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Arch Ophthalmol. 2004;122:1717-1719.

Familial exudative vitreoretinopathy (FEVR) is an inherited vitreoretinal dystrophy with a variable clinical course. Early disease with no retinal detachment has been shown to respond well to primary laser treatment.¹ The following case manifested symmetrically but responded asymmetrically to appropriate and aggressive laser treatment.

Report of a Case
A 17-month-old girl was referred to the Vitreoretinal Surgical Service of the Bascom Palmer Eye Institute, Miami, Fla, for a strong family history of FEVR. The patient was delivered at full term, her medical history was unremarkable, and her developmental milestones were intact. She had no previous ocular history, but her mother noticed that the patient was bringing objects close to her face, squinting, and bumping into things. External examination revealed primary alternating esotropia. Dilated retinal examination of both eyes showed symmetric anterior ischemic retinopathy and secondary ridge neovascularization consistent with FEVR. Focal traction in this region was noted. The patient was treated with large spot–size laser ablation by indirect ophthalmoscopy to the anterior ischemic retina. One month following treatment, the patient had good regression of the neovascularization (Figure 1). Despite this regression, extramacular vitreoretinal traction alterations persisted in both eyes. Four months later, there was minimal progression of traction in the left eye with a localized area of extramacular schisis formation. The right eye showed progressive tractional alterations through the macula with development of a falciform retinal fold (Figure 2).

View larger version (34K): [in this window] [in a new window] Figure 1. Familial exudative vitreoretinopathy 1 month after laser therapy. There has been complete resolution of peripheral neovascularization with mild peripheral tractional changes in both eyes. A, Right eye. B, Left eye.

View larger version (34K): [in this window] [in a new window] Figure 2. Familial exudative vitreoretinopathy 4 months after laser therapy. There is progressive tractional alteration with falciform fold formation in the right eye (A) but minimal change in the left eye (B).

Comment
Familial exudative vitreoretinopathy is an inherited vitreoretinal dystrophy characterized by premature arrest of vascularization of the peripheral retina. There is a broad spectrum of disease involvement from mild avascular retinal changes that are slowly progressive to rapidly progressive tractional and/or exudative changes with total retinal detachment early in life. There has never been a prospective clinical trial for the management of active FEVR in infancy. Avascular retinal periphery with extraretinal vascularization alone has been shown to respond to laser treatment in retrospective reviews.¹ There is a broad spectrum of treatment response, and FEVR does not always respond like retinopathy of prematurity when the avascular retina is treated with laser. Advanced disease with retinal detachment usually requires scleral buckling and/or vitreous surgery.²

Previous authors have observed that patients whose onset of symptoms is before 3 years of age are at increased risk of poor visual out come.³ It appears that older patients have a better prognosis because they are likely to have asymmetric disease with only 1 eye severely affected. However, loss of good visual acuity may occur even well into adulthood.

Our patient was diagnosed at 17 months of age with symmetric anterior ischemic retinopathy and secondary ridge neovascularization with limited traction. There was no evidence of retinal detachment or falciform fold at initial examination. Both eyes were treated with near confluent laser to the avascular retina. While both eyes showed regression of arborizing vessels, tractional changes progressed in 1 eye with the development of a falciform retinal fold. Pendergast and Trese¹ described 7 patients who had similar disease involvement on initial examination as  our patient. Only 1 of these patients progressed to retinal detachment after laser treatment, requiring surgical intervention. Surgical intervention was considered in our patient; however, falciform detachments are difficult to eradicate and visual outcomes are often poor. Our experience emphasizes the possibility for disease progression despite appropriate laser therapy and initial vascular involution.

AUTHOR INFORMATION
Correspondence: Dr Murray, Bascom Palmer Eye Institute, 900 NW 17th St, Miami, FL 33136 (tmurray{at}med.miami.edu).

Financial Disclosure: None.

Ron Margolis, BA; Stephen S. Couvillion, MD; Elias C. Mavrofrides, MD; Ditte Hess, CRA; Timothy G. Murray, MD

REFERENCES
1. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy: results of surgical management. Ophthalmology. 1998;105:1015-1023. FULL TEXT | WEB OF SCIENCE | PUBMED 2. Shaikh S, Trese MT. Retinal reattachment facilitated by short-term perfluorocarbon liquid tamponade in a case of fever and rhegmatogenous retinal detachment. Retina. 2002;22:674-676. PUBMED 3. Shubert A, Tasman W. Familial exudative vitreoretinopathy: surgical intervention and visual acuity outcomes. Graefes Arch Clin Exp Ophthalmol. 1997;235:490-493. PUBMED

SECTION EDITOR: W. RICHARD GREEN, MD

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