Ranibizumab for eyes previously treated with pegaptanib or bevacizumab without clinical response.

BACKGROUND AND OBJECTIVE: To assess the safety and efficacy of ranibizumab in patients who had inadequate response to pegaptanib or bevacizumab. PATIENTS AND METHODS: In this single-center study, 19 patients with subfoveal choroidal neovascularization secondary to age-related macular degeneration (AMD) previously treated with pegaptanib (n = 1), bevacizumab (n = 13), or both (n = 5) received 12 monthly ranibizumab injections (0.5 mg). Outcomes were measured from start of previous therapy (baseline) to start of ranibizumab treatment (day 0) through 12 months. RESULTS: No drug- or injection-related adverse events and no serious adverse events were reported. At 6 and 12 months, 4 and 5 patients gained 3 or more lines of VA, respectively; 3 patients lost less than 3 lines of VA at 6 months, and 6 patients lost less than 3 lines at 12 months. At 6 and 12 months, VA increased by a mean (± standard error of the mean) of 2.06 ± 1.23 and 1.17 ± 0.62 lines, respectively. Central retinal thickness decreased by a mean of 62.65 ± 22.46 and 62.16 ± 29.20 µm at months 6 and 12, respectively. When stratified by pigment epithelial detachment (PED) status, patients without PED had better visual and anatomical outcomes than patients with PED. CONCLUSION: Ranibizumab has favorable safety and efficacy profiles for patients with AMD without previous response to pegaptanib or bevacizumab.

Postoperative complications associated with 25-gauge pars plana vitrectomy.

BACKGROUND AND OBJECTIVE: To report postoperative complications in eyes undergoing 25-gauge pars plana vitrectomy (PPV). PATIENTS AND METHODS: Seventy consecutive eyes that underwent 25-gauge PPV for various indications, including epiretinal membrane, non-clearing vitreous hemorrhage, and idiopathic macular hole, and had a minimum follow-up of 12 weeks were reviewed retrospectively. Main outcome measures included best-corrected Snellen visual acuity, intraocular pressure (IOP), intraoperative complications, and postoperative complications. RESULTS: The mean visual acuity improved from 20/368 preoperatively to 20/105 postoperatively (P < .00005). Intraoperative complications included retinal tears in 2 eyes (2.9%). Postoperative complications included cataract progression in 17 eyes (42.5%), cystoid macular edema exacerbation in 5 eyes (7.1%), and retinal detachment in 1 eye (1.4%). Postoperative day 1 IOP was statistically lower than preoperative IOP in fluid-filled eyes (P = .031) but not in eyes filled with intravitreal air (P = .30) or gas (P = .52). Sclerotomy sutures were required intraoperatively in 5 eyes (7.1%) and postoperative day 1 hypotony was noted in 4 eyes (5.7%). All of these complications were noted in fluid-filled eyes except for one case of postoperative day 1 hypotony with gas tamponade. CONCLUSIONS: Intraoperative and postoperative complications were rare in this series of 25-gauge PPV. Postoperative cataract progression and hypotony were the most common complications. Fluid-filled eyes appear to have a higher risk of wound leakage and postoperative hypotony after 25-gauge PPV than eyes with air or gas tamponade.

Pars plana vitrectomy versus combined pars plana vitrectomy-scleral buckle for primary repair of pseudophakic retinal detachment.

PURPOSE: To evaluate pars plana vitrectomy (PPV) versus a combined PPV and scleral buckle (PPV/SB) for repair of noncomplex, pseudophakic retinal detachment. DESIGN: Retrospective, nonrandomized, comparative interventional study. PARTICIPANTS: One hundred fifty-two eyes of 152 patients followed up for a mean of 10 months. The case series included 68 consecutive patients who underwent PPV and 84 consecutive patients who underwent a PPV/SB for primary repair of primary pseudophakic retinal detachment at Wills Eye Hospital between 2002 and 2004. METHODS: All primary PPV cases were performed by 2 surgeons who perform primary vitrectomy without regard to location of detachment, number and location of break(s), refractive error, or macula status. All primary PPV/SB were performed by a group of surgeons who solely perform PPV/SB on pseudophakic retinal detachments. All eyes underwent a standard 3-port 20-gauge PPV under wide-field viewing and scleral depression. Endolaser photocoagulation was applied either around the retinal tears or 360 degrees to the vitreous base region followed by gas tamponade. Patients with proliferative vitreoretinopathy grade C or worse were excluded from the study. MAIN OUTCOME MEASURES: (1) Single surgery anatomic success rates, (2) preoperative and postoperative visual acuity, and (3) complications. RESULTS: The single surgery anatomic success rate in the primary PPV group was 63 of 68 eyes (92.6%; 95% confidence interval [CI], 84%-98%) and in the primary PPV/SB group was 79 of 84 eyes (94.0%; 95% CI, 87%-98%). Both groups obtained 100% final reattachment rate. There was no statistically significant difference between the success rates (P = 0.75, Fisher exact test). The PPV group's best-corrected postoperative visual acuity demonstrated a +0.10 logarithm of the minimum angle of resolution improvement over the PPV/SB group (P = 0.07). The PPV group had a smaller incidence of postoperative complications (13/68 patients [19.1%] vs. 27/84 patients [32.1%]; P = 0.10, Fisher exact test). CONCLUSIONS: Primary PPV and PPV/SB seem to have similar efficacy in the repair of a matched group of patients with primary noncomplex pseudophakic retinal detachment. There was no statistically significant difference in complication rate between the 2 groups.

Laser treatment for retinopathy of prematurity: evolution in treatment technique over 15 years.

PURPOSE: To assess the number of laser burns applied to the peripheral retina to treat retinopathy of prematurity (ROP) in a recent population of patients and to compare that number with historical data. METHODS: The authors performed a retrospective chart review of all patients treated for ROP within a single practice from 2002-2004. There were 183 eyes from 93 patients. Sufficient data for analysis were available on 112 eyes from 57 patients. Data from these patients were compared to those of patients reported upon in 1991. RESULTS: The average number of laser burns applied to the 112 eyes was 2163. Eyes with zone 1 disease had an average of 3077 spots and those with zone 2 disease had an average of 2072 spots. Greater than three months follow-up was available on 42 eyes from 21 patients. Of those eyes, 90% had complete regression of ROP. In an initial report of laser treatment for ROP by several of the same authors in 1991, the average number of laser burns applied for treatment of ROP was 816. There was a 265% increase in the average number of laser applications from the early study to the present. CONCLUSION: The data indicate that there has been a trend towards more confluent laser treatment to the avascular zone in ROP.

Photodynamic therapy in young patients.

BACKGROUND AND OBJECTIVE: To present a consecutive case series of patients 50 years or younger who underwent photodynamic therapy (PDT) for choroidal neovascularization (CNV) from etiologies other than age-related macular degeneration. PATIENTS AND METHODS: Retrospective chart review of 35 consecutive eyes of 34 patients. RESULTS: Visual acuity remained stable or improved in 20 of 35 eyes. Thirteen eyes with myopic degeneration had pre-treatment and post-treatment mean visual acuities of 20/100 and 20/200, respectively. Eight eyes with idiopathic CNV had an improvement of mean visual acuity from 20/200 to 20/125. Six eyes with ocular histoplasmosis displayed a stable mean visual acuity of 20/50. Of 3 eyes with angioid streaks, visual acuity remained stable in 2 eyes and declined in 1 eye. Five eyes with other etiologies all had improved vision. CONCLUSION: Although the current literature shows evidence that PDT is beneficial in treating CNV secondary to myopic degeneration, the evidence for etiologies such as idiopathic causes, ocular histoplasmosis, and angioid streaks is optimistic but remains unproven. Our study suggests that PDT may be beneficial in stabilizing and improving vision when treating CNV from these etiologies.

Retinopathy of prematurity: the life of a lifetime disease.

PURPOSE: To provide information on retrolental fibroplasias (RLF), later known as retinopathy of prematurity. DESIGN: Review of the literature on the subject and a first-person account of what was then RLF by one of the authors (A.P.) who was involved in the earliest days in research regarding RLF. METHODS: MEDLINE search on the topics of RLF and retinopathy of prematurity plus a first-person historic review of original work that dealt with RLF. RESULTS: In 1942, elevated levels of oxygen were thought to play a major role in the development of the disease; at that time, no treatment was available. During the lifetime of this disease, other possible causes have been investigated. These include vitamin E as a prophylaxis against retinopathy of prematurity and the efficacy of light reduction to prevent retinopathy of prematurity. It has been shown that the light reduction does not play a role in reducing the progression of retinopathy of prematurity. Vitamin E studies were inconclusive; some studies show a positive effect and others do not. A major advance occurred with the development of the International Classification of Ophthalmology in 1984, which laid the groundwork for collaborative studies to determine whether cryotherapy of the avascular zone of retina would reduce the incidence of blindness in newborn infants, when compared with control subjects. The study showed that cryotherapy was effective; this was followed by laser photocoagulation when lasers became portable enough to take to the neonatal intensive care unit. At the same time, improved surgical techniques moved from scleral buckling for retinal detachment to vitrectomies (some lens sparing) for more desperate cases that had progressed to stage 4 and stage 5 retinopathy of prematurity. Late changes in adults who were born before any treatment and are now baby boomers ran the gamut from the dragging of the retina in the posterior pole to retinal detachment, cataract, and myopia. CONCLUSION: Retinopathy of prematurity is a lifetime disease for which preventive and better treatment modalities continue to evolve.

A comparison of laser photocoagulation with cryotherapy for threshold retinopathy of prematurity at 10 years: part 1. Visual function and structural outcome.

OBJECTIVE: To assess visual and structural outcomes after laser photocoagulation and transscleral cryotherapy for threshold retinopathy of prematurity (ROP) after 10 years. DESIGN: Extended follow-up of a randomized controlled clinical trial. PARTICIPANTS: One hundred eighteen eyes from 66 patients were randomly assigned to receive either cryotherapy or laser photocoagulation for threshold ROP. Forty-four eyes from 25 patients were examined for 10-year follow-up evaluations. INTERVENTION: Early Treatment of Diabetic Retinopathy Study (ETDRS) visual acuity, slit-lamp, and fundus examination; fundus photography; and B-scans (eyes with retinal detachments) were performed. Patients' histories were taken to elicit past amblyopia therapy. Based on fundus photographs, independent observers graded the degree of retinal dragging as none, mild, moderate, or severe. MAIN AND SECONDARY OUTCOME MEASURES: Best-corrected ETDRS visual acuity (BCVA). The degree of dragging was determined clinically and photographically. In addition, the presence of strabismus or amblyopia and/or any history of treatment for amblyopia were noted accordingly. RESULTS: Eyes treated with laser had a mean BCVA of 20/66 (Snellen equivalent), whereas cryotherapy-treated eyes had a mean BCVA of 20/182 (Snellen equivalent) (P = 0.015, n = 42). Compared with eyes treated with cryotherapy, eyes treated with laser photocoagulation were 5.2 times more likely to have a 20/50 or better BCVA (95% confidence interval, 1.37-19.8, n = 42). Eyes treated with cryotherapy were 7.2 times (95% confidence interval, 1.54-33.6, n = 33) more likely to develop retinal dragging compared with laser treatment. By linear regression analysis, ETDRS visual acuity was inversely proportionate to the degree of retinal dragging in both laser (r = -0.637, P = 0.006) and cryotherapy (r = -0.517, P = 0.040) treated eyes. Among the 21 patients with favorable outcomes in both eyes, 13 had strabismus (62%) and 6 had received amblyopia therapy (29%). Ptosis, loss of cilia, and cortical cataract were among probable treatment-related complications that were noted in this study. CONCLUSIONS: Overall, laser-treated eyes had better structural and functional outcome compared with eyes treated with cryotherapy.

A comparison of laser photocoagulation with cryotherapy for threshold retinopathy of prematurity at 10 years: part 2. Refractive outcome.

OBJECTIVE: To compare the refractive outcome of eyes treated with cryotherapy for threshold retinopathy of prematurity (ROP) with eyes treated with laser photocoagulation. DESIGN: Extended follow-up of a randomized controlled clinical trial. PARTICIPANTS: One hundred eighteen eyes from 66 patients were randomly assigned to receive either cryotherapy or laser photocoagulation for threshold ROP. Twenty-five patients (44 eyes treated) were available for follow-up examination 10 years later. INTERVENTION: Cycloplegic autorefraction or retinoscopy was performed. Immersion ultrasound biometry (A-scan) was also performed, and an autokeratometer was used for keratometry. If an A-scan could not be tolerated or the patient was not cooperative, a B-scan through-the-lid biometry was performed. Corneal thickness was measured using optical coherence tomography. MAIN AND SECONDARY OUTCOME MEASURES: Refractive error. In addition, anterior chamber depth, lens thickness, and axial length were measured. Central corneal thickness measurements and keratometric readings were also obtained. RESULT: Eyes treated with cryotherapy were significantly more myopic than those treated with laser photocoagulation. When comparing patients with bilateral treatment, the mean spherical equivalent (SE) of eyes treated with laser was -4.48 diopters (D) compared with a mean SE of -7.65 D for eyes treated with cryotherapy (n = 15 pairs of eyes, P = 0.019). Cryotherapy-treated eyes had a mean axial length of 21.7 mm versus 22.9 mm for laser-treated eyes (P = 0.024, n = 12 pairs of eyes). The anterior chamber depth and lens thickness averaged 2.86 mm and 4.33 mm, respectively, in the cryotherapy-treated eyes compared with 3.42 mm and 3.95 mm in the laser-treated eyes (P < 0.001, n = 12 pairs for both measurements). There were no statistical differences in anterior corneal curvature and central corneal thickness between the two treatment modalities. Crystalline lens power bore the strongest correlation to refractive outcomes in both laser-treated (r = 0.885, P < 0.001) and cryotherapy-treated eyes (r = 0.591, P = 0.026). Although keratometric readings were higher than normal values in these eyes, there was no correlation to the degree of myopia. CONCLUSIONS: Laser-treated eyes were significantly less myopic than cyrotherapy-treated eyes. Lens power seemed to be the predominant factor contributing to the excess myopia.

Newfoundland rod-cone dystrophy, an early-onset retinal dystrophy, is caused by splice-junction mutations in RLBP1.

Some isolated populations exhibit an increased prevalence of rare recessive diseases. The island of Newfoundland is a characteristic geographic isolate, settled by a small number of families primarily during the late 1700s and early 1800s. During our studies of this population, we identified a group of families exhibiting a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression, a disorder that we termed "Newfoundland rod-cone dystrophy" (NFRCD). The size of one of these families was sufficient to allow us to perform a genomewide screen to map the NFRCD locus. We detected significant linkage to markers on the long arm of chromosome 15, in a region encompassing RLBP1, the gene encoding the cellular retinaldehyde-binding protein. Previously, mutations in RLBP1 have been associated with other retinal dystrophies, leading us to hypothesize that RLBP1 mutations might also cause NFRCD. To test this hypothesis, we sequenced all coding exons and splice junctions of RLBP1. We detected two sequence alterations, each of which is likely to be pathogenic, since each segregates with the disease and is predicted to interfere with mRNA splicing. In contrast to some previously reported RLBP1 mutations, which yield a protein that may retain some residual activity, each NFRCD mutation is likely to give rise to a null allele. This difference may account for the severe phenotype in these families and exemplifies the molecular continuum that underlies clinically distinct but genetically related entities.

Axenfeld-Rieger syndrome associated with Bull's eye maculopathy

A síndrome de Axenfeld-Rieger representa um espectro de anomalias de desenvolvimento caracterizadas por alteraçöes da periferia da córnea, íris e seio camerular, além de malformaçöes ósseas e dentárias. Existem algumas descriçöes esparsas de anormalidades de fundo de olho, porém estas näo foram documentadas. Nós descrevemos, pela primeira vez, um caso de Síndrome de Axenfeld-Rieger associada à maculopatia bilateral do tipo "bull'eyes"