Retinal vascular stasis associated with closed funnel stage 5 retinopathy of prematurity.
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Biomedical subjects
Publications and source records attributed to Michael T Trese.
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PURPOSE: To determine indications and document anatomic and functional outcomes for lens-sparing vitreous surgery in eyes with predominantly posterior persistent fetal vasculature syndrome and nonaxial lens opacification. STUDY DESIGN: Retrospective interventional clinical series. METHODS: The authors performed lens-sparing vitreous surgery on nine eyes of infants with predominantly posterior persistent fetal vasculature syndrome and nonaxial lens opacification without removing the crystalline lens. Indications for surgery included primarily posterior complications of persistent fetal vasculature syndrome (localized tractional retinal detachment), a centrally clear lenticular axis, and frequently strabismus. RESULTS: Postoperatively, seven of nine eyes showed no evidence of increasing lens opacification with a median follow-up period of 16.8 months (range, 5-29 months). The first eye operated on developed a rapidly progressive cataract and prompted modification of the surgical technique. One additional eye also required cataract extraction for progressive lenticular opacification 23 months after surgery. No eyes developed postoperative retinal detachment, and all eyes maintained vision at the final follow-up. Of the seven eyes with strabismus, five improved after vitreous surgery alone, and two required additional strabismus surgery. CONCLUSIONS: This series shows that, in selected cases of persistent fetal vasculature syndrome that are predominantly posterior in nature with nonaxial lens opacification, lens-sparing vitrectomy can be of benefit.
Familial exudative vitreoretinopathy (FEVR) is a hereditary ocular disorder characterized by a failure of peripheral retinal vascularization. Loci associated with FEVR map to 11q13-q23 (EVR1; OMIM 133780, ref. 1), Xp11.4 (EVR2; OMIM 305390, ref. 2) and 11p13-12 (EVR3; OMIM 605750, ref. 3). Here we have confirmed linkage to the 11q13-23 locus for autosomal dominant FEVR in one large multigenerational family and refined the disease locus to a genomic region spanning 1.55 Mb. Mutations in FZD4, encoding the putative Wnt receptor frizzled-4, segregated completely with affected individuals in the family and were detected in affected individuals from an additional unrelated family, but not in normal controls. FZD genes encode Wnt receptors, which are implicated in development and carcinogenesis. Injection of wildtype and mutated FZD4 into Xenopus laevis embryos revealed that wildtype, but not mutant, frizzled-4 activated calcium/calmodulin-dependent protein kinase II (CAMKII) and protein kinase C (PKC), components of the Wnt/Ca(2+) signaling pathway. In one of the mutants, altered subcellular trafficking led to defective signaling. These findings support a function for frizzled-4 in retinal angiogenesis and establish the first association between a Wnt receptor and human disease.
BACKGROUND: The Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) demonstrated the efficacy of treatment for threshold ROP and indicated the need for worldwide ROP screening. Previous guidelines for ROP screening have been largely based on clinical impression; we can now develop evidence-based screening recommendations. OBJECTIVE: To define the appropriate ages and retinal ophthalmoscopic signs that determine when to commence and conclude acute phase ROP screening. DESIGN: Analysis of data from 2 prospective randomized controlled trials: CRYO-ROP (January 1, 1986, to November 30, 1987) and Light Reduction in ROP (LIGHT-ROP) (July 1, 1995, to March 31, 1997). SETTING: Neonatal intensive care units in 23 study centers in the United States for CRYO-ROP and 3 centers for LIGHT-ROP. PATIENTS: Eyes were examined sequentially in 4099 infants with birth weight less than 1251 g (CRYO-ROP study) and in 361 infants with birth weight less than 1251 g and gestational age less than 31 weeks (LIGHT-ROP study). RESULTS: In 99% of infants, retinal conditions indicating a risk of poor outcome were not observed before 31 weeks' postmenstrual age or 4 weeks' chronologic age. Signs indicating that the risk of visual loss from ROP was minimal or had passed were the infant's attainment of 45 weeks' postmenstrual age without the development of prethreshold ROP or worse, progression of retinal vascularization into zone III without previous zone II ROP, and full vascularization. CONCLUSIONS: The initial eye examination should be conducted by 31 weeks' postmenstrual age or 4 weeks' chronologic age, whichever is later. Acute phase ROP screening can be discontinued when any of the 3 signs is present, indicating that the risk of visual loss from ROP is minimal or passed.
PURPOSE: To report a case of presumed endogenous bilateral Candida endophthalmitis that developed in an infant with bilateral posterior stage 3 retinopathy of prematurity. DESIGN: Interventional case report. METHODS: In a very low birth weight and extremely premature infant, presumed endogenous bilateral Candida endophthalmitis was successfully eradicated from each eye and retinopathy of prematurity was managed. RESULTS: Candida endophthalmitis was successfully eradicated from each eye by vitrectomy with instillation of 5 microg amphotericin B. Stage 3 retinopathy of prematurity without "plus disease" in the right eye regressed without ablation of the avascular peripheral retina. Stage 3 retinopathy with "plus disease" in the left eye was successfully managed with endolaser photocoagulation. CONCLUSIONS: Concomitant Candida endophthalmitis and posterior stage 3 retinopathy of prematurity often portend a dismal prognosis. We describe an infant for whom prompt diagnosis and aggressive therapy of both conditions resulted in a favorable anatomic outcome in both eyes.
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