeneration, degenerative retinoschisis, cystic retinal tufts, and, rarely, zonular traction tufts, can result in a rhegmatogenous retinal detachment. Therefore, these lesions have been considered for prophylactic therapy; however, adequate studies have not been performed to date. CONCLUSIONS: Well-designed, prospective, randomized clinical studies are necessary to determine the benefit-risk ratio of prophylactic treatment. In the meantime, the evidence available suggests that most of the peripheral retinal degenerations should not be treated except in rare, high-risk situations.

Verteporfin therapy of subfoveal choroidal neovascularization in pathologic myopia: 2-year results of a randomized clinical trial--VIP report no. 3.

PURPOSE: To report 24-month vision and fluorescein angiographic outcomes from trials evaluating photodynamic therapy with verteporfin in patients with subfoveal choroidal neovascularization (CNV) caused by pathologic myopia. DESIGN AND SETTING: Multicenter, double-masked, placebo-controlled, randomized clinical trial at 28 ophthalmology practices in Europe and North America. PARTICIPANTS: Patients with subfoveal choroidal neovascular lesions caused by pathologic myopia measuring no more than 5400 micro m and best-corrected visual acuity (approximate Snellen equivalent) of 20/100 or better. METHODS: Similar to methods described for 1-year results with follow-up examinations beyond 1 year, continuing every 3 months (except Photograph Reading Center evaluations only at the month 24 examination). During the second year, the same regimen (with verteporfin or placebo as applied at baseline) was used if angiography showed fluorescein leakage from CNV. MAIN OUTCOME MEASURES: The primary outcome was the proportion of eyes with fewer than 8 letters (approximately 1.5 lines) of visual acuity loss at the month 24 examination, adhering to an intent-to-treat analysis and using the last observation carried forward method to impute for any missing data. RESULTS: Seventy-seven of 81 patients (95%) in the verteporfin group, compared with 36 of 39 patients (92%) in the placebo group, completed the month 24 examination. At this time point, 29 of 81 verteporfin-treated patients (36%) compared with 20 of 39 placebo-treated patients (51%) lost at least 8 letters (P = 0.11). The distribution of change in visual acuity at the month 24 examination was in favor of a benefit for the cases assigned to verteporfin (P = 0.05). This included improvement by at least 5 letters (equivalent to at least 1 line) in 32 verteporfin-treated cases [40%] vs. five placebo-treated cases (13%) and improvement by at least 15 letters (equivalent to at least 3 lines) in 10 verteporfin-treated cases (12%) vs. zero placebo-treated cases. No additional photosensitivity adverse reactions or injection site adverse events were associated with verteporfin therapy in the second year of follow-up. CONCLUSIONS: Verteporfin therapy for subfoveal CNV caused by pathologic myopia safely maintained a visual benefit compared with a placebo therapy through 2 years of follow-up. Although the primary outcome was not statistically significantly in favor of verteporfin therapy at 2 years as it had been at 1 year of follow-up, the distribution of change in visual acuity at the month 24 examination was in favor of the verteporfin-treated group and showed that this group was more likely to have improved visual acuity through the month 24 examination. The VIP Study Group recommends verteporfin therapy for subfoveal CNV resulting from pathologic myopia based on both the 1- and 2-year results of this randomized clinical trial.