for the BEAT-ROP Cooperative Group IMPORTANCE Children born prematurely who develop retinopathy of prematurity (ROP) often develop myopia, and those who require laser treatment may develop very high myopia, which has considerable clinical consequences.OBJECTIVE To report refractive outcomes in preterm infants who developed ROP in zone I or zone II posterior as stage 3+ ROP or aggressive posterior ROP (APROP). DESIGN, SETTING, AND PARTICIPANTSAll infants received intravitreal bevacizumab or laser therapy in a prospective, stratified, randomized, controlled, masked, multicenter clinical trial, Bevacizumab Eliminates the Angiogenic Threat for ROP (BEAT-ROP). Children who received intravitreal bevacizumab or laser in the BEAT-ROP clinical trial, with treatment randomized by infant, underwent cycloplegic retinoscopic refraction at a mean age of 2½ years. Fifteen centers with both pediatric and vitreoretinal ophthalmologists participating in level 3 neonatal intensive care units in academic centers with institutional review board approval were included in the trial. Of the originally enrolled 150 infants (300 eyes) in the BEAT-ROP clinical trial, 13 infants (26 eyes) died (6 received intravitreal bevacizumab; 7 received laser) and 19 eyes had intraocular surgery (6 infants bilaterally). Thus, 45 eyes (19 infants bilaterally) were excluded, leaving 131 infants (255 eyes, including 21 eyes that received a successful second treatment for recurrence).INTERVENTIONS Follow-up of the BEAT-ROP cohort. MAIN OUTCOMES AND MEASURES Spherical equivalent refractive outcomes and their distribution by ROP zone and treatment.RESULTS Refractions were available for 109 of 131 eligible infants (83.2%) and 211 of 255 eyes (82.7%). Mean (SD) spherical equivalent refractions were as follows: zone I, −1.51 (3.42) diopters (D) in 52 eyes that received intravitreal bevacizumab and −8.44 (7.57) D in 35 eyes that received laser treatment (P < .001); and zone II posterior, −0.58 (2.53) D in 58 eyes that received intravitreal bevacizumab and −5.83 (5.87) D in 66 eyes that received laser treatment (P < .001). Very high myopia (Ն−8.00 D) occurred in zone I in 2 of 52 (3.8%) eyes that received intravitreal bevacizumab and in 18 of 35 (51.4%) eyes that received laser treatment (P < .001). Very high myopia occurred in zone II posterior in 1 of 58 (1.7%) eyes that received intravitreal bevacizumab and in 24 of 66 (36.4%) eyes that received laser treatment (P < .001).CONCLUSIONS AND RELEVANCE More very high myopia was found in eyes that received laser treatment than in eyes that received intravitreal bevacizumab. This difference is possibly related to anterior segment development that is present with intravitreal bevacizumab but minimal or absent following laser treatment. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00622726

Aim To describe our experience in surgical management of stages 4 and 5 retinopathy of prematurity (ROP)

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Choroidal granulomas related to systemic sarcoidosis respond well to oral corticosteroids. They may recur but good vision can be maintained. They are not typically associated with concomitant iritis and also do not appear to be associated with intracranial granulomas.

The Wnt pathway plays important yet diverse roles in health and disease. Mutations in the Wnt receptor FZD4 gene have been confirmed to cause familial exudative vitreoretinopathy (FEVR). FEVR is characterized by incomplete vascularization of the peripheral retina, which can lead to vitreous bleeding, tractional retinal detachment, and blindness. We screened for mutations in the FZD4 gene in five families with FEVR and identified five mutations (C45Y, Y58C, W226X, C204R, and W496X), including three novel mutations (C45Y, Y58C, and W226X). In the retina, Norrin serves as a ligand and binds to FZD4 to activate the Wnt signaling pathway in normal angiogenesis and vascularization. The cysteine-rich domain (CRD) of FZD4 has been shown to play a critical role in Norrin-FZD4 binding. We investigated the effect of mutations in the FZD4 CRD in Norrin binding and signaling in vitro and in vivo. Wild-type and mutant FZD4 proteins were assayed for Norrin binding and Norrin-dependent activation of the canonical Wnt pathway by cell-surface and overlay binding assays and luciferase reporter assays. In HEK293 transfection studies, C45Y, Y58C, and C204R mutants did not bind to Norrin and failed to transduce FZD4-mediated Wnt/␤-catenin signaling. In vivo studies using Xenopus embryos showed that these FZD4 mutations disrupt Norrin/␤-catenin signaling as evidenced by decreased Siamois and Xnr3 expression. This study identified a new class of FZD4 gene mutations in human disease and demonstrates a critical role of the CRD in Norrin binding and activation of the ␤-catenin pathway.Pathological growth of new blood vessels in the retinal vasculature (neovascularization) has been implicated in several human diseases, including familial exudative vitreoretinopathy (FEVR), 3 retinopathy of prematurity, age-related macular degeneration, and diabetic retinopathy. Complications of neovascularization include bleeding, retinal detachment, and irreversible scarring of the retina. FEVR is a developmental disorder characterized by incomplete vascularization of the peripheral retina (1-3). Mutations in FZD4 (Frizzled 4) have been linked to autosomal dominant forms of FEVR (4). The primary effect of FZD4 mutations is caused by the premature arrest of retinal angiogenesis within the peripheral retina (5). Complications arising from incomplete retinal vascularization and subsequent retinal ischemia include development of hyperpermeable vessels, neovascularization, bleeding, and tractional retinal detachment. In severely affected patients with FZD4 mutations, blindness may occur before 10 years of age, whereas mildly affected individuals may not be aware of symptoms and are diagnosed only by fluorescein angiography.Activation of the canonical Wnt pathway in the retina has been shown to be developmental stage-dependent and spatially modulated and is also important in retinal regeneration (6, 7). FZD4 is a member of the Frizzled family of seven-transmembrane Wnt-binding receptors. The N-terminal extracellular cysteine-rich domain (CRD), conserved among F...

Vitreoretinal complications occurred in 6.8% of patients undergoing therapy for retinoblastoma. These included retinal tears, rhegmatogenous and tractional retinal detachment, subretinal fibrosis, vitreous traction bands, preretinal fibrosis, and pseudo-vitreous seeding. They were more often seen when systemic chemotherapy was combined with external beam radiation, cryotherapy, and local chemotherapy.

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