Ophthalmology 1998 Jun;105(6):1015-23 Related Articles, Links

Familial exudative vitreoretinopathy. Results of surgical management.

Pendergast SD, Trese MT.

William Beaumont Eye Institute, Royal Oak, Michigan, USA.

OBJECTIVE: The purpose of the study was to report the results of surgical management of familial exudative vitreoretinopathy (FEVR). DESIGN: The study design was a retrospective clinical study. PARTICIPANTS: A consecutive series of 52 eyes of 26 patients with FEVR were studied. INTERVENTION: All eyes underwent a complete ocular examination and were graded using a new classification system. Depending on the severity of disease, eyes were treated with peripheral laser photocoagulation, scleral buckling, or vitrectomy. MAIN OUTCOME MEASURES: Preoperative and postoperative visual functions and anatomic status of the macula were the main parameters evaluated. RESULTS: A total of 40 eyes were treated. Seven eyes required no treatment and five eyes had inoperable retinal detachments. Fifteen eyes were treated with peripheral laser ablation initially and 25 eyes presenting with retinal detachments required vitreoretinal surgery. Of the 15 eyes treated initially with laser, 8 eyes required no further treatment, whereas 7 eyes progressed to retinal detachment requiring vitreoretinal surgery. A total of 32 eyes (including 7 previously lasered eyes) underwent vitreoretinal surgery. Twenty-nine of these 32 eyes had at least 6 months of follow-up. At the last follow-up visit, the macula was attached completely in 18 eyes (62.1%). Visual acuity ranged from 20/25 to light perception, with 10 (34.5%) of the 29 eyes achieving Snellen acuities of 20/100 or better. Two eyes (6.3%) progressed to no light perception. CONCLUSION: These data suggest that surgical intervention can be beneficial in selected cases of FEVR.

PMID: 9627651 [PubMed – indexed for MEDLINE]



Graefes Arch Clin Exp Ophthalmol 1997 Aug;235(8):490-3 Related Articles, Links
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Familial exudative vitreoretinopathy: surgical intervention and visual acuity outcomes.

Shubert A, Tasman W.

Wills Eye Hospital, Philadelphia, PA 19107, USA.

BACKGROUND: Familial exudative vitreoretinopathy (FEVR) is a hereditary condition that may lead to vitreous hemorrhage and traction retinal detachment necessitating surgical intervention. In this paper we review the results of surgery on seven such patients (eight eyes). METHODS: Seven patients (eight eyes) were followed up after surgery that had been performed because of vitreous hemorrhage and/or traction retinal detachment due to FEVR, in an effort to evaluate outcomes. Parameters that were noted were the current age, gender, age at the time of first surgery, length of follow-up and postoperative retinal status and visual acuity. RESULTS: Seven patients (eight eyes) ranging in age from 6 months to 44 years with a mean of 24.7 and a median of 26 years were followed. There were three females and four males. The lowest age at which surgery was first performed was 6 months and the highest was 28 years, with a mean of 14.7 and a median of 17 years. Six of the 8 eyes were reattached following surgery, although some required multiple procedures. CONCLUSION: Vitreoretinal surgery may be of benefit in helping to preserve some degree of vision in eyes of patients with FEVR who develop vitreous hemorrhage and/or retinal detachment.

PMID: 9285217 [PubMed – indexed for MEDLINE]



J Glaucoma 1997 Feb;6(1):47-9 Related Articles, Links

Familial exudative vitreoretinopathy associated with nonneovascular chronic angle-closure glaucoma.

Azuara-Blanco A, Pesin SR, Katz LJ, Michael AJ, Tasman WS.

Glaucoma Service, Wills Eye Hospital, Jefferson Medical School, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.

PURPOSE: Cases of angle-closure glaucoma in patients with familial exudative vitreoretinopathy have been reported secondary to neovascularization of the anterior segment. Cases secondary to nonneovascular mechanisms have not been previously reported. METHODS: Two cases are presented of angle-closure glaucoma as a result of a nonneovascular mechanism. RESULTS: Neovascularization was found to be a very unlikely explanation for the angle closure in these two cases. CONCLUSION: There may be an association between familial exudative vitreoretinopathy and angle-closure glaucoma as a direct result of a retrolental process or more likely a relative lens-iris pupillary block with a large lens.

PMID: 9075081 [PubMed – indexed for MEDLINE]



Am J Ophthalmol 1995 Oct;120(4):471-9 Links

Improved surgical treatment of familial exudative vitreoretinopathy in children.

Glazer LC, Maguire A, Blumenkranz MS, Trese MT, Green WR.

Rush Medical College, Chicago, Illinois, USA.

PURPOSE: To evaluate current surgical results in patients with familial exudative vitreoretinopathy and study the histologic findings of epiretinal membranes obtained at the time of surgery. METHODS: We performed phakic pars plana vitrectomy and membrane peeling on five patients (six eyes) with familial exudative vitreoretinopathy, who ranged in age from 18 months to 9 years and had traction retinal detachment. Membranes from two eyes (two patients) were analyzed by light and electron microscopy. RESULTS: The macula was reattached in all six eyes. Visual acuity improved after surgery in five of the six eyes, with two eyes improving from a preoperative visual acuity of 20/400 to a postoperative visual acuity of 20/25 and 20/60. Electron microscopic analysis of the membranes from two patients disclosed thick fibrocellular fragments with vascular elements and astrocytes. CONCLUSION: With current surgical techniques, there appears to be improvement in the anatomic reattachment rate and visual outcome in patients with familial exudative vitreoretinopathy. Amblyopia, reproliferation, and vitreous hemorrhage may limit long-term improvement in vision.

PMID: 7573305 [PubMed – indexed for MEDLINE]


Klin Oczna 1991 Jul-Aug;93(7-8):202-4 Links

[Familial exudative vitreoretinopathy–vitreoretinopathia familiaris exsudative. I. Review of the literature]

[Article in Polish]

Starzycka M.

Kliniki Okulistycznej, Krakowie, Poland.

Presented are the contemporary opinions of the etiopathogenesis, signs, the clinical course as well as the differentiation and therapy of the familial exudative vitreoretinopathy. Attention is called to the variety of the clinical picture of this disease and the importance of fluorescein angiography in diagnosis.

Publication Types:
  • Review
  • Review, Multicase

PMID: 1762366 [PubMed – indexed for MEDLINE]



Am J Ophthalmol 1991 Jan 15;111(1):34-41 Related Articles, Links

Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy.

van Nouhuys CE.

Department of Ophthalmology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands.

Between 1979 and 1989, I examined 106 patients (16 pedigrees) with signs of familial exudative vitreoretinopathy. Of these patients, 101 had familial exudative vitreoretinopathy, and five had a sporadic manifestation. The complications of familial exudative vitreoretinopathy, deformation of the posterior retina, vitreous hemorrhage, amblyopia, and retinal detachment, caused diminished visual acuity. Of 170 eyes, retinal neovascularization was observed in 18 eyes (11%), and retinal exudates were observed in 16 eyes (9%). Several forms of retinal detachment occurred in 37 of 180 eyes (21%), which often took an unfavorable course. A falciform retinal fold was observed in 14 eyes (8%). Retinal surgery was performed in 14 eyes; reattachment of the retina was successful in only seven eyes. Platelet aggregation studies disclosed no significant differences between seven patients with familial exudative vitreoretinopathy and ten control subjects. The pathogenesis of the disease is based on a premature arrest of the vascular development of the retina.

PMID: 1985487 [PubMed – indexed for MEDLINE]