Splenial Restricted Diffusion as MRI Correlate of Diaschisis in a Blind Infant With Unilateral Posterior Cerebral Artery Stroke.

ABSTRACT: A 3-month-old male infant appeared on multiple clinical examinations to have acutely developed bilateral retrogeniculate blindness. Electroencephalography showed focal status epilepticus confined to the left posterior cerebral hemisphere. MRI demonstrated restricted diffusion in the domain of the left posterior cerebral artery consistent with acute stroke. Notably, the restricted diffusion extended across the midline in the splenium of the corpus callosum. This splenial sign may be the imaging correlate of cerebral diaschisis, a well-described phenomenon in which patients with new brain lesions develop acutely impaired neurologic function in related but nonlesioned brain regions. Diaschisis has been posited as the explanation for the temporary bilateral blindness in adult patients suffering from unilateral occipital infarctions.

Treatment of Congenital Ptosis in Infants With Associated Amblyopia Using a Frontalis Muscle Flap Eyelid Reanimation Technique.

PURPOSE: To determine the efficacy of a frontalis muscle flap eyelid reanimation technique for correction of severe congenital ptosis and associated amblyopia in infants. METHODS: The authors performed a retrospective chart review of patients 12 months of age or younger with unilateral or bilateral congenital ptosis and associated amblyopia or deemed at high risk for amblyopia due to visual deprivation. Following ptosis repair via a frontalis muscle flap technique, primary outcomes of postoperative eyelid position and amblyopia reversal were assessed. RESULTS: Seventeen eyes of 12 participants were included for study. Seven of these patients had simple congenital ptosis, and the remainder had ptosis as part of a syndrome. Nine were diagnosed with amblyopia preoperatively, and the remaining 3 were too young for acuity testing but had occlusion of the visual axis by the ptotic eyelid in primary gaze. Postoperatively, the mean margin-to-reflex distance 1 was 2.4 mm (range: 0.0-4.0), and 9 patients (75%) demonstrated no evidence of amblyopia. Only 2 patients had eyelid asymmetry greater than 2 mm, which in both cases was due to lack of frontalis activation by the patient secondary to ongoing visual impairment. The most common complication was lagophthalmos in 6 eyes (35.3%), with no significant associated surface keratopathy. CONCLUSIONS: The frontalis muscle flap technique may offer a new and effective approach to treating infants with severe congenital ptosis causing poor eyelid excursion and associated amblyopia while avoiding use of an implant.

Autoimmune retinopathy associated with monoclonal gammopathy of undetermined significance: a case report.

BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell dyscrasia and precursor to multiple myeloma. It has known ocular manifestations, but has not previously been shown to have an association with autoimmune retinopathy. CASE PRESENTATION: A 57 year-old female presented with 1 year of progressive, bilateral, peripheral vision loss, photopsias, and nyctalopia. Her fundus examination and extensive ancillary testing were concerning for hereditary versus autoimmune retinopathy. The patient was found to have anti-retinal antibodies against carbonic anhydrase II and enolase proteins with a negative genetic retinal dystrophy panel. Malignancy work-up was negative, but the patient was diagnosed with MGUS, a premalignant condition. The patient was treated with immunosuppressive therapies, with rituximab demonstrating the most robust therapeutic response with respect to patient symptoms and ophthalmic testing. CONCLUSIONS: MGUS should be considered as a potential etiology of autoimmune retinopathy in patients without other autoimmune or malignant disease processes. Immunosuppressive therapy may be helpful in limiting disease progression, with rituximab showing efficacy in retinopathy refractory to other agents.

RHEGMATOGENOUS RETINAL DETACHMENT AFTER INITIATION OF PILOCARPINE HYDROCHLORIDE OPHTHALMIC SOLUTION 1.25% FOR TREATMENT OF PRESBYOPIA.

BACKGROUND/PURPOSE: Retinal detachment has previously been reported in association with topical miotic use for the treatment of glaucoma. Pilocarpine hydrochloride 1.25% was recently approved by the Food and Drug Administration for the treatment of presbyopia, with no reports of associated retinal detachments in the clinical trial data. METHODS: Case report. RESULTS: Two novel cases of unilateral retinal detachment occurring within 10 days of the initiation of pilocarpine 1.25% for the treatment of presbyopia were described. The patients were pseudophakic men in their 60s or 70s with preexisting retinal detachment risk factors, such as high myopia, lattice degeneration, and prior retinal detachment. Both affected eyes were treated with pars plana vitrectomy and gas endotamponade with an uncomplicated postoperative course. CONCLUSION: Retinal detachment may be associated with the use of pilocarpine 1.25%. Caution should be used when considering prescribing this medication in patients with preexisting retinal abnormality.

Exudative Retinal Detachment and Ciliochoroidal Effusion in Preeclampsia.

Purpose: To describe a novel case of focal exudative retinal detachment, choroidal effusion, and acute angle closure in preeclampsia. Methods: A case report is presented. Results: A 37-year-old woman at 38 weeks gestation presented with 2 weeks of progressive blurred vision in the left eye. She had a visual acuity (VA) of 20/800 and an intraocular pressure (IOP) of 26 mm Hg in the left eye (compared with 17 mm Hg in the right eye). Examination showed subretinal fluid in the posterior pole, ciliochoroidal effusion, and angle closure in the left eye without pathology in the right eye. She was found to have hypertension and proteinuria consistent with preeclampsia. The visual symptoms resolved after delivery. At the 1-month follow-up, she had a VA of 20/60 OS, symmetric IOPs, and resolved subretinal and choroidal effusions. Conclusions: To our knowledge, this is the first reported case of ciliochoroidal effusion in the setting of preeclampsia. It may aid in the diagnosis of preeclampsia's ocular manifestations and broaden pathophysiological understanding.

Association of metformin and development of dry age-related macular degeneration in a U.S. insurance claims database.

PURPOSE: To assess whether metformin is associated with dry age-related macular degeneration (dAMD) development. METHODS: In this retrospective cohort study, patients enrolled in a nationwide U.S. medical insurance claims database from 2002 to 2016 were included if they had diabetes mellitus, were ⩾55 years old, and were enrolled for ⩾2 years without a prior AMD diagnosis. The primary exposure was metformin use analyzed as either active or prior use or cumulative metformin dosage over the study period. A time updating Cox proportional hazard regression was used to estimate the hazard ratio of dAMD incidence with metformin exposure. RESULTS: Among 1,007,226 diabetic enrollees, 53.3% were female and 66.4% were white with a mean hemoglobin A1c of 6.8%. Of eligible enrollees, 166,115 (16.5%) were taking metformin at the index date. Over the study period, 29,818 (3.0%) participants developed dAMD. In the active versus prior use of metformin model, active use conferred an increased hazard of developing dAMD (HR, 1.08; 95% CI, 1.04-1.12) while prior use had a decreased hazard (HR, 0.95; 95% CI 0.92-0.98). The cumulative metformin dosage model showed a significant trend toward increased hazard of dAMD incidence with increasing cumulative dosage (p < 0.001), with the lowest dosage quartile having decreased hazard of dAMD incidence (HR, 0.95; 95% CI, 0.91-0.99) and the highest having increased hazard (HR, 1.07; 95% CI, 1.01-1.13). CONCLUSIONS: Small, conflicting associations between metformin exposure and development of dAMD were observed depending on cumulative dosage and whether drug use was active, suggesting metformin did not substantially affect the development of dAMD.

Analysis of residual disease in periocular basal cell carcinoma following hedgehog pathway inhibition: Follow up to the VISORB trial.

Basal cell carcinoma (BCC) is a common skin cancer caused by deregulated hedgehog signaling. BCC is often curable surgically; however, for orbital and periocular BCCs (opBCC), surgical excision may put visual function at risk. Our recent clinical trial highlighted the utility of vismodegib for preserving visual organs in opBCC patients: 67% of patients displayed a complete response histologically. However, further analysis of excision samples uncovered keratin positive, hedgehog active (Gli1 positive), proliferative micro-tumors. Sequencing of pre-treatment tumors revealed resistance conferring mutations present at low frequency. In addition, one patient with a low-frequency SMO W535L mutation recurred two years post study despite no clinical evidence of residual disease. Sequencing of this recurrent tumor revealed an enrichment for the SMO W535L mutation, revealing that vismodegib treatment enriched for resistant cells undetectable by traditional histology. In the age of targeted therapies, linking molecular genetic analysis to prospective clinical trials may be necessary to provide mechanistic understanding of clinical outcomes. Trial Registration: ClinicalTrials.gov Identifier: NCT02436408.

Healthcare disparities contribute to missed follow-up visits after cataract surgery in the USA: results from the perioperative care for intraocular lens study.

OBJECTIVE: To identify factors that contribute to missed cataract surgery follow-up visits, with an emphasis on socioeconomic and demographic factors. METHODS: In this retrospective cohort study, patients who underwent cataract extraction by phacoemulsification at Massachusetts Eye and Ear between 1 January and 31 December 2014 were reviewed. Second eye cases, remote and international patients, patients with foreign insurance and combined cataract cases were excluded. RESULTS: A total of 1931 cases were reviewed and 1089 cases, corresponding to 3267 scheduled postoperative visits, were included. Of these visits, 157 (4.8%) were missed. Three (0.3%) postoperative day 1, 40 (3.7%) postoperative week 1 and 114 (10.5%) postoperative month 1 visits were missed. Age<30 years (adjusted OR (aOR)=8.2, 95% CI 1.9 to 35.2) and ≥90 years (aOR=5.7, 95% CI 2.0 to 15.6) compared with patients aged 70-79 years, estimated travel time of >2 hours (aOR=3.2, 95% CI 1.4 to 7.4), smokers (aOR=2.7, 95% CI 1.6 to 4.8) and complications identified up to the postoperative visit (aOR=1.4, 95% CI 1.0 to 2.1) predicted a higher rate of missed visits. Ocular comorbidities (aOR=0.7, 95% CI 0.5 to 1.0) and previous visit best-corrected visual acuity (BCVA) of 20/50-20/80 (aOR=0.4, 95% CI 0.3 to 0.7) and 20/90-20/200 (aOR=0.4, 95% CI 0.2 to 0.9), compared with BCVA at the previous visit of 20/40 or better, predicted a lower rate of missed visits. Gender, race/ethnicity, language, education, income, insurance, alcohol use and season of the year were not associated with missed visits. CONCLUSIONS: Medical factors and demographic characteristics, including patient age and distance from the hospital, are associated with missed follow-up visits in cataract surgery. Additional studies are needed to identify disparities in cataract postoperative care that are population-specific. This information can contribute to the implementation of policies and interventions for addressing them.

Association of Ocular Antihypertensive Medications and the Development and Progression of Age-related Macular Degeneration in a U.S. Insurance Claims Database.

Purpose/Aim: To assess whether ocular antihypertensives are associated with the development and progression of age-related macular degeneration (AMD).Materials and Methods: This retrospective, observational cohort study using healthcare claims data from a U.S. nationwide managed-care network between January 1, 2006 and December 31, 2016, included enrollees ≥40 years old with primary open-angle glaucoma with or without a diagnosis of nonexudative AMD at the index date. Hazard ratios (HR) for developing AMD or progressing from nonexudative to exudative AMD with exposure to ocular antihypertensive medications were analyzed.Results: Of 132 963 eligible enrollees, 118 174 (87.5%) had no diagnosis of AMD at baseline while 14 789 (12.5%) had adiagnosis of nonexudative AMD. Prostaglandin analog exposure had adecreased hazard of developing AMD among individuals without baseline disease (HR, 0.90; 95% CI, 0.87-0.94; p< .0001), while topical alpha2-agonist exposure demonstrated an increased hazard of AMD development (HR, 1.08; 95% CI, 1.03-1.14; p= .004). Among patients with baseline nonexudative AMD, topical carbonic anhydrase inhibitor exposure was associated with adecreased hazard of progressing to exudative disease (HR, 0.84; 95% CI, 0.71-0.99; p= .04) while topical alpha2-agonists had increased hazard (HR, 1.17; 95% CI, 1.01-1.36; p= .04).Conclusions: Certain ocular antihypertensive medications may be associated with development or progression of AMD. Their role in AMD pathogenesis should be better understood as they are considered for therapeutics in this disease.

Incidence of Management Changes at the Postoperative Week 1 Visit after Cataract Surgery: Results from the Perioperative Care for IntraOcular Lens Study.

PURPOSE: To ascertain the incidence of unexpected management changes at the postoperative week 1 visit in asymptomatic patients who have had an uncomplicated cataract surgery and a routine postoperative day 1 examination. DESIGN: Retrospective observational study. METHODS: A retrospective chart review was conducted of all cases of cataract extraction by phacoemulsification with intraocular lens insertion performed by the Comprehensive Ophthalmology Service at Massachusetts Eye and Ear between January 1, 2014 and December 31, 2014. The preoperative consultation, operative report, and postoperative day 1 and week 1 (postoperative days 5-14) visits were reviewed. Cases with intraoperative complications, as well as clinical findings at postoperative day 1 requiring close follow-up, were excluded. The main outcome measure was incidence of unexpected management changes at the postoperative week 1 visit after cataract surgery, defined as an unanticipated change in postoperative drops, additional procedures, or urgent referral to a specialty service. RESULTS: Overall, 1938 surgical cases of 1471 patients were reviewed, and 1510 cases (77.9%) underwent uncomplicated phacoemulsification with intraocular lens implantation with a routine postoperative day 1 examination. Of these 1510 cases, 238 (15.8%) reported symptoms at the postoperative week 1 visit, including flashes, floaters, redness, pain, or decreased vision, which warranted an examination. In total, 1272 cases were asymptomatic, and only 11 of these cases (0.9%) had an unexpected management change at postoperative week 1. Eight of 11 patients were asymptomatic steroid responders requiring alteration of their postoperative drops. Two of these patients had an intraocular pressure >30 mm Hg. CONCLUSIONS: Unexpected management changes at the postoperative week 1 timepoint after cataract surgery are rare in asymptomatic patients who have had uncomplicated cataract surgery and a routine postoperative day 1 examination. Limited data are available to outline an optimal postoperative regimen after cataract surgery. The results of this study suggest that postoperative week 1 examinations could potentially be performed on an as-needed basis in the appropriate subgroup of patients after cataract surgery.

Outcomes in resident-performed cataract surgeries with iris challenges: Results from the Perioperative Care for Intraocular Lens study.

PURPOSE: To assess the outcomes of resident-performed cataract surgeries with iris challenges and to compare these outcomes with similar surgeries performed by attending surgeons. SETTING: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA. DESIGN: Retrospective chart review. METHODS: All cases of cataract extraction by phacoemulsification with intraocular lens implantation, performed by comprehensive ophthalmologists between January 1 and December 31, 2014, were reviewed. Cases with preoperative or intraoperative miosis, iris prolapse, and intraoperative floppy iris syndrome, were included for analysis. Visual outcomes and the rate of perioperative adverse events were compared between resident and attending surgeon cases. Factors predicting adverse events were also assessed. RESULTS: In total, 1931 eye cases of 1434 patients were reviewed, and 65 resident cases and 168 attending surgeon cases were included. The mean logarithm of the minimum angle of resolution corrected distance visual acuity was better in the resident group 1 month after surgery (0.051 ± 0.10 [SD] versus 0.132 ± 0.30, P = .03); however, the difference was eliminated when controlling for macular disease. The mean operative time was 43.8 ± 26.5 minutes and 30.9 ± 12.6 minutes for cases performed by resident surgeons and attending surgeons, respectively (P  .0001). Residents utilized supplemental pharmacologic dilation or retraction more frequently than attending surgeons (98% versus 87% of cases, P = .008). The overall rate of adverse events was no different between residents and attending surgeons (P = 0.16). Dense nuclear sclerosis predicted adverse events in cataract cases with iris challenges (adjusted odds ratio, 1.86; 95% confidence interval, 1.17-2.94; P = .001). CONCLUSION: Although requiring longer operative times and more surgical manipulation, residents who performed cataract surgeries with iris challenges achieved outcomes comparable to those performed by attending surgeons, and residents should be given the opportunity to operate on these eyes.