Development of a rapid point-of-care patient reported outcome measure for cataract surgery in India.

BACKGROUND: For patient undergoing cataract surgery in India, existing patient-reported outcome (PRO) measures are either not culturally relevant, have not been adequately validated, or are too long to be used in a busy clinical setting. We sought to develop and validate a brief and culturally relevant point-of-care PRO measure to address this need. METHODS: Twelve items from the Indian Visual Functioning Questionnaire (IND-VFQ) were selected based on preliminary data. Patients 18 years and older were prospectively recruited at Aravind Eye Care System in Madurai, India. Clinical and sociodemographic data were collected and the 12-item short-form IND-VFQ (SF-IND-VFQ) was administered pre- and post-operatively to 225 patients; Factor analysis and Rasch modeling was performed to assess its psychometric properties. RESULTS: One item that did not fit a unidimensional scale and had poor fit with the Rasch model was eliminated from the questionnaire. The remaining 11 items represented a single construct (no residual correlations> 0.1) and were largely unaffected by differential item functioning. Five items had disordered thresholds resolved by collapsing the response scale from four to three categories. The survey had adequate reliability (0.80) and good construct (infit range, 0.77-1.29; outfit range, 0.56-1.30) and content (item separation index, 5.87 logits) validity. Measurement precision was fair (person separation index, 1.97). There was evidence that items were not optimally targeted to patients' visual ability (preoperatively, - 1.92 logits; overall, - 3.41 logits), though the survey measured a very large effect (Cohen's d 1.80). In a subset of patients, the average time to complete the questionnaire was 2 min 6.3 s. CONCLUSIONS: The SF-IND-VFQ is a valid, reliable, sensitive, and rapidly administered point-of-care PRO measure to assess changes in visual functioning in patients undergoing cataract surgery in India.

PROSE Treatment for Ocular Chronic Graft-Versus-Host Disease as a Clinical Network Expands.

BACKGROUND: Keratoconjunctivitis sicca occurs in 40% to 90% of patients with ocular chronic graft-versus-host disease (cGVHD). Ocular symptoms can have profound effects in both the visual function and quality of life of patients with GVHD. We report the impact of prosthetic replacement of the ocular surface ecosystem (PROSE) treatment in patients with cGVHD as a clinical network expands. METHODS: We queried the BostonSight PROSE manufacturing database from January 2002 to December 2011. Patients treated for ocular cGVHD were reported by age, gender, year, and network site where the treatment was undertaken. The baseline and six-month follow-up scores of visual function using a standardized validated instrument, the National Eye Institute Visual Function Questionnaire (NEI VFQ-25), were evaluated for a period in 2006 and again in 2010 after network expansion had occurred. RESULTS: A total of 407 patients with a male:female ratio of 226:181, mean age was 51 years with ocular cGVHD underwent PROSE treatment from January 2002 to December 2011. By 2011, 67% of all cases were treated at network clinics. Baseline characteristics of patients treated throughout the network in 2010 were similar to that of 2006 and 2010 cohorts from the main center. There was a significant improvement of 41 points (P<0.001) in composite NEI VFQ score among patients treated across the network in 2010, similar to the improvement of 30 points (P<0.001) seen among the patients treated at the main center in 2010. There was a trend toward lower baseline self-reported general health status (SRGHS) and VFQ scores among patients treated at network clinics, suggesting that expansion of the network allows treatment of sicker patients (lower general health status) or those more severely affected by ocular cGVHD. CONCLUSIONS: PROSE treatment of ocular cGVHD has increased in the last decade with the establishment of BostonSight network clinics across the United States. Patients treated at network clinics showed similar levels of baseline visual function and SRGHS, and achieved a similar high level of improvement in visual function as those treated at the main center. Patient-reported measures of functional status are useful in evaluating treatment options for patients with cGVHD. PROSE treatment has significant positive impact on the visual function of patients with ocular cGVHD regardless of whether the patient is treated at the main center or at a network site.

Prosthetic replacement of the ocular surface ecosystem as treatment for ocular surface disease in patients with a history of Stevens-Johnson syndrome/toxic epidermal necrolysis.

PURPOSE: To report the visual outcomes of prosthetic replacement of the ocular surface ecosystem (PROSE) treatment in patients with ocular surface disease related to Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). DESIGN: Retrospective cohort study. SUBJECTS: We included 86 patients (167 eyes) with history of SJS/TEN who underwent PROSE treatment from January 1, 2006, to January 1, 2011. METHODS: Etiology, previous interventions, change in visual acuity, change in visual function, and duration of follow-up are reported. Paired t test and Friedman test with Dunn's post hoc test for multiple comparisons were used for statistical analysis. MAIN OUTCOME MEASURES: Visual acuity at last follow-up and visual function based on the National Eye Institute 25-item Visual Functioning Questionnaire (NEI VFQ-25) at 6 months. RESULTS: We treated 35 males and 51 females with a history of SJS/TENS; median age was 36 years. The most common reported etiologies for SJS/TENS were antibiotics (n = 25), ibuprofen (n = 15), and lamotrigine (n = 11). The median visual acuity at the initial visit was 20/60 (range, 20/400-20/25; 0.48 logarithm of the minimum angle of resolution [logMAR]), and the visual acuity at completion of customization was 20/25 (range, 20/200-20/20; 0.096 logMAR; P < 0.001), with no decline in median acuity at the end of follow-up. Median duration of follow-up was 16 months. There was a significant improvement in the visual function of the patients based on the NEI VFQ-25 questionnaire (mean of 48 points at baseline vs. mean of 72 points at 6 months; P < 0.001). In addition, there was also an improvement in the self-reported general health of the patients (mean of 57 points at baseline vs. mean of 65 points at 6 months; P < 0.01). CONCLUSIONS: In a large cohort of patients with chronic ocular surface disease related to SJS/TEN, PROSE treatment offers sustained and significant large improvement in visual function and acuity.

Early retinoic acid deprivation in developing zebrafish results in microphthalmia.

Vitamin A deficiency causes impaired vision and blindness in millions of children around the world. Previous studies in zebrafish have demonstrated that retinoic acid (RA), the acid form of vitamin A, plays a vital role in early eye development. The objective of this study was to describe the effects of early RA deficiency by treating zebrafish with diethylaminobenzaldehyde (DEAB), a potent inhibitor of the enzyme retinaldehyde dehydrogenase (RALDH) that converts retinal to RA. Zebrafish embryos were treated for 2 h beginning at 9 h postfertilization. Gross morphology and retinal development were examined at regular intervals for 5 days after treatment. The optokinetic reflex (OKR) test, visual background adaptation (VBA) test, and the electroretinogram (ERG) were performed to assess visual function and behavior. Early treatment of zebrafish embryos with 100 µM DEAB (9 h) resulted in reduced eye size, and this microphthalmia persisted through larval development. Retinal histology revealed that DEAB eyes had significant developmental abnormalities but had relatively normal retinal lamination by 5.5 days postfertilization. However, the fish showed neither an OKR nor a VBA response. Further, the retina did not respond to light as measured by the ERG. We conclude that early deficiency of RA during eye development causes microphthalmia as well as other visual defects, and that timing of the RA deficiency is critical to the developmental outcome.

The effect of optic asphericity on visual rehabilitation of corneal ectasia with a prosthetic device.

OBJECTIVES: To study the effect of optic asphericity on visual rehabilitation of corneal ectasia with a prosthetic device. METHODS: Subjects with corneal ectasia treated with a fluid-ventilated gas-permeable prosthetic device of diameter 18.0-19.0 mm, who had subjective improvement of good Snellen vision with introduction of optic asphericity, were studied. Best corrected Snellen visual acuity (BCVA) under standard illumination, high contrast visual acuity (HCVA), low contrast visual acuity (LCVA), and wavefront aberrations were measured in a sequence of devices that varied per patient only in presence or amount of ellipsoidal front surface optical eccentricity (FSE). RESULTS: Five eyes of 5 subjects were studied. (M:F = 3:2; Age: 20-76). Mean steepest SimK was 57.72±8.30 D. BCVA was ≥20/30 in all eyes in all prosthetic devices, regardless of FSE. Although FSE improved BCVA, HCVA, and LCVA in each patient, no optimal amount could be identified in this small series. Asphericity in the form of 0.6 or 0.8 FSE improved HCVA, LCVA, or both in each patient. FSE was associated with a trend toward reduction of higher-order aberrations, particularly coma. CONCLUSIONS: Optic asphericity shows promise for optimization of vision in the rehabilitation of corneal ectasia with a prosthetic device.

Diabetic and Retinal Vascular Eye Disease.

The retinal vasculature is the only neurovascular system directly visible to the human eye, easily evaluated by fundoscopy and many imaging modalities. This window allows physicians to diagnose and treat retinal pathologies and detect systemic diseases including diabetes, hypertension, hypercoagulable/hyperviscosity syndromes, and vasculitis. Diabetic retinopathy is the most common retinal vascular disease, followed by retinal vein and artery occlusion. Patients with these conditions require medical optimization to prevent further damage to the eyes and to the other organs. Both the internists and medical subspecialists play a crucial role in the prevention, detection, evaluation, and management of vision-threatening retinal vascular diseases.

Longitudinal Changes on Optical Coherence Tomography Angiography in Retinal Vein Occlusion.

BACKGROUND: To evaluate the longitudinal changes on optical coherence tomography angiography (OCTA) in retinal vein occlusion (RVO). METHODS: Retrospective study of patients with RVO treated with intravitreal anti-vascular endothelial growth factors (VEGF) for macular edema. Foveal avascular zone (FAZ) area, vessel density (VD), vessel length density (VLD), and adjusted flow index (AFI) were calculated. The unaffected eye of each participant was used as a control. RESULTS: Twelve RVO eyes were included, receiving 6 ± 3 anti-VEGF injections over a follow-up of 10.4 ± 3.1 months. Compared to fellow eyes, RVO eyes had lower VD and VLD at inclusion (p = 0.07 and p = 0.04) and last visit (p = 0.002 and p < 0.001). VD, AFI, and VLD did not change over time, while FAZ area increased in RVO eyes (+0.016 ± 0.024 mm2, p = 0.04). AFI correlated with duration of disease (r = 0.63, p = 0.02). Visual acuity was inversely related to VD and VLD over the follow-up. CONCLUSIONS: OCTA parameters remained stable with sustained anti-VEGF treatment in RVO, while changes in the FAZ area may suggest capillary remodeling after RVO.

Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman.

PURPOSE: To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. OBSERVATIONS: Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. CONCLUSIONS AND IMPORTANCE: Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology.

Stevens-Johnson Syndrome and Corneal Ectasia: Management and a Case for Association.

PURPOSE: To report the occurrence of corneal ectasia (ECT) in patients with history of Stevens-Johnson syndrome (SJS), and to make the case for an association between these 2 diagnoses. We also report the impact of prosthetic replacement of the ocular surface ecosystem (PROSE) treatment on visual acuity (VA) in these patients. DESIGN: Retrospective cohort study. METHODS: A manufacturing database of PROSE patients from 2002 to 2014 at Boston Foundation for Sight (BFS), a single-center clinical practice, was reviewed to identify patients with diagnoses of both SJS and ECT. RESULTS: Nine patients were identified with diagnoses of both SJS and ECT. In each case, review of the medical record revealed that diagnosis of SJS preceded that of ECT. The prevalence of ECT in this population exceeded that in the general population (P < .0001). Videokeratography was available for 13 eyes in 7 patients; using Krumeich's classification of keratoconus, 3 eyes were found to be at stage 1, 3 at stage 2, 1 at stage 3, and 6 at stage 4. Sixteen of 18 eyes underwent PROSE treatment. Of these 16 eyes, initial median VA was 20/200 (range, count fingers to 20/20; logMAR 1.0). Median VA after PROSE customization was 20/30 (range, 20/60-20/15; logMAR 0.1761, P < .0025). CONCLUSIONS: ECT occurs at a higher-than-expected rate in patients with a history of SJS. PROSE treatment improves VA in these patients. The basis of the association between SJS and ECT is considered, as well as the role of plausible contributory factors such as corneal microtrauma and matrix metalloproteinases.

Burn unit care of Stevens Johnson syndrome/toxic epidermal necrolysis: A survey.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a systemic disease that can be associated with debilitating acute and chronic complications across multiple organ systems. As patients with acute SJS/TEN are often treated in a burn intensive care unit (BICU), we surveyed burn centers across the United States to determine their approach to the care of these patients. The goal of our study was to identify best practices and possible variations in the care of patients with acute SJS/TEN. We demonstrate that the method of diagnosis, use of systemic therapies, and involvement of subspecialists varied significantly between burn centers. Beyond supportive care provided to every patient, our data highlights a lack of standardization in the acute care of patients with SJS/TEN. A comprehensive guideline for the care of patients with acute SJS/TEN is indicated.

A Sustainable Model For Delivering High-Quality, Efficient Cataract Surgery In Southern India.

Cataracts are a leading cause of reversible blindness in India, where millions of people can be effectively treated for this condition with surgery. The Aravind Eye Care System in southern India developed an efficient system for delivering high-quality and low-cost cataract surgery. We provide a detailed accounting of costs of cataract surgery at the system and a cost-utility analysis. Total costs per operation were US$120, or $195 per quality-adjusted life-year gained. Using these data and population-based estimates of cataract prevalence, we calculate that eliminating cataract-related blindness and low vision in India would cost $2.6 billion and would yield a net societal benefit of $13.5 billion. Factors contributing to the highly cost-effective care at the Aravind Eye Care System include the domestic manufacturing of supplies, the use of a specialized workforce and standardized protocols, and the presence of few regulatory hurdles. Lessons learned from the system can help improve the delivery of cataract surgery and other ambulatory care surgeries in India and abroad.

Pilot Study for OCT Guided Design and Fit of a Prosthetic Device for Treatment of Corneal Disease.

Purpose. To assess optical coherence tomography (OCT) for guiding design and fit of a prosthetic device for corneal disease. Methods. A prototype time domain OCT scanner was used to image the anterior segment of patients fitted with large diameter (18.5-20 mm) prosthetic devices for corneal disease. OCT images were processed and analyzed to characterize corneal diameter, corneal sagittal height, scleral sagittal height, scleral toricity, and alignment of device. Within-subject variance of OCT-measured parameters was evaluated. OCT-measured parameters were compared with device parameters for each eye fitted. OCT image correspondence with ocular alignment and clinical fit was assessed. Results. Six eyes in 5 patients were studied. OCT measurement of corneal diameter (coefficient of variation, CV = 0.76%), cornea sagittal height (CV = 2.06%), and scleral sagittal height (CV = 3.39%) is highly repeatable within each subject. OCT image-derived measurements reveal strong correlation between corneal sagittal height and device corneal height (r = 0.975) and modest correlation between scleral and on-eye device toricity (r = 0.581). Qualitative assessment of a fitted device on OCT montages reveals correspondence with slit lamp images and clinical assessment of fit. Conclusions. OCT imaging of the anterior segment is suitable for custom design and fit of large diameter (18.5-20 mm) prosthetic devices used in the treatment of corneal disease.

PROSE treatment of corneal ectasia.

PURPOSE: Prosthetic replacement of the ocular surface ecosystem (PROSE) uses custom designed and fabricated prosthetic devices in a treatment that restores vision, supports healing, reduces symptoms and improves quality of life in patients with complex corneal disease. We report the success rate for PROSE treatment of corneal ectasia. METHODS: Records of 59 patients with corneal ectasia seen in consultation over 6 months were reviewed. Candidacy for treatment, topographic indices, change in visual acuity, achievement of satisfactory fit, device wear status and change in visual function at 6 months were recorded. RESULTS: Sixteen eyes were non-candidates because conventional correction was adequate. Trial devices were inserted but not dispensed for 13 eyes. No eyes were excluded for severity of ectasia. In the remaining 89 eyes, satisfactory fit was achieved and a device was dispensed. Twenty-one eyes (15 patients) had undergone penetrating keratoplasty. Device wear at 6 months was documented in 78/89 eyes (88%). NEI VFQ-25 score improved 27.6 points (p<0.001) on a 100 point scale in patients wearing a device at 6 months. CONCLUSION: All candidate eyes with corneal ectasia could be fitted with a PROSE device. PROSE treatment has a high success rate when measured by ability to achieve satisfactory fit, impact on visual acuity and 6 month data on both rate of continued wear and impact on visual function. PROSE treatment is an alternative to penetrating keratoplasty for patients with corneal ectasia who are contact lens intolerant.