Longitudinal Changes in Vision and Retinal Morphology in Wolfram Syndrome.

Purpose: To report long-term ophthalmic findings in Wolfram syndrome, including rates of visual decline, macular thinning, retinal nerve fiber layer (RNFL) thinning, and outer plexiform layer (OPL) lamination.

Design: Single-center, cohort study.

Methods: A total of 38 participants were studied, who underwent a complete ophthalmic examination as well as optical coherence tomography imaging of the macula and nerve on an annual basis.

SUBHYALOID HEMORRHAGE IN EVANS SYNDROME.

Purpose: Evans syndrome is a rare disorder characterized by autoimmune hemolytic anemia and immune thrombocytopenia. We report the first case of ophthalmic involvement in a pediatric patient with Evans syndrome, in which painless vision loss was the presenting symptom.

Method: A 15-year-old girl presented with acute painless loss of vision in her right eye and was found to have bilateral subhyaloid hemorrhages.

ENDOGENOUS ENDOPHTHALMITIS CAUSED BY GROUP B STREPTOCOCCUS IN A HEALTHY, TERM NEONATE.

Purpose: The purpose of this study was to describe an unusual case of unilateral, endogenous endophthalmitis in an otherwise healthy, term neonate.

Methods: A 3-week-old otherwise healthy, term male infant was referred to St. Louis Children's Hospital for a second opinion of presumed panuveitis of the right eye.

Anterior Chamber Angles after Intravitreal Injections for Macular Degeneration.

The mechanism underlying IOP elevation associated with intravitreal injections is unknown. Using anterior segment OCT, we found no significant differences in angle width associated with number of intravitreal injections that might explain this phenomenon.

Identification of Retinal Vascular Lesions Using Ultra-Widefield Angiography in Hereditary Hemorrhagic Telangiectasia Patients.

Purpose: To determine the presence and to characterize location of retinal vascular lesions in patients with hereditary hemorrhagic telangiectasia (HHT).

Design: Prospective cross-sectional pilot descriptive study.

Participants: Eighteen patients (age range, 22-65 years) with a clinical diagnosis of HHT.

Successful treatment of an exudative choroidal hemangioma with oral propranolol in a 10-year-old boy.

A 10-year-old boy was referred for a circumscribed choroidal hemangioma with underlying exudative detachment of the left eye. To avoid general anesthetics required for laser-based therapy in a child, we began a trial of oral propranolol. The patient's exudative detachment resolved, with resulting improvement in visual acuity, and remained quiescent for 3 years.

Association of Preclinical Alzheimer Disease With Optical Coherence Tomographic Angiography Findings.

Importance: Biomarker testing for asymptomatic, preclinical Alzheimer disease (AD) is invasive and expensive. Optical coherence tomographic angiography (OCTA) is a noninvasive technique that allows analysis of retinal and microvascular anatomy, which is altered in early-stage AD.

Objective: To determine whether OCTA can detect early retinal alterations in cognitively normal study participants with preclinical AD diagnosed by criterion standard biomarker testing.

BILATERAL CHOROIDAL DETACHMENTS SECONDARY TO IPILIMUMAB AND PEMBROLIZUMAB USE.

Purpose: To report the occurrence of bilateral choroidal detachments due to the use of ipilimumab and pembrolizumab immunochemotherapeutics to treat widely metastatic cutaneous melanoma and to raise awareness about this potentially vision-threatening adverse drug event.

Methods: A 77 year-old man presented with acute onset, painless, and bilateral blurry vision. He had started ipilimumab and pembrolizumab 2 weeks prior for Stage IV metastatic cutaneous melanoma.

Cytokine profiles in clinical subtypes of ophthalmic Graves' disease.

Purpose: To examine the association of cytokines in the two clinical subtypes of ophthalmic Graves' disease by comparing cytokine expression in the fat and ethmoid tissue of type I and type II patients.

Methods: Patients needing orbital decompression or eyelid surgery were identified and enrolled into a prospective study. Patients were assigned to the type I or type II subclassification, based on the presence of diplopia.

Honokiol inhibits pathological retinal neovascularization in oxygen-induced retinopathy mouse model.

Aberrant activation of the hypoxia inducible factor (HIF) pathway is the underlying cause of retinal neovascularization, one of the most common causes of blindness worldwide. The HIF pathway also plays critical roles during tumor angiogenesis and cancer stem cell transformation. We have recently shown that honokiol is a potent inhibitor of the HIF pathway in a number of cancer and retinal pigment epithelial cell lines.

The genetic control of avascular area in mouse oxygen-induced retinopathy.

Purpose: The C57BL/6ByJ and BALB/cByJ inbred strains of mice are, respectively, susceptible and resistant to oxygen-induced retinopathy (OIR). The purpose of this work was to investigate the genetic control of the retinal avascular area in mouse OIR using a mapping cross.

Methods: The central retinal avascular area was measured on postnatal day 16 (P16) in C57BL/6ByJ, BALB/cByJ, 101 (C57BL/6ByJ x BALB/cByJ)F₂, and 116 (BALB/cByJ x C57BL/6ByJ)F₂ mice that had been subjected to the OIR protocol.

Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome.

In this study we evaluated the relationship between amphetamine-induced behavioral alterations and dopamine release and uptake characteristics in Fmr1 knockout (Fmr1 KO) mice, which model fragile X syndrome. The behavioral analyses, obtained at millisecond temporal resolution and 2 mm spatial resolution using a force-plate actometer, revealed that Fmr1 KO mice express a lower degree of focused stereotypy compared to wild type (WT) control mice after injection with 10 mg/kg (ip) amphetamine. To identify potentially related neurochemical mechanisms underlying this phenomenon, we measured electrically-evoked dopamine release and uptake using fast-scan cyclic voltammetry at carbon-fiber microelectrodes in striatal brain slices.