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Jonathan H Tzu, Harry W Flynn Jr, Audina M Berrocal, William E Smiddy, Timothy G Murray, Yale L FisherDepartment of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USAPurpose: The purpose of this retrospective study was to evaluate the characteristic features, including spectral-domain optical coherence tomography (SD-OCT), clinical course, and outcome of treatment if given for patients with optic disc pit maculopathy.Methods: We investigated a consecutive series of patients with a diagnosis of optic pit maculopathy treated between 2001 and 2012 at the Bascom Palmer Eye Institute. Patients were divided into two main groups, ie, patients who were observed without surgery and patients who received surgical intervention. The main outcome measures were presenting and final visual acuity, and changes in SD-OCT imaging were recorded. Other data including age, gender, eye, age of onset, length of follow-up, location of optic pit, and location of fluid by OCT were also recorded.Results: On OCT, 67% (12/18) of the eyes showed schisis-like cavities, 22% (4/18) had only subretinal fluid, and 17% (3/18) had only a schisis-like cavity without subretinal fluid. In the patients managed by observation, visual acuity was ≥20/200 in 6/8 eyes initially and 6/8 eyes at last follow-up. Ten of 18 patients received either focal laser, surgery or both. Six of 10 eyes undergoing surgery had initial visual acuity ≥ 20/200, and 8 of 10 eyes undergoing surgery had a visual acuity of ≥20/200 at last follow-up.Conclusion: In this study, many eyes were observed and remained stable during follow-up. In eyes with reduced vision, surgical intervention produced variable outcomes, and persistent intraretinal/subretinal fluid was a common occurrence.Keywords: optic pit maculopathy, spectral-domain optical coherence tomography

Purpose: To evaluate the visual outcomes with unexplained vision loss during or after silicone oil (SO) tamponade. Methods: This multicenter retrospective case series comprised patients with unexplained vision loss associated with SO tamponade or its removal. Eyes with other clear secondary identifiable causes of vision loss were excluded. Results: Twenty-nine eyes of 28 patients (64% male) were identified. The mean age was 50 ± 13 years (range, 13-78 years). The mean duration of SO tamponade was 148 ± 38 days. Eighteen eyes (62%) developed unexplained vision loss while under SO; 11 (38%) had vision loss after SO removal. The most common optical coherence tomography (OCT) finding was ganglion cell layer (GCL) thinning (55%). Eyes with vision loss after SO removal had a mean logMAR best-corrected visual acuity (BCVA) of 0.6 ± 0.7 (Snellen 20/85) before SO tamponade and 1.2 ± 0.4 (20/340) before SO removal. By the last follow-up after SO removal, the BCVA had improved to 1.1 ± 0.4 (20/235). In eyes with vision loss after SO removal, the BCVA before SO removal was 0.7 ± 0.7 (20/104), which deteriorated to 1.4 ± 0.4 (20/458) 1 month after SO removal. By the last follow-up, the BCVA had improved to 1.0 ± 0.5 (20/219). Conclusions: Unexplained vision loss can occur during SO tamponade or after SO removal. Vision loss was associated with 1000-centistoke and 5000-centistoke oil and occurred in macula-off and macula-on retinal detachments. The duration of tamponade was 3 months or longer in the majority of eyes. Most eyes had GCL thinning on OCT. Gradual visual recovery can occur yet is often incomplete., Competing Interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr. Yannuzzi is on Advisory Board of Genentech and RegenXbio. Dr. Mantopoulos is a consultant to Alimera Sciences. Dr. Goduni has served on the Alimera Fellows Advisory Board and is an independent contractor for Clover Therapeutics. Dr. Jung is a consultant to Carl Zeiss Meditec, Inc., (© The Author(s) 2023.)

Purpose: To describe the safety and efficacy of rhegmatogenous retinal detachment (RRD) repair with external drainage of subretinal fluid using a 28-gauge External Drainage and Depression device (Vortex Surgical, Chesterfield, MO)., Methods: Retrospective review of patients who underwent primary rhegmatogenous retinal detachment repair with scleral buckle, pars plana vitrectomy, or scleral buckle/pars plana vitrectomy using the drainage device from August 2018 through March 2020, performed by four surgeons at two vitreoretinal practices., Results: Eighty-three eyes of 83 patients were included. At presentation, 28% had proliferative vitreoretinopathy. Surgery included 65% scleral buckle/pars plana vitrectomy, 33% pars plana vitrectomy, and 2% scleral buckle. There were no cases of retinal incarceration and two subretinal hemorrhages at the drainage site (both < 2 DD), 2 cases of recurrent RD with proliferative vitreoretinopathy (1 had proliferative vitreoretinopathy at presentation), and 6 (10%) new epiretinal membranes (3 were mild). There were no other complications. Mean follow-up was 274 days. Single operation success rate for those with ≥ 6-month follow-up was 97% (57/59)., Conclusion: External drainage of subretinal fluid during rhegmatogenous retinal detachment repair demonstrated a favorable safety profile with a high single operation success rate. Further study of the role of external drainage in rhegmatogenous retinal detachment repair is warranted., Competing Interests: D. W. Parke and E. Chang receive royalties from Vortex Surgical for their patent on the external drainage and depression device. The remaining authors have no other conflicts of interest to disclose.

Background and Objective: The authors investigate the efficacy of combination treatment with laser photocoagulation, intravitreal bevacizumab (IVB) (Avastin; Genentech, South San Francisco, CA), and sub-Tenon corticosteroids in Coats' disease., Patients and Methods: Patient records at the Bascom Palmer Eye Institute were reviewed from August 2002 to January 2014. Primary outcome measures were anatomic success, globe salvage, and final visual acuity., Results: Of the 26 patients (26 eyes) reviewed, 20 patients had stage 3A2, four patients had stage 3A1, and two patients had stage 2A. Mean follow-up time was 2.26 years. The median number of applications was five and three for laser and IVB, respectively. Five patients received sub-Tenon triamcinolone injections. Global salvage was achieved in all patients. Final visual acuity outcomes were reasonable: 20/20 to 20/50 in five patients, 20/60 to 20/100 in five patients, and 20/200 or less in 16 patients., Conclusion: Intravitreal injections of bevacizumab used in combination with repetitive laser photocoagulation therapy yielded anatomic success, global salvage, and reasonable visual acuity outcomes. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:443-449.]., (Copyright 2016, SLACK Incorporated.)

Purpose: To describe the long-term outcomes of intravitreal bevacizumab (IVB) for the treatment of pediatric retinal and choroidal diseases., Methods: This was a multicenter, retrospective, consecutive case series of patients <18 years of age treated with IVB from 2005 to 2013. Primary outcome measures included visual acuity and central macular thickness at 12 months' follow-up., Results: A total of 95 eyes of 90 patients (average age, 8.7 years [range, 0.33-17 years]) were included, in which 352 injections (average, 3.7/eye) were administered for choroidal neovascular membrane (CNVM, n = 35), Coats disease (n = 35), familial exudative vitreoretinopathy (FEVR, n = 13), cystoid macular edema (CME, n = 6), and other (n = 6). Mean follow-up was 679 ± 581 days. IVB was used as monotherapy in 27 eyes and as part of combination therapy in 68. Mean Snellen equivalent visual acuity improved from 20/224 at baseline to 20/120 at 6 months (P = 0.034) and 20/108 at 12 months (P = 0.005). Mean central macular thickness improved from 426 μm to 349 μm at 6 months (P = 0.025) and 340 μm at 12 months (P = 0.002). Statistically significant visual acuity gains at 12 months were achieved in patients with CNVM (P = 0.009) but not in eyes with CME (P = 0.06), Coats disease (P = 0.15), or FEVR (P = 0.93). Adverse effects included ocular hypertension in 8 eyes and worsening tractional retinal detachment in 3 eyes., Conclusions: Patients receiving IVB as part of the treatment for pediatric retinal and choroidal diseases experienced significant visual acuity gains and reductions in central macular thickness. IVB was generally well tolerated, although safety concerns persist., (Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the frequency of ocular surface symptoms and their potential impact on dry eye specific quality of life (QoL) in patients using versus not using glaucoma medications., Material and Methods: The study was a single-center, cross-sectional survey of patients seen at the Miami Veterans Affairs (VA) ophthalmology and optometry clinics from June to August, 2010. Patients were invited to complete the Dry Eye Questionnaire 5 (DEQ5) and the Impact of Dry Eye on Everyday Life (IDEEL) at their visit. Of 1348 patients seen in the Miami VA eye clinics during this three-month period, 467 patients completed the DEQ5 and 391 responded to both questionnaires. Outcome measures comprised ocular surface symptoms and their impact on dry eye specific QoL in patients using versus not using glaucoma drops., Results: An increasing number of glaucoma drops was significantly associated with an increased percentage of severe dry eye symptoms: no medications, 25% (n=89/353); 1 or 2 medications, 27% (n=17/62); 3 or more medications, 40% (n=21/52); p=0.03 (Armitage's test for linear-trend in proportions). There was an association between increasing number of drops and decreasing emotional well-being scores (linear p<0.001; quadratic p=0.029). Black patients had higher dry eye symptoms and lower emotional QoL scores compared to white patients at every level of medication use., Conclusion: An increasing number of glaucoma medications were associated with an increased frequency of severe dry eye symptoms and decreased emotional QoL. Additionally, dry eye specific emotional QoL was more severely affected in black versus white patients., (Published by Elsevier Ltd.)

Background and Objective: To investigate the clinical course and outcomes of patients with vitreomacular traction (VMT) managed initially by observation., Patients and Methods: This noncomparative case series included patients with a diagnosis of VMT based on clinical symptoms and findings on spectral-domain optical coherence tomography (SD-OCT) between 2005 and 2014. VMT was documented using a standardized grading system based on the degree of distortion of the foveal contour. Data were collected at five retina clinics using standardized collection forms. Visual acuity, changes in SD-OCT findings, and timing of the release of VMT as seen on SD-OCT were recorded., Results: The study included 230 eyes of 185 patients. Mean age was 72.5 years, and mean follow-up was 32 months. At baseline, VMT grading was grade 1 in 92 eyes (40%), grade 2 in 118 eyes (51.3%), and grade 3 in 20 eyes (8.7%). By last follow-up, spontaneous release of VMT occurred in 73 eyes (31.7%). Spontaneous release of VMT occurred at a mean of 18 months (median: 10.9 months) after initial visit. Mean logMAR best corrected visual acuity (BCVA) was 0.28 (20/55) (range: 20/20 to 20/400) at baseline and 0.25 (20/51) (range: 20/20 to 20/400) at last follow-up. Pars plana vitrectomy was performed in 10 eyes (4.1%) for macular hole (six eyes) and increased VMT (four eyes); BCVA was at least 20/40 in eight of the 10 eyes at last follow-up., Conclusion: Patients with VMT generally had a favorable clinical course when managed initially by observation. Spontaneous release of VMT occurred in approximately one-third of patients. At last follow-up, pars plana vitrectomy was performed in fewer than 5% of patients., (Copyright 2015, SLACK Incorporated.)

Purpose: This study was designed to evaluate the refractive outcomes of combined cataract extraction and glaucoma surgery., Design: Retrospective case series., Methods: A retrospective chart review of patients undergoing simultaneous cataract extraction with trabeculectomy or glaucoma drainage device surgery was performed. The main outcome measure evaluated was whether or not spherical equivalent of -1.00 to +0.50 D was achieved at 3 to 6 months postoperatively. Secondary outcomes included: reduction in intraocular pressure, amount of cylinder induced by combined surgery, and individual patient characteristics that may have affected refractive outcome. Outcomes were compared with an age-matched and sex-matched control group of patients who had uncomplicated cataract surgery during the same time period., Results: Forty-three eyes of 36 patients underwent combined cataract extraction and glaucoma surgery. A refractive outcome of spherical equivalent between -1.00 and +0.50 D was achieved in 32 of 43 eyes (74%) at 3 to 6 months after surgery. Logistic regression analysis found a 1.14 increased risk of the refraction being outside this defined refractive range in older compared with younger patients (by year, 95% CI, 1.04-1.27). Type of lens implant used, type of glaucoma surgery, and preoperative best-corrected visual acuity did not significantly affect refractive outcome. In a subset of 22 eyes with available preoperative keratometry measures, a mean 1.31 D (SD=0.86; range, 0.26 to 3.76) of corneal astigmatism was induced by combined surgery. In comparison, a matched control group who had cataract surgery alone achieved target refractive outcome in 34 of 40 eyes (85%, P=0.001) and had a trend for less induced cylinder (0.99, SD=0.72, P=0.11)., Conclusions: Favorable refractive outcomes were achieved in the majority of patients despite the potential alteration of preoperative measurements and introduction of error into lens selection when using a combined approach. There does not seem to be a difference in the refractive outcome with regard to the type of glaucoma surgery performed. Control patients who had cataract surgery alone had a higher percentage of achieving target refractive goal and less induced cylinder.

Purpose: To phenotypically and genotypically characterize a large Puerto Rican kindred with X-linked retinitis pigmentosa associated with a novel RP GTPase regulator (RPGR) genotype., Methods: A total of 100 family members of a single kindred with X-linked RP were evaluated with ophthalmic examinations and blood DNA analysis. Visual fields, OCT, and full-field ERG were obtained on all affected males and carriers., Results: Of the 100 family members examined, 13 were affected males and 18 were carriers. A deletion of 2 base pair of the RPGR gene in the ORF15 region at position c.2267-2268 (Lys756del2aaAG hemi) was identified with the affected and carriers. Best eye visual acuity was correlated with age (Spearman coefficient = 0.95) with hand-motion acuity by age 35 and light perception to no light perception by age 50-60. Visual fields were minimally plottable by age 40, and ERG responses reached non-detectable levels by late teens. Carriers had no or mild visual symptoms. All carriers had visual acuity of at least 20/50 or better in one eye, and the amount of retinal degeneration was variable with ERG responses ranging from severely impaired to normal., Conclusions: Profound visual loss occurred by the second decade of life with progression to near no light perception by age 60 in this kindred of X-linked RP associated with the RPGR genotype. Female carriers maintained visual acuity with age and were identifiable by clinical and ERG examination. The information from this study is important to determine the optimal age for intervention, as new RP treatments are being developed and tested.

The authors describe a 34-month-old boy who presented with a bilateral and asymmetric exudative retinopathy with similarities to Coats’ disease. The patient’s medical history was remarkable for hypotonia, developmental delay, seizures, and intracranial calcifications. Genetic testing revealed a diagnosis of Coats’ plus. This rare genetic disease should be in the differential diagnosis in patients who present with a bilateral and asymmetric Coats’-like retinopathy in the presence of other systemic abnormalities.

This is a report of a 5-year-old boy who presented with an exudative retinopathy consistent with Coats' disease. Optical coherence tomography confirmed the concurrent presence of retinoschisis in the same eye, adjacent to the areas of exudation and ischemia. Treatment with laser photocoagulation, corticosteroids, and anti-VEGF therapy led to the resolution of the schisis cavity 1 year later. This represents the second published account of retinoschisis in the setting of Coats' disease., (Copyright 2014, SLACK Incorporated.)

A 16-year-old adolescent girl with multiple risk factors for thrombosis presented with acute onset of headache, decreased vision, and papilledema. Evaluation demonstrated cerebral venous thrombosis (CVT) involving the left transverse and sigmoid sinuses and left internal jugular vein. Following bilateral optic nerve sheath fenestration (ONSF), she experienced improvement in vision and resolution of papilledema. In selected cases, ONSF is an effective surgical option for the treatment of papilledema due to CVT after medical treatment has failed.

The pigmented fundus lesions associated with familial adenomatous polyposis (FAP) often resemble those characteristic of congenital hypertrophy of the retinal pigment epithelium (CHRPE). However, some fundus lesions in FAP resemble hamartomatous lesions of the retinal pigment epithelium (RPE). Intraretinal extension of the RPE is a feature not seen in CHPRE lesions that may be unique to some of the pigmented lesions of FAP. The authors report the spectral-domain optical coherence tomography findings of the pigmented ocular lesions associated with FAP in a 10-year-old boy.

The authors present the case of a 6-month-old female infant with a known diagnosis of incontinentia pigmenti with a normal examination by indirect ophthalmoscopy. However, fluorescein angiography revealed vascular abnormalities that were not detected by indirect ophthalmoscopy. Follow-up examination revealed progressive vascular changes that again were only detectable by fluorescein angiography. Because vision loss can cause significant morbidity in incontinentia pigmenti, the use of fluorescein angiography as an adjunctive tool with exams under anesthesia may provide invaluable information in the detection of early vascular changes in this disease., (Copyright 2013, SLACK Incorporated.)

Purpose: To report the occurrence of chronic hypotony as a complication following Boston type 1 keratoprosthesis (KPro) implantation and to determine associated risk factors., Design: Retrospective case series., Methods: The medical records of all patients undergoing KPro implantation between January 2004 and November 2010 at the Wilmer Eye Institute were reviewed. Patients who developed chronic hypotony following Boston type 1 KPro implantation not attributable to anatomic problems (eg, retinal detachment, overfiltering glaucoma tube shunts, tissue necrosis with aqueous leak) or other causes were identified. Demographics and preoperative, intraoperative, and postoperative clinical findings were analyzed. Light microscopic examination of an enucleated globe as well as fibrous retroprosthetic membranes excised at the time of vitrectomy from another patient was performed., Results: Sixty-eight eyes received KPro implantation over the study period. Six eyes experienced chronic hypotony, with a median time of 18.5 months between KPro implantation and the onset of hypotony. The incidence of chronic hypotony in this series was calculated to be 3.7% at 1 year (95% CI = 0.9%-14.0%) and 13.3% at 2 years (95% CI = 5.5%-30.0%). Of the 6 eyes that developed chronic hypotony, 5 had a previous history of glaucoma or ocular hypertension, but only 3 had a glaucoma drainage implant. All eyes progressing to chronic hypotony were noted to have a retroprosthetic membrane prior to the onset of hypotony. Cox regression modeling demonstrated an increased risk of chronic hypotony in eyes with retroprosthetic membranes (P < .01) but no increase in risk for older patients (P > .1), eyes with glaucoma drainage implants (P > .5), or a previous history of multiple donor corneal transplants (P > .5)., Conclusion: Chronic hypotony can be a significant complication of KPro implantation and should be assessed carefully in eyes with retroprosthetic membranes. Further study of an association between hypotony and retroprosthetic membrane formation may be useful to gain insight into the mechanism of this complication and potential prevention or treatment options., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the long-term effectiveness of the combination of topical cyclosporine drops and tacrolimus ointment in the treatment of steroid-dependent atopic keratoconjunctivitis (AKC)., Methods: Ten patients with moderate to severe AKC participated in the study. Topical cyclosporine 0.05% was used, as a monotherapy, 6 times daily during the first month of the study, followed by 4 times daily during the second month. The patients were then instructed to self-treat with topical cyclosporine at a dose ranging from 2 to 6 times daily depending on the severity of the disease. Tacrolimus ointment 0.03% was applied on the lid skin. Follow-up examinations were performed approximately every 3 months. Each patient completed a follow-up period of at least 12 months. Symptoms and signs of AKC were assessed on the day of enrollment, on days 28, 56, and 63, and at subsequent follow-up visits. Flare-ups of AKC requiring steroid use and progression of the disease findings were also recorded., Results: All patients experienced significant improvement of their symptoms and signs during the first 2 months of the study. Two patients were lost to follow-up after the initial 2 months. One patient was noncompliant and continued the treatment only for 7 months. During the median treatment period of 21.5 months for the 7 continuing patients, a total of only 2 flare-up episodes were noted requiring topical steroids., Conclusions: Adequate topical immunomodulation using topical calcineurin inhibitors may eliminate the need for steroids and favorably alter the long-term prognosis of patients with AKC.

Purpose: To report the clinical outcomes of lamellar keratoplasty using sterile, gamma-irradiated corneal tissues (VisionGraft) for partial-thickness corneal defects., Design: Interventional case series., Methods: The medical records of 10 patients with partial-thickness corneal defects who were operated at The Wilmer Eye Institute between April 2009 and December 2009 were retrospectively reviewed. Indications for surgery included corneal melt with microperforation (n = 6), keratoprosthesis-associated corneal melt (n = 2), and noninflammatory limbal lesions (n = 2). The grafts were fashioned from full- or partial-thickness tissues using disposable trephines, based on the depth, shape, and size of the defect in the recipient bed, and were secured with multiple interrupted 10/0 nylon sutures. Rate of epithelialization and clarity of the grafts at the last visit were assessed retrospectively., Results: All but 1 graft became epithelialized between postoperative 1st and 13th days. Corneal inflammation progressed in the 1 patient with Boston type 1 keratoprosthesis-associated corneal melt, probably attributable to the progression of underlying Sjögren syndrome, despite initiation of systemic immunomodulatory treatment, and required replacement of the device. The donor tissues remained clear in all the other cases over a period of 7 to 15 months. No immune rejection, infection, significant opacification, or neovascularization of the donor tissues were noted during a follow-up period., Conclusions: VisionGraft may be considered in lieu of fresh donor corneas for lamellar corneal patch grafts because of its availability, easy handling, and lack of immunogenicity., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Here we present a potential novel surgical technique consisting of fixation of a posterior-chamber intraocular lens to the iris that may be used in the treatment of aphakia or the management of intraocular lens complications when capsular support has been compromised. The technique was performed in a laboratory model using cadaveric human eyes. A commercially available neurovascular clip was used to securely fasten the intraocular lens to the iris with minimal trauma. The use of a metal clip has the advantage of avoiding potential risks of suture fixation such as suture breakage. Also, this technique is easier than suturing and may potentially serve as another tool in a cornea surgeon's armamentarium.