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OBJECTIVE: This study evaluates the 24 month follow up for the NICHD Neonatal Research Network (NRN) Inositol for Retinopathy Trial. STUDY DESIGN: Bayley Scales of Infants Development-III and a standardized neurosensory examination were performed in infants enrolled in the main trial. Moderate/severe NDI was defined as BSID-III Cognitive or Motor composite score

Niacin, a treatment for dyslipidemia, is known to induce vasodilation as a secondary effect. Previous instances of patients with chronic central retinal vein occlusion (CRVO) and cystoid macular edema (CME) have been observed to spontaneously improve when placed on systemic niacin for hypercholesterolemia. The purpose of this study was to evaluate the effects of niacin on CRVO and associated ocular complications. A prospective, single-center, non-randomized, interventional case series of niacin for CRVO was conducted. Best-correct visual acuity (BCVA), central macular thickness (CMT), and ocular complications were analyzed in 50 patients over 1 year. Eight patients were controls. The mean initial logMAR BCVA was 0.915, and improved with niacin to 0.745 (P = 0.12), 0.665 (P = 0.02) and 0.658 (P = 0.03) after 3, 6, and 12 months of follow-up, respectively. At baseline, mean CMT was 678.9 μm, and improved to 478.1 μm (P = 0.001), 388.6 μm (P

The risk of developing treatment-warranted Type 1 retinopathy of prematurity (ROP) might be reduced in preterm infants by modifying certain systemic factors. There are steps that can be taken both early and late in the course of retinal vascular maturation that may potentially reduce an infant's risk of developing Type 1 ROP. In prethreshold stage 2-3 ROP without plus disease, a combination of supplemental oxygen, correction of severe anemia, and light adaptation to reduce rod photoreceptor oxygen consumption helped us to reduce ROP severity, and encouraged a return to a more physiologic retinal vascular maturation pattern. Thus, it may be possible to reduce the risk of developing Type 1 ROP by making adjustments in certain systemic parameters aimed at reducing retinal hypoxia, thereby gently lowering pathologically elevated levels of vascular endothelial growth factor (VEGF) within the eye.

The natural course of high-axial myopia is variable and the development of pathologic myopia is not fully understood. Advancements in optical coherence tomography (OCT) technology have revealed peculiar intraocular structures in highly myopic eyes and unprecedented pathologies that cause visual impairment. New OCT findings include posterior precortical vitreous pocket and precursor stages of posterior vitreous detachment; peripapillary intrachoroidal cavitation; morphological patterns of scleral inner curvature and dome-shaped macula. Swept source OCT is capable of imaging deeper layers in the posterior pole for investigation of optic nerve pits, stretched and thinned lamina cribrosa, elongated dural attachment at posterior scleral canal, and enlargement of retrobulbar subarachnoid spaces. This has therefore enabled further evaluation of various visual field defects in high myopia and the pathogenesis of glaucomatous optic neuropathy. OCT has many potential clinical uses in managing visual impairing conditions in pathologic myopia. Understanding how retinal nerve fibers are redistributed in axial elongation will allow the development of auto-segmentation software for diagnosis and monitoring progression of glaucoma. OCT is indispensable in the diagnosis of various conditions associated with myopic traction maculopathy and monitoring of post-surgical outcomes. In addition, OCT is commonly used in the multimodal imaging assessment of myopic choroidal neovascularization. Biometry and topography of the retinal layers and choroid will soon be validated for the classification of myopic maculopathy for utilization in epidemiological studies as well as clinical trials.

IMPORTANCE: Previous studies of myo-inositol in preterm infants with respiratory distress found reduced severity of retinopathy of prematurity (ROP) and less frequent ROP, death, and intraventricular hemorrhage. However, no large trials have tested its efficacy or safety. OBJECTIVE: To test the adverse events and efficacy of myo-inositol to reduce type 1 ROP among infants younger than 28 weeks’ gestational age. DESIGN, SETTING, AND PARTICIPANTS: Randomized clinical trial included 638 infants younger than 28 weeks’ gestational age enrolled from 18 neonatal intensive care centers throughout the United States from April 17, 2014, to September 4, 2015; final date of follow-up was February 12, 2016. The planned enrollment of 1760 participants would permit detection of an absolute reduction in death or type 1 ROP of 7% with 90% power. The trial was terminated early due to a statistically significantly higher mortality rate in the myo-inositol group. INTERVENTIONS: A 40-mg/kg dose of myo-inositol was given every 12 hours (initially intravenously, then enterally when feeding; n = 317) or placebo (n = 321) for up to 10 weeks. MAIN OUTCOMES AND MEASURES: Type 1 ROP or death before determination of ROP outcome was designated as unfavorable. The designated favorable outcome was survival without type 1 ROP. RESULTS: Among 638 infants (mean, 26 weeks’ gestational age; 50% male), 632 (99%) received the trial drug or placebo and 589 (92%) had a study outcome. Death or type 1 ROP occurred more often in the myo-inositol group vs the placebo group (29% vs 21%, respectively; adjusted risk difference, 7% [95% CI, 0%-13%]; adjusted relative risk, 1.41 [95% CI, 1.08-1.83], P = .01). All-cause death before 55 weeks’ postmenstrual age occurred in 18% of the myo-inositol group and in 11% of the placebo group (adjusted risk difference, 6% [95% CI, 0%-11%]; adjusted relative risk, 1.66 [95% CI, 1.14-2.43], P = .007). The most common serious adverse events up to 7 days of receiving the ending dose were necrotizing enterocolitis (6% for myo-inositol vs 4% for placebo), poor perfusion or hypotension (7% vs 4%, respectively), intraventricular hemorrhage (10% vs 9%), systemic infection (16% vs 11%), and respiratory distress (15% vs 13%). CONCLUSIONS AND RELEVANCE: Among premature infants younger than 28 weeks’ gestational age, treatment with myo-inositol for up to 10 weeks did not reduce the risk of type 1 ROP or death vs placebo. These findings do not support the use of myo-inositol among premature infants; however, the early termination of the trial limits definitive conclusions.

Purpose: The purpose of this study was to report a case of an impending central retinal artery occlusion with hypoperfusion in a moyamoya patient. Methods: A young, surgically revascularized moyamoya patient experienced severe unilateral vision loss from 20/25 to hand motions because of impending central retinal artery occlusion. The patient was treated with a combination of intermittent ocular massage, intraocular pressure–lowering medications, and aspirin. Patients: A case of a moyamoya patient at Stanford University Medical Center. Results: Visual acuity was restored to baseline by improving the ocular arterial–venous gradient after prompt administration of ocular massage, intraocular pressure–lowering drops, and aspirin. Conclusion: This dramatic result suggests that, if performed in a timely manner, augmentation of ocular perfusion can result in complete restoration of vision in some cases of incipient central retinal artery occlusion.

The authors present the first case of peripheral avascular retina in a term male neonate with pancreatic exocrine insufficiency, atypical microvillus inclusion disease, flat tympanograms, and recurrent urinary tract infections. Clinical examination showed avascular peripheral retina to posterior zone II temporally, with a flat stage 1-like demarcation line, and no plus disease. Genetic testing results were normal. The patient developed peripheral neovascularization and underwent panretinal photocoagulation. This case likely represents mild Norrie disease, familial exudative vitreoretinopathy, or incontinentia pigmenti due to a Wnt signaling abnormality. While these conditions are usually more severe, a variable spectrum of Wnt abnormalities exists throughout the body. [ Ophthalmic Surg Lasers Imaging Retina . 2015;46:589–591.]

Purpose Hairy cell leukemia (HCL) is a rare disorder that occasionally has visual symptoms after diagnosis. The authors present a case of HCL in which bilateral visual symptoms led to the initial diagnosis. Methods Observational case report. Results Bilateral decreased vision to 20/30 in the right eye and 20/40 in the left due to intraretinal and pre-retinal hemorrhages with no other systemic signs or symptoms prompted a hematologic evaluation in which HCL was found to be the causative disorder in a previously healthy 41-year-old man. Conclusions The authors present a rare case in which bilateral visual complaints led to the diagnosis of HCL. Thus, though uncommon, HCL should be considered in the differential diagnosis of otherwise unexplained retinal hemorrhages.

Purpose: To review systemic modifiable factors that might downregulate pathologic levels of vascular endothelial growth factor (VEGF) in prethreshold retinopathy of prematurity (ROP), and thereby reduce the risk of blindness in affected infants. Methods: Review of the author's clinical experience as compared to the STOP-ROP study, and discussion and literature review of the potential effects of oxygen supplementation, light adaptation state, and correction of severe anemia on the course of prethreshold retinopathy of prematurity (ROP). Results: Moderate oxygen supplementation, combined with light adaptation to reduce retinal oxygen consumption and correction of severe anemia, were associated, in the author's experience, with improvement in the clinical course of prethreshold ROP. Withdrawal of these measures was often associated with deterioration of ROP. Conclusion: Systemic measures can be taken that may reduce the risk of blindness in infants with prethreshold ROP. For those infants who require laser treatment for threshold ROP, prior efforts to moderate excessive levels of VEGF may improve the chances of a successful surgical result, by slowing the momentum of the disease.

Objective: To determine whether earlier treatment of high-risk, prethreshold retinopathy of prematurity (ROP) improves retinal structural outcome at 2 years of age. Methods: Infants with bilateral high-risk prethreshold ROP had one eye randomly assigned to treatment with peripheral retinal ablation. The fellow eye was managed conventionally, and either treated at threshold ROP or observed if threshold was never reached. In patients with asymmetrical disease, the high-risk, prethreshold eye was randomised to earlier treatment or to conventional management. At 2 years of age, children were examined comprehensively by certified ophthalmologists to determine structural outcomes for their eyes. For the purposes of this study, an unfavourable structural outcome was defined as (1) a posterior retinal fold involving the macula, (2) a retinal detachment involving the macula or (3) retrolental tissue or "mass" obscuring the view of the posterior pole. Results of the 2-year examination were compared with those from the 9 months examination. Results: Data were available on 339 of 374 (90.6%) surviving children. Unfavourable structural outcomes were reduced from 15.4% in conventionally managed eyes to 9.1% in earlier-treated eyes (p = 0.002) at 2 years of age. Ophthalmic side effects (excluding retinal structure) from the ROP or its treatment were similar in the earlier-treated eyes and the conventionally managed eyes. Conclusion: The benefit of earlier treatment of high-risk prethreshold ROP on retinal structure endures to 2 years of age, and is not counterbalanced by any known side effect caused by earlier intervention. Earlier treatment improves the chance for long-term favourable retinal structural outcome in eyes with high-risk prethreshold ROP. Long-term follow-up is planned to determine structural and functional outcomes at 6 years of age., link_to_OA_fulltext

Purpose The purpose of this study was to report a case of a chorioretinal coloboma mimicking a second optic disk in an asymptomatic 12-year-old boy. Methods This is a case report. Results An asymptomatic 12-year-old boy presented with a chorioretinal coloboma of the right eye giving the impression of a second optic disk. The patient also had a superior visual field defect. Conclusion True optic nerve duplication is a rare entity that can be mimicked by other etiologies, including choroidal colobomas and postinflammatory lesions, requiring careful examination by clinicians.

Objective To report an unusual case of diffuse retinal hemorrhage associated with strangulation in a neonate with a tightly wrapped nuchal cord around his neck at birth who was noted to have eyelid and subconjunctival petechial hemorrhages upon delivery. Methods Clinical diagnostic examination as well as postmortem gross and histopathologic examination of ocular and central nervous system structures was performed. Results Funduscopic and gross pathologic examination of the eyes revealed extensive unilateral retinal hemorrhage of the right eye only. This was corroborated by histopathologic studies, which revealed unilateral diffuse hemorrhage throughout all nuclear layers of the retina with a particularly dense nerve fiber layer and sub-internal limiting membrane hemorrhage in the macula along with extraocular muscle and episcleral hemorrhage. Clinical, gross, and microscopic examination also revealed multiple areas of hemorrhage involving the right side of the brain and throughout the cerebellum. Conclusion Although retinal findings in nonaccidental trauma are common, accidental strangulation retinopathy in neonates is a rare occurrence. To the authors' knowledge, this case is the only documented histopathologic study of hemorrhagic retinopathy associated with strangulation in the literature.

The results of the STOP-ROP study, published elsewhere in this month's issue,1suggest that the effect of supplemental oxygen on the conversion rate of prethreshold retinopathy of prematurity (ROP) to threshold disease may be less pronounced than that found in 2 previous studies, one by Gaynon et al2 at Stanford and the other by Seiberth et al3 in Germany. For the STOP-ROP group as a whole, the reduction in the rate of conversion from prethreshold ROP to threshold was from 48.5% for the conventional oxygen group down to 40.9% for the supplemental oxygen group. STOP-ROP did show a trend toward better outcome, which for the group as a whole, did not reach statistical significance, given the ultimate sample size of 649 infants, down from the initially planned 880 infant enrollment. Results were more dramatically beneficial for those infants without plus disease. Taking this group as a whole, the conversion rate to threshold dropped from 46% for the conventional group to 32% for the supplemental group. This is consistent with the notion that plus disease (tortuous and engorged retinal vessels) is a sign of high blood flow through arteriovenous shunting attributable to intraretinal (stage 2+) or extraretinal (stage 3+) neovascularization. Lack of, or resolution of, plus disease is a favorable sign suggesting that any neovascularization present, as a component of prethreshold ROP, may be resolving. The time it took for threshold disease to appear was longer for the supplemental group as compared with the conventional group, both for the study as a whole as well as for patient subsets stratified according to disease severity, suggesting a moderating effect of supplemental oxygen on disease momentum. At the same time there was evidence that to some extent, chronic lung disease was worse in those infants in STOP-ROP given supplemental oxygen …

This 25-year-old woman went bungee jumping from 150 ft (46 m). Immediately afterward, she noticed a substantial decrease in vision in her left eye.

Retinal neovascularization, a rare complication of ocular toxoplasmosis, is a source of vitreous hemorrhage. We examined three patients (all women, 70, 36, and 19 years old) with inactive toxoplasmosis scars associated with occlusion of a retinal arteriole or venule passing through the scar, capillary nonperfusion, and adjacent retinal neovascularization. In two patients photocoagulation of the ischemic areas produced a cessation of hemorrhage and the disappearance of neovascularization. In the third case, the neovascular frond was avulsed from its supplying retinal arteriole without treatment.

We examined two children with presumed shaken baby syndrome. Both children suffered severe, indirect closed head trauma with intracranial hemorrhage, sharply increased intracranial pressure, and extensive neurologic damage. In addition to extensive retinal and preretinal hemorrhages, bilateral symmetric white ring-shaped retinal folds were seen encircling the macula outside the vascular arcades. These retinal folds may be a hallmark of shaking injuries in child abuse victims.

• A further subdivision of functions during vitrectomy surgery has been enabled. By means of a separate, suturesecured fiberoptic endoilluminator and a modified infusion cannula located in the inferior quadrants, two superior sclerotomies are kept free for the use of a variety of simple and complex instruments, alone or in combination. Included among these are vitreous scissors, forceps, picks, spatulas, diathermy probes, and endophotocoagulators.

Clinical neurophysiologists may be asked to participate in the evaluation of patients with injured eyes. We describe a method for eliciting evoked potentials of cerebral origin using electrical pulse stimuli delivered to the globe of the eye through a contact lens electrode mounted on the cornea. These visual electrical evoked potentials (VEEPs) are contrasted with conventional flash visual evoked potentials in normal subjects and in 19 eyes of 17 patients with severe ocular damage, mostly recent trauma. The findings suggest that the site of transcorneal electrical excitation is not the photoreceptors, but more likely one of the nerve cell layers of the retina. VEEP recordings offer a way to circumvent the opacification of the ocular media by blood, which may otherwise hamper the evaluation of retinal function in the injured eye. Preserved VEEP response does not necessarily predict the capacity to recover visual function. Absent VEEP response is an unfavorable prognostic sign, which may be considered in arriving at a decision about enucleation.

• Macular photocoagulation requires precise localization of abnormalities to be treated, in order to accomplish the aims of therapy without unnecessarily damaging adjacent tissue. A method is described by which a color fundus photo or a fluorescein image can be projected through a beam splitter into the ocular of a laser slit lamp, thus affording an image congruent with the surgeon's view of the patient's macula.

Four patients, including three with the acquired immunodeficiency syndrome (AIDS), were treated with high-dose, buffy coat-derived alpha-interferon for progressive cytomegalovirus retinitis. Two of these patients had decreased viruria during therapy and the other two had increased viruria. There was evidence of progression of disease despite therapy in all patients, although the retinitis eventually became quiescent in the patient without AIDS. The severe immunosuppression encountered in AIDS patients complicates the management of cytomegalovirus and other opportunistic infections.

A 56-year-old man sustained an intraocular injury by a piece of steel followed by endophthalmitis, which resolved after lensectomy, vitrectomy, and intravitreal injections of gentamicin. Acinetobacter anitratus was the organism responsible for the endophthalmitis. It is a Gram-negative polymorphic organism that can resemble several other pathogens and possesses unpredictable antibiotic susceptibility. A. anitratus is an important cause of nosocomial infection but has not previously been reported as a cause of endophthalmitis following trauma.