Phenotypes for disease #02391 (EVR4 (vitreoretinopathy, exudative, type 4 (EVR4)), OMIM:601813)

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AscendingPhenotype ID     

Phenotype details     

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0000308393 visual acuity: fix-and-follow, minimal in the right eye; intraocular pressure: 18 mm Hg both eyes; no iris or lens abnormality; funduscopy: right eye: extensive exudative maculopathy with shallow retinal detachment and peripheral telengiectatic retinal vessels extending temporally; left fundus: clinically normal; fluorescein angiography: right eye: temporal retinal dragging, extensive peripheral retinal non-perfusion, telangiectatic vessels, left eye: minimal peripheral non-perfusion and vascular looping without dragging, telangiectasia, or leakage; right xanthocoria and slight exotropia; subclinically affected father - familial exudative vitreoretinopathy Familial, autosomal dominant 1y8m 1y8m - xanthocoria in the right eye, with eventual exotropia - LOVD 00416881
0000308394 fundus: mild signs of FEVR: hyperacute retinal vascular branching and slight retinal vascular traction - familial exudative vitreoretinopathy Familial, autosomal dominant - - - asymptomatic - LOVD 00416882
0000308395 total tractional retinal detachment requiring multiple vitreoretinal surgeries in his right eye; last-recorded visual acuity 3 months earlier, right/left eye: 20/100, 20/50; ophthalmological examination at 17y: a decrease in visual acuity in his left eye to 20/63; fundus: temporal dragging of retinal vessels, peripheral exudation, and vitreoretinal tractions without retinal detachment; spectral-domain optical coherence tomography: vitreomacular traction with intraretinal cysts; central macular thickness: 283 um; re-evaluation 15 days later: progression to a lamellar macular hole; surgical procedure was suggested, 1 week later (before the surgery could be performed) acute metamorphopsia, visual acuity drop to 20/200; spectral-domain optical coherence tomography: full-thickness macular hole. Surgical management by 23-gauge pars plana vitrectomy with associated encircling buckle; posterior vitreous detachment was induced, internal limiting membrane peeled after staining with brilliant blue G, and SF6 was used as gas tamponade; 3 months postoperatively, visual acuity: 20/25. spectral-domain optical coherence tomography: sustained closure of the macular hole, with associated thinning of the neurosensory retina (central macular thickness: 166 um) - familial exudative vitreoretinopathy Isolated (sporadic) 17y 13y - - - LOVD 00416883
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