BILATERAL CHOROIDAL EXCAVATION IN JUVENILE LOCALIZED SCLERODERMA.

PURPOSE: To describe a case of bilateral choroidal excavation in a patient with juvenile localized scleroderma. METHODS: Case report. RESULTS: An asymptomatic 12-year-old boy with localized scleroderma presented for examination and was found to have bilateral areas of choroidal excavation temporal to the fovea. CONCLUSION: Previous reports of ocular complications of localized scleroderma have primarily described adnexal and anterior segment changes. This is the second report of choroidal changes in a patient with localized scleroderma, and the first in a pediatric patient.

Incomplete Retinal Vascularization After Ranibizumab Treatment of Retinopathy of Prematurity.

A former 24-week-old premature infant was treated with intravitreal ranibizumab (Lucentis; Genentech, South San Francisco, CA) in one eye and conventional laser in the other eye for aggressive posterior retinopathy of prematurity in both eyes. Fluorescein angiography performed at 149 weeks of age showed persistent avascularity of the temporal peripheral retina in the ranibizumab-treated eye. This case report confirms the need for long-term follow-up of patients treated with ranibizumab monotherapy. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:75-78.].

DIDANOSINE RETINAL TOXICITY.

PURPOSE: To report nine new cases of retinal degeneration secondary to didanosine toxicity and to summarize the previously reported cases in the literature. METHODS: This was a multicenter, retrospective, observational case study from seven institutions. Medical records of patients who demonstrated well-demarcated severe midperipheral chorioretinal degeneration and who were previously treated with didanosine therapy were collected and the following information was reviewed: age, gender, medical history, detailed medication history including current and previous antiretroviral use, ocular and retinal examination findings, and multimodal imaging findings with optical coherence tomography, fundus photography, wide-field fundus autofluorescence, and wide-field fluorescein angiography. When available, findings with electrophysiology testing and automated perimetry were also collected and reviewed. A literature review was also performed to collect all reported cases of chorioretinal degeneration secondary to didanosine toxicity. RESULTS: Nine patients were identified who had findings consistent with peripheral retinal toxicity secondary to didanosine use. Eight of the 9 patients were men, and the median age was 54 years at the time of presentation (mean: 55 years, range, 42-71 years). Snellen distance acuity ranged from 20/20 to 20/32. At least three of the cases in the series demonstrated progression of the peripheral retinal pigment epithelium and photoreceptor atrophy despite didanosine cessation. A review of the literature revealed 10 additional cases of didanosine toxicity. Seven of the 10 cases were in men (70%), and the average age was 26 years with a wide range (2-54 years). Chorioretinal findings were very similar to this cohort. CONCLUSION: Herein, we report the largest series of nine cases of peripheral chorioretinal degeneration secondary to didanosine toxicity in adults. When combined with the cases in the literature, 19 cases of didanosine toxicity, 4 of which occurred in children, were collected and analyzed. Three of the new cases presented showed clear progression of degeneration despite didanosine cessation. Newer nucleoside reverse transcriptase inhibitors may potentiate mitochondrial DNA damage and lead to continued chorioretinal degeneration.

Combined rhegmatogenous and traction detachment associated with vasoproliferative tumor secondary to sickle cell retinopathy.

PURPOSE: To report the surgical management of a combined rhegmatogenous and traction retinal detachment associated with a vasoproliferative tumor secondary to sickle cell retinopathy. OBSERVATIONS: A 29 year old man from Ghana presented with unilateral vision loss, ischemic retina and sea fan neovascularization in both eyes and a retinal detachment nearby a vasoproliferative tumor (VPT) in the left eye. Hemoglobin electrophoresis led to the diagnosis of sickle cell disease. The patient underwent vitrectomy with scleral buckle surgery, resection of the tumor, and removal of subretinal membranes in the left eye. Laser photocoagulation was targeted to areas of ischemic retina in both eyes. CONCLUSIONS: and Importance: To our knowledge, this is the first report of a combined rhegmatogenous and traction retinal detachment associated with a VPT in sickle cell retinopathy managed by modern vitrectomy techniques. Prompt recognition of the condition and surgical management addressing both rhegmatogenous and tractional components can lead to improved outcome.

INTRAVITREAL SULFUR HEXAFLUORIDE INJECTION FOR THE TREATMENT OF VITREOMACULAR TRACTION SYNDROME.

PURPOSE: Vitreomacular traction (VMT) syndrome can cause symptomatic metamorphopsia and decreased visual acuity. Although it is typically treated with vitrectomy or intravitreal ocriplasmin injection, these procedures can be invasive and costly. The purpose of this retrospective, consecutive case series was to evaluate the efficacy of intravitreal expansile sulfur hexafluoride gas injection for the treatment of symptomatic VMT syndrome. METHODS: Nine eyes of 9 patients with symptomatic VMT syndrome on spectral domain optical coherence tomography received an intravitreal injection of 0.3 mL of 100% sulfur hexafluoride. The primary outcome was the number of eyes with release of VMT on spectral domain optical coherence tomography at 1 month after treatment. Secondary outcomes included change in visual acuity and central subfield thickness 1 month after treatment. RESULTS: Five patients (55.6%) had release of VMT on spectral domain optical coherence tomography by 1 month after injection. Two patients who had Stage I macular holes before injection had closure of the macular holes. Mean visual acuity at 1 month improved slightly after injection by 0.09 logMAR units, although this change was not statistically significant (P = 0.15). Central subfield thickness on spectral domain optical coherence tomography decreased by an average of 35.3 microns after injection (P = 0.004). All eyes with release of VMT had pretreatment vitreomacular adhesion of less than 521 microns and none had epiretinal membranes. One patient (11.1%) developed a peripheral retinal hole at 1 month after injection. CONCLUSION: Intravitreal injection of expansile sulfur hexafluoride gas is a low-cost and minimally invasive alternative for the treatment of symptomatic VMT syndrome. Further study is warranted.

Vitamin D deficiency in neovascular versus nonneovascular age-related macular degeneration.

PURPOSE: To compare 25-hydroxyvitamin D (25OHD) levels in patients with neovascular age-related macular degeneration (NVAMD) with patients with nonneovascular age-related macular degeneration and control patients. METHODS: Medical records of all patients diagnosed with age-related macular degeneration and tested for serum 25OHD level at a single medical center were reviewed. Control patients were selected from patients diagnosed with pseudophakia but without age-related macular degeneration. The lowest 25OHD level available for each patient was recorded. RESULTS: Two hundred sixteen patients with nonneovascular age-related macular degeneration, 146 with NVAMD, and 100 non-age-related macular degeneration control patients were included. The levels of 25OHD (mean ± SD) were significantly lower in NVAMD patients (26.1 ± 14.4 ng/mL) versus nonneovascular age-related macular degeneration (31.5 ± 18.2 ng/mL, P = 0.003) and control (29.4 ± 10.1 ng/mL, P = 0.049) patients. The prevalence of vitamin D insufficiency (<30 ng/mL 25OHD), deficiency (<20 ng/mL), and severe deficiency (<10 ng/mL) were highest in the NVAMD group. The highest quintile of 25OHD was associated with a 0.35 (95% confidence interval, 0.18-0.68) odds ratio for NVAMD. CONCLUSION: This is the largest study to compare 25OHD levels in patients with the different clinical forms of age-related macular degeneration. Mean 25OHD levels were lower and vitamin D deficiency was more prevalent in NVAMD patients. These associations suggest that further research is necessary regarding vitamin D deficiency as a potentially modifiable risk factor for the development of NVAMD.

Preclinical evaluation and intraoperative human retinal imaging with a high-resolution microscope-integrated spectral domain optical coherence tomography device.

PURPOSE: The authors have recently developed a high-resolution microscope-integrated spectral domain optical coherence tomography (MIOCT) device designed to enable OCT acquisition simultaneous with surgical maneuvers. The purpose of this report is to describe translation of this device from preclinical testing into human intraoperative imaging. METHODS: Before human imaging, surgical conditions were fully simulated for extensive preclinical MIOCT evaluation in a custom model eye system. Microscope-integrated spectral domain OCT images were then acquired in normal human volunteers and during vitreoretinal surgery in patients who consented to participate in a prospective institutional review board-approved study. Microscope-integrated spectral domain OCT images were obtained before and at pauses in surgical maneuvers and were compared based on predetermined diagnostic criteria to images obtained with a high-resolution spectral domain research handheld OCT system (HHOCT; Bioptigen, Inc) at the same time point. Cohorts of five consecutive patients were imaged. Successful end points were predefined, including ≥80% correlation in identification of pathology between MIOCT and HHOCT in ≥80% of the patients. RESULTS: Microscope-integrated spectral domain OCT was favorably evaluated by study surgeons and scrub nurses, all of whom responded that they would consider participating in human intraoperative imaging trials. The preclinical evaluation identified significant improvements that were made before MIOCT use during human surgery. The MIOCT transition into clinical human research was smooth. Microscope-integrated spectral domain OCT imaging in normal human volunteers demonstrated high resolution comparable to tabletop scanners. In the operating room, after an initial learning curve, surgeons successfully acquired human macular MIOCT images before and after surgical maneuvers. Microscope-integrated spectral domain OCT imaging confirmed preoperative diagnoses, such as full-thickness macular hole and vitreomacular traction, and demonstrated postsurgical changes in retinal morphology. Two cohorts of five patients were imaged. In the second cohort, the predefined end points were exceeded with ≥80% correlation between microscope-mounted OCT and HHOCT imaging in 100% of the patients. CONCLUSION: This report describes high-resolution MIOCT imaging using the prototype device in human eyes during vitreoretinal surgery, with successful achievement of predefined end points for imaging. Further refinements and investigations will be directed toward fully integrating MIOCT with vitreoretinal and other ocular surgery to image surgical maneuvers in real time.

Comparison of Humphrey Matrix frequency doubling technology to standard automated perimetry in neuro-ophthalmic disease.

PURPOSE: We compared Humphrey Matrix FDT 30-2 (FDT) and Humphrey Visual Field Analyzer 30-2 SITA standard (SAP) in the assessment of anterior (optic nerve or chiasm) and posterior (retro-chiasmal) afferent visual pathway defects. MATERIALS AND METHODS: In this retrospective comparative study, the charts of 37 patients (16 males, range 13-84 years, mean 72.1), with neuro-ophthalmic visual field defects who were tested with both FDT and SAP, were reviewed. Two masked graders assessed the concordance and extent of field defects between the perimeters. The mean concordance between anterior and posterior disease was compared using the Wilcoxon rank sum test. The mean deviation (MD) and pattern standard deviation (PSD) of each perimeter were correlated with the Spearman coefficient. RESULTS: Twenty-eight patients had anterior and nine had posterior disease. Most had a fair or good concordance (89.3% anterior, 88.9% posterior). When comparing anterior to posterior disease, the mean concordance of the defects of the two parameters was not statistically different (P = 0.94 and P = 0.61 for total deviation and pattern deviation, respectively). The MD and PSD between perimeters had a significant correlation. CONCLUSIONS: Our series, using 30-2 field analysis, demonstrates fair to good correlation between FDT and SAP in the majority of patients. In roughly 10% findings between FDT and SAP were discordant. This difference was similar for anterior and posterior disease.

Medicare costs for neovascular age-related macular degeneration, 1994-2007.

PURPOSE: To assess changes in Medicare payments for neovascular age-related macular degeneration (AMD) since introduction of anti-vascular endothelial growth factor (VEGF) therapies. DESIGN: Retrospective, longitudinal cohort study. METHODS: Using the Medicare 5% sample, beneficiaries with new diagnoses of neovascular AMD in 1994 (N = 2497), 2000 (N = 3927), and 2006 (N = 6041) were identified using International Classification of Diseases (ICD-9-CM). The total first-year health care and eye care costs were calculated for each beneficiary. Propensity score matching was used to match individuals in the 2000 and 2006 cohorts with the 1994 cohort on age, sex, race, Charlson Comorbidity Index, and low vision/blindness. RESULTS: The number of beneficiaries newly diagnosed with neovascular AMD more than doubled between the 1994 and 2006 cohorts. Overall yearly Part B payments per beneficiary increased significantly from $3567 for the 1994 to $5991 for the 2006 cohort (P < .01) in constant 2008 dollars. Payments for eye care alone doubled from $1504 for the 1994 cohort to $3263 for the 2006 cohort (P < .01). Most of the increase in payments for eye care in 2006 reflected payments for anti-VEGF injections, which were $1609 over 1 year. Mean annual numbers of visits and imaging studies also increased significantly between the 1994 and 2006 cohort. Results were similar in the matched sample. CONCLUSIONS: The introduction of anti-VEGF intravitreal injections has offered remarkable clinical benefits for patients with neovascular AMD, but these benefits have come at the cost of an increased financial burden of providing care for these patients.

Ocular complications after anti-vascular endothelial growth factor therapy in Medicare patients with age-related macular degeneration.

PURPOSE: To determine longitudinal rates of ocular complications after anti-vascular endothelial growth factor (VEGF) treatment for neovascular age-related macular degeneration (AMD) in a nationally representative longitudinal sample. DESIGN: Retrospective, longitudinal case-control study. METHODS: Using the Medicare 5% claims database, diagnoses of neovascular AMD and anti-VEGF injections of ranibizumab, bevacizumab, or pegaptanib were identified from International Classification of Diseases and Current Procedural Terminology procedure codes. Six thousand one hundred fifty-four individuals undergoing anti-VEGF treatment for neovascular AMD (total of 40 903 injections) were compared with 6154 matched controls with neovascular AMD who did not undergo anti-VEGF treatment. Propensity score matching was used to match individuals receiving anti-VEGF injections with controls. Rates of postinjection adverse outcomes (endophthalmitis, rhegmatogenous retinal detachment, retinal tear, uveitis, and vitreous hemorrhage) were analyzed by cumulative incidence and Cox proportional hazards model to control for demographic factors and ocular comorbidities. RESULTS: At the 2-year follow-up, the rates of endophthalmitis per injection (0.09%; P<.01), uveitis (0.11%; P<.01), and vitreous hemorrhage per injection (0.23%; P < .01) were significantly higher in the anti-VEGF treatment group. With Cox proportional hazards modeling, the anti-VEGF treatment group had a 102% higher risk of severe ocular complications overall and a 4% increased risk per injection, both of which were statistically significant (P<.01). CONCLUSIONS: Rates of endophthalmitis, uveitis, and vitreous hemorrhage were higher in the group treated with anti-VEGF injection than in the control group, although these nevertheless were rare in both groups. The overall risk of severe ocular complications was significantly higher in the anti-VEGF treatment group.

Cone structure in retinal degeneration associated with mutations in the peripherin/RDS gene.

PURPOSE: To study cone photoreceptor structure and function associated with mutations in the second intradiscal loop region of peripherin/RDS. METHODS: High-resolution macular images were obtained with adaptive optics scanning laser ophthalmoscopy and spectral domain optical coherence tomography in four patients with peripherin/RDS mutations and 27 age-similar healthy subjects. Measures of retinal structure and fundus autofluorescence (AF) were correlated with visual function, including best-corrected visual acuity (BCVA), kinetic and static perimetry, fundus-guided microperimetry, full-field electroretinography (ERG), and multifocal ERG. The coding regions of the peripherin/RDS gene were sequenced in each patient. RESULTS: Heterozygous mutations in peripherin/RDS were predicted to affect protein structure in the second intradiscal domain in each patient (Arg172Trp, Gly208Asp, Pro210Arg and Cys213Tyr). BCVA was at least 20/32 in the study eye of each patient. Diffuse cone-greater-than-rod dysfunction was present in patient 1, while rod-greater-than-cone dysfunction was present in patient 4; macular outer retinal dysfunction was present in all patients. Macular AF was heterogeneous, and the photoreceptor-retinal pigment epithelial (RPE) junction layer showed increased reflectivity at the fovea in all patients except patient 1, who showed cone-rod dystrophy. Cone packing was irregular, and cone spacing was significantly increased (z-scores >2) at most locations throughout the central 4° in each patient. CONCLUSIONS: peripherin/RDS mutations produced diffuse AF abnormalities, disruption of the photoreceptor/RPE junction, and increased cone spacing, consistent with cone loss in the macula. The abnormalities observed suggest that the integrity of the second intradiscal domain of peripherin/RDS is critical for normal macular cone structure.

One-year outcomes after retinal detachment surgery among medicare beneficiaries.

PURPOSE: To determine longitudinal rates of second retinal detachment operation and postoperative adverse outcomes after retinal detachment surgery in a nationally representative sample of older Americans. DESIGN: Retrospective, longitudinal cohort analysis. METHODS: A total of 9216 Medicare beneficiaries were identified from the Medicare 5% sample who were diagnosed with rhegmatogenous retinal detachment and underwent primary pars plana vitrectomy (PPV), scleral buckle, pneumatic retinopexy, or laser photocoagulation or cryotherapy alone. Rhegmatogenous retinal detachment, PPV, scleral buckle, pneumatic retinopexy, or laser photocoagulation/cryotherapy was ascertained from International Classification of Diseases and Current Procedural Terminology procedure codes. Rates of second retinal detachment operation and postoperative adverse outcomes were analyzed by cumulative incidence and logistic regression to control for prior adverse outcome measures and demographic factors. RESULTS: At 1-year follow-up, the rate of receipt of a second retinal detachment operation for beneficiaries who had undergone primary pneumatic retinopexy was much higher (40.6%, P < .0001) relative to the scleral buckle (19.2%) group. After controlling for demographic variables and ocular comorbidities, pneumatic retinopexy individuals were nearly 3 times more likely to receive a second retinal detachment surgery than scleral buckle individuals. No significant differences exist in risk of second retinal detachment surgery for the PPV compared to the scleral buckle group. Individuals receiving PPV were 2 times more likely to suffer adverse outcomes than were those undergoing scleral buckle. Results were robust in sensitivity analysis. CONCLUSIONS: Rates of second operation were much higher after pneumatic retinopexy than PPV or scleral buckle, and rates of adverse outcomes were higher in PPV, even after controlling for risk factors and demographic variables.

Retinopathy of prematurity malpractice claims: the Ophthalmic Mutual Insurance Company experience.

OBJECTIVE: To examine the causes of retinopathy of prematurity (ROP) malpractice claims filed with the Ophthalmic Mutual Insurance Company. METHODS: All closed ROP malpractice claims were reviewed. RESULTS: Eight cases involved failure of transfer of care on patient discharge from the hospital, 3 cases demonstrated inappropriately long periods between follow-up examinations, 1 case was due to failure of outpatient referral from screening to the treating ophthalmologist, and 1 case concerned unsupervised resident provision of ROP care. CONCLUSIONS: Many preventable factors can be addressed to improve ROP care. It is essential to ensure that ophthalmologists, neonatologists, pediatricians, and families are updated on current guidelines for ROP screening and treatment and to facilitate follow-up appointments before patient discharge from the hospital. Doing so can help avoid future malpractice claims and patient harm.

Interactive image-guided endoscopic dacryocystorhinostomy.

The authors describe dacryocystorhinostomy performed with CT guidance. A 53-year-old man with a history of cocaine abuse was referred for bilateral nasolacrimal duct obstruction. Nasal speculum examination revealed an oronasal fistula and obliteration of the inferior nasal septum. Bilaterally, the inferior and middle turbinates were contracted, forming thick scar tissue conglomerates. The loss of normal anatomic landmarks and extensive contracted scar tissue precluded standard dacryocystorhinostomy approaches. Endoscopic dacryocystorhinostomy was therefore undertaken using CT-guided navigation. The location of the superior nasolacrimal duct was identified and subsequently exposed. Dissection was continued superiorly, marsupializing the lacrimal sac. Silicon stents were placed and tied intranasally. Right and left sides were managed similarly. Several months later, following stent removal, the patient was without epiphora. This case demonstrates that CT guidance can be a useful adjunct to standard dacryocystorhinostomy techniques, allowing otherwise challenging surgical cases to be managed effectively and safely.

Spontaneous resolution of a traumatic cataract caused by an intralenticular foreign body.

A 49-year-old man presented with an intralenticular metal foreign body incurred while he was sawing wood. The metal chard had violated the lens capsule and was lodged in the cortex of the lens. It was removed using a lens-preservation technique during open-globe repair. Subsequently, a dense posterior cortical cataract developed, which spontaneously resolved over the ensuing months. The cataract had a cruciate configuration with wave-like disruption of the stromal lamellae. To our knowledge, this is the first case of spontaneous resolution of a cataract after capsule violation by an intralenticular foreign body. The unique appearance of the cataract and its unusual resolution led to a new theory of lens injury by shockwave.

The relationship between second-year medical students' OSCE scores and USMLE Step 2 scores.

RATIONALE, AIMS AND OBJECTIVES: A valid tool to measure clinical competency early in medical school could identify students who may require special educational attention. The overall aim is to assess the relationship between students' scores on an objective structured clinical examination (OSCE) given in the second year of medical school and their subsequent performance on Step 2 of the United States Medical Licensing Examination (USMLE Step 2). METHODS: Participants were 390 second-year medical students participating in a required OSCE; complete data (Medical College Admission Test, OSCE, USMLE Step 1 and Step 2 scores) were available for 340 students (87%). Univariate correlations and linear regression analyses were performed. RESULTS: Total OSCE score was moderately correlated with USMLE Step 2 score (r = 0.395, P < 0.001), as were two skills subscores of the OSCE, differential diagnosis (r = 0.343, P < 0.001) and identification of abnormality (r = 0.322, P < 0.001). In linear regression analysis, neither OSCE total score nor any of the subscores independently predicted Step 2 scores; only Step 1 score (beta = 0.687, P < 0.001) and female sex (beta = 0.152, P < 0.001) remained independent correlates of Step 2 score. CONCLUSION: OSCEs early in medical school can be useful in the early assessment of clinical competence.