Customize your JAMA Network experience by selecting one or more topics from the list below.
Familial exudative vitreoretinopathy (FEVR) is an inherited vitreoretinaldystrophy with a variable clinical course. Early disease with no retinal detachmenthas been shown to respond well to primary laser treatment.1 Thefollowing case manifested symmetrically but responded asymmetrically to appropriateand aggressive laser treatment.
Report of a Case
A 17-month-old girl was referred to the Vitreoretinal Surgical Serviceof the Bascom Palmer Eye Institute, Miami, Fla, for a strong family historyof 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 herface, squinting, and bumping into things. External examination revealed primaryalternating esotropia. Dilated retinal examination of both eyes showed symmetricanterior ischemic retinopathy and secondary ridge neovascularization consistentwith FEVR. Focal traction in this region was noted. The patient was treatedwith large spot–size laser ablation by indirect ophthalmoscopy to theanterior ischemic retina. One month following treatment, the patient had goodregression of the neovascularization (Figure 1). Despite this regression, extramacular vitreoretinal tractionalterations persisted in both eyes. Four months later, there was minimal progressionof traction in the left eye with a localized area of extramacular schisisformation. The right eye showed progressive tractional alterations throughthe macula with development of a falciform retinal fold (Figure 2).
Familial exudative vitreoretinopathy1 month after laser therapy. There has been complete resolution of peripheralneovascularization with mild peripheral tractional changes in both eyes. A,Right eye. B, Left eye.
Familial exudative vitreoretinopathy4 months after laser therapy. There is progressive tractional alteration withfalciform fold formation in the right eye (A) but minimal change in the lefteye (B).
Familial exudative vitreoretinopathy is an inherited vitreoretinal dystrophycharacterized by premature arrest of vascularization of the peripheral retina.There is a broad spectrum of disease involvement from mild avascular retinalchanges that are slowly progressive to rapidly progressive tractional and/orexudative changes with total retinal detachment early in life. There has neverbeen a prospective clinical trial for the management of active FEVR in infancy.Avascular retinal periphery with extraretinal vascularization alone has beenshown to respond to laser treatment in retrospective reviews.1 Thereis a broad spectrum of treatment response, and FEVR does not always respondlike retinopathy of prematurity when the avascular retina is treated withlaser. Advanced disease with retinal detachment usually requires scleral bucklingand/or vitreous surgery.2
Previous authors have observed that patients whose onset of symptomsis before 3 years of age are at increased risk of poor visual out come.3 It appears that older patients have a better prognosisbecause they are likely to have asymmetric disease with only 1 eye severelyaffected. However, loss of good visual acuity may occur even well into adulthood.
Our patient was diagnosed at 17 months of age with symmetric anteriorischemic 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 changesprogressed in 1 eye with the development of a falciform retinal fold. Pendergastand Trese1 described 7 patients who had similardisease involvement on initial examination as our patient. Only 1 ofthese patients progressed to retinal detachment after laser treatment, requiringsurgical intervention. Surgical intervention was considered in our patient;however, falciform detachments are difficult to eradicate and visual outcomesare often poor. Our experience emphasizes the possibility for disease progressiondespite appropriate laser therapy and initial vascular involution.
Correspondence: Dr Murray, Bascom PalmerEye Institute, 900 NW 17th St, Miami, FL 33136 (email@example.com).
Financial Disclosure: None.
Margolis R, Couvillion SS, Mavrofrides EC, Hess D, Murray TG. Progression of Familial Exudative Vitreoretinopathy After Laser Treatment. Arch Ophthalmol. 2004;122(11):1717–1719. doi:10.1001/archopht.122.11.1717
Coronavirus Resource Center