1/7. Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy.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.- - - - - - - - - - ranking = 1keywords = vitreoretinopathy (Clic here for more details about this article) |
2/7. genetic linkage of autosomal dominant neovascular inflammatory vitreoretinopathy to chromosome 11q13.Autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) is an inherited eye disease characterized by retinal and iris neovascularization, abnormal retinal pigmentation, anterior chamber and vitreous inflammation, cystoid macular edema, vitreous hemorrhage, and traction retinal detachment. Some of these clinical features are shared by more common, potentially blinding, conditions including diabetic retinopathy, uveitis, and retinitis pigmentosa. Elucidation of the molecular pathogenesis of ADNIV has the potential to provide insight into the mechanisms of these common disorders. One hundred and sixteen members of an eight generation family affected with ADNIV were examined. A combination of slit lamp biomicroscopy, ophthalmoscopy, and electroretinography was used to establish the diagnosis and 34 family members were found to be affected. blood samples were obtained from thirty-three of these individuals and nine spouses and used for chromosome linkage analysis with denaturing gradient gel and short tandem repeat polymorphisms. Two markers that map to chromosome 11q13 were found to be significantly linked to the ADNIV phenotype. There were no recombinants between the disease phenotype and marker D11S527 and multipoint analysis yielded a maximum lod score of 11.9 centered on this marker.- - - - - - - - - - ranking = 1keywords = vitreoretinopathy (Clic here for more details about this article) |
3/7. iris neovascularization in proliferative vitreoretinopathy.PURPOSE: The purpose of this study is to report on the prevalence, incidence, and associated risk factors of iris neovascularization in nondiabetic patients undergoing vitrectomy for retinal detachment complicated by proliferative vitreoretinopathy (PVR). methods: The authors conducted a retrospective review of 141 consecutive non-diabetic patients undergoing vitrectomy for recurrent retinal detachment resulting from PVR. Univariate and multivariate analyses were performed on all patients to determine which preoperative, intraoperative, and postoperative factors were associated with the development of postoperative iris neovascularization. RESULTS: Twenty-seven of the 141 (19%) patients were noted with preoperative and/or postoperative iris neovascularization. Four of eight patients presenting with preoperative iris neovascularization had complete regression after successful reattachment of the retina. Results of analysis of the remaining 133 patients without iris neovascularization preoperatively showed residual retinal detachment as the most significant risk factor for postoperative iris neovascularization. In the absence of panretinal photocoagulation, none of the 27 patients developed neovascular glaucoma. CONCLUSIONS: The development of iris neovascularization preoperatively or post-operatively is not necessarily a predictor of a poor anatomic and/or visual result. iris neovascularization in PVR rarely if ever progresses to neovascular glaucoma. Panretinal photocoagulation is not indicated in these patients. Retinal reattachment is the most important factor in the prevention and/or resolution of postoperative iris neovascularization. The development of iris neovascularization in PVR appears to be a multifactorial process requiring multiple variables acting in concert.- - - - - - - - - - ranking = 1keywords = vitreoretinopathy (Clic here for more details about this article) |
4/7. Optic disc neovascularization in dominant exudative vitreoretinopathy.Dominant exudative vitreoretinopathy (DEVR) is inherited retinal vascular disorder which is characterized by peripheral retinal neovascularization, tractional retinal detachment, subretinal, exudation, and vitreous fibrosis. A milder form of involvement characterized by capillary nonperfusion and straightening of the retinal vessels may be present in asymptomatic individuals. We describe a 15-year-old patient who presented with optic disc neovascularization, a finding not previously reported in association with DEVR. Angiographic evidence of DEVR in successive generations confirmed the diagnosis. The findings in mild and advanced DEVR are reviewed, and the pathogenesis of the capillary nonperfusion is discussed.- - - - - - - - - - ranking = 1keywords = vitreoretinopathy (Clic here for more details about this article) |
5/7. Foveal translocation: a comparison of two techniques.Subfoveal choroidal neovascular membrane is a leading cause of legal blindness. Photocoagulation has been effective, but photocoagulation of the fovea causes a decrease in vision immediately after treatment. Surgical removal of the choroidal neovascular membrane is effective for choroidal neovascular membrane in some cases, but it restores useful vision for reading (20/40 or better) in a small number of cases of choroidal neovascular membrane not due to presumed ocular histoplasmosis syndrome. A new treatment for subfoveal choroidal neovascular membrane, foveal translocation, is an innovative procedure in which the fovea is translocated onto healthier retinal pigment epithelium. Three techniques have been developed to relocate the retina, 2 of which (retinotomy and scleral shortening) we performed in 5 cases each. Preliminary results in these 10 cases indicate that foveal translocation provides improvement of visual acuity in 40% of eyes and final visual acuities useful for reading (better than 20/40) in 20% of eyes undergoing translocation with either technique. Further study is essential to refine the amount of translocation needed and to decrease complications of the 2 techniques, including retinal detachment, proliferative vitreoretinopathy, macular pucker, corneal astigmatism, and constricted visual field.- - - - - - - - - - ranking = 0.2keywords = vitreoretinopathy (Clic here for more details about this article) |
6/7. Translocation of the retina for management of subfoveal choroidal neovascularization II: a preliminary report in humans.PURPOSE: To report a surgical method for translocation of the foveal retina in eyes with subfoveal choroidal neovascularization. methods: In three eyes of three patients, a crescent-shaped, partial-thickness scleral resection was performed near the equator at either the superotemporal or the inferotemporal quadrant. A near-total retinal detachment was created; then the edges of the resected sclera were sutured, causing shortening of the sclera with subsequent reattachment of the retina, resulting in translocation of the fovea to an area overlying nonfoveal retinal pigment epithelium and choroid. RESULTS: In three eyes of three patients, the fovea was surgically translocated to overlie retinal pigment epithelium that preoperatively was not underlying the fovea. In two patients, laser photocoagulation was applied to the choroidal neovascularization that, after translocation of the fovea, was no longer subfoveal, so that the photocoagulation was not associated with immediate visual loss. After a follow-up of 4 to 6 months, the visual acuity had improved in all patients (from 20/126 preoperatively to 20/70 in one patient, from 20/200 preoperatively to 20/70 in the second, and from 20/160 to 20/30 in the third). The patients noted distortion or tilting of the images, which improved over time. CONCLUSIONS: Limited foveal translocation may offer a therapeutic modality to preserve or improve vision in cases of subfoveal choroidal neovascularization. Additional follow-up is needed to assess the impact of potential complications associated with the surgical procedure, such as retinal detachment, proliferative vitreoretinopathy, and cataract, as well as the possibility of recurrent choroidal neovascularization.- - - - - - - - - - ranking = 0.2keywords = vitreoretinopathy (Clic here for more details about this article) |
7/7. Neovascular complications associated with rubeosis iridis and peripheral retinal detachment after retinal detachment surgery.PURPOSE: To report clinical features and surgical management of neovascular complications associated with rubeosis iridis and peripheral retinal detachment after retinal detachment surgery in nondiabetic patients. methods: Seven consecutive eyes of seven nondiabetic patients who developed neovascular complications associated with rubeosis iridis and peripheral retinal detachment after scleral buckling and vitrectomy procedures were retrospectively reviewed. None of the eyes had clinical evidence of anterior segment ischemia or retinal vascular disease, but each eye developed rubeosis iridis and neovascular complications. RESULTS: Of the seven eyes with rubeosis iridis and peripheral retinal detachment, six developed recurrent or progressive vitreous hemorrhage, and three developed progressive neovascular glaucoma. Four eyes underwent a revision procedure to repair the peripheral retinal detachment, and anterior proliferative vitreoretinopathy was found in each of these cases. Rubeosis iridis regressed in all three eyes in which surgery resulted in complete reattachment of the retina. In one eye with persistent peripheral retinal detachment and in the three remaining eyes that did not undergo revision surgery, rubeosis iridis persisted and was associated with long-term neovascular complications. Final corrected visual acuity was 20/70 to 20/400 in three eyes with total retinal reattachment and no light perception to hand motions in four eyes with persistent peripheral retinal detachment and rubeosis iridis. CONCLUSION: Visually significant neovascular complications may occur in eyes that develop rubeosis iridis associated with peripheral retinal detachment after retinal detachment surgery in nondiabetic patients. Successful repair of the peripheral retinal detachment may induce regression of rubeosis iridis, reduce associated complications, and improve the long-term prognosis of these eyes.- - - - - - - - - - ranking = 0.2keywords = vitreoretinopathy (Clic here for more details about this article) |