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Clinicopathologic Reports, Case Reports, and Small Case Series
March 2004

New Insights Into Progressive Visual Loss in Adult Retinopathy of Prematurity

Author Affiliations
 

W. RICHARDGREENMD

Arch Ophthalmol. 2004;122(3):404-406. doi:10.1001/archopht.122.3.404

An abnormal vitreous and aberrant vitreoretinal traction are significantfactors in the development of cicatricial changes and poor outcomes in neonateswith retinopathy of prematurity. Late complications of retinopathy of prematurity(ie, retinal tears or rhegmatogenous retinal detachment) have also been describedand these, too, can be attributed to an abnormal vitreoretinal relationship.1 Tasman and Brown2 observedthat progressive visual loss may be noted in adult retinopathy of prematuritywithout a definitive clinical cause. Tangential vitreoretinal traction wasspeculated to result in cystoid macular edema or compromised photoreceptorswith resulting pigmentary disturbances and visual deterioration.2 Hereinwe report our observations in a similar case of adult retinopathy of prematurityand with the aid of optical coherence tomography note that subclinical tractionalretinal detachment may be the cause of visual loss in at least some of thesecases.

Report of a Case

A 29-year-old woman had been delivered at gestational age 28 weeks witha birth weight of 1304 g. She had a history of retinopathy of prematurity.She was referred to our center for examination of a 7-month history of progressivevisual loss in her left eye. The patient had previously undergone multipleprocedures in the left eye for treatment of glaucoma and subsequent cataractextraction with intraocular lens placement at 16 years of age. Her baselinevisual acuity was 20/60 OS before the onset of her recent symptoms. She hadnot previously undergone cryotherapy or laser treatment in either eye. Herright eye had been enucleated 2 years previously for end-stage glaucoma andcomplications following rhegmatogenous retinal detachment related to previousintraocular surgeries.

On her initial visit, her visual acuity measured 20/400 OS. Biomicroscopicexamination results of the left eye revealed a quiet anterior segment witha posterior chamber intraocular lens and a central opening in the posteriorcapsule. Examination results of the right orbit revealed an ocular prosthesis.Funduscopic examination results of the left eye showed pulled retinal vesselsat the optic nerve (ie, a dragged disc) with temporal dragging of the fovea.A small organized area of central vitreous attached to the optic disc waspresent. Retinal pigment epithelial change was noted in the macula, but noclinically appreciable cystoid macular edema, epiretinal membrane formation,macular hole, retinal detachment, or other macular pathologic features werenoted. The peripheral retina was attached and had an avascular appearanceconsistent with a history of retinopathy of prematurity. The patient was askedto undergo fluorescein sodium angiography and optical coherence tomographytesting.

At a follow-up examination 1 week later, the patient had a further decreasein visual acuity to 20/800 OS. Slitlamp and funduscopic examination resultsof the left eye remained unchanged. Fluorescein angiography results of theleft eye revealed retinal pigment epithelial clumping without intraretinaledema or other abnormalities of the posterior segment except for dragging(Figure 1). Optical coherence tomographyfindings, however, did reveal a very shallow but obvious retinal detachmentinvolving the center of the macula (Figure2).

Figure 1.
Late-phase fluorescein sodiumangiogram of the left eye (507 seconds) shows retinal pigment epithelial clumpingwithout intraretinal edema or other abnormalities of the posterior segmentother than dragging.

Late-phase fluorescein sodiumangiogram of the left eye (507 seconds) shows retinal pigment epithelial clumpingwithout intraretinal edema or other abnormalities of the posterior segmentother than dragging.

Figure 2.
Preoperative horizontal opticalcoherence tomography section results reveal a very shallow but obvious maculardetachment with subretinal fluid.

Preoperative horizontal opticalcoherence tomography section results reveal a very shallow but obvious maculardetachment with subretinal fluid.

An uncomplicated vitrectomy with adjuvant autologous plasmin enzymewas subsequently performed. The hyaloid was detached and all lenticular remnantswere removed. Her postoperative course was complicated by a filamentary keratitisthat required an extended course of topical antibiotic therapy and a therapeuticcontact lens. For this reason, her visual acuity remained at light perceptionto bare hand motions for the first 2 postoperative visits. However, at postoperativeweek 4, the patient began using a therapeutic contact lens and reported improvedvision concomitant with resolution of her filamentary keratitis. Visual acuityat this time measured 20/70 OS at distance. Funduscopic examination resultsof the left eye revealed a clear vitreous cavity but an otherwise identical-appearingposterior segment and peripheral retina when compared with the preoperativeexamination findings. However, repeat optical coherence tomography resultsshowed resolution of the macular detachment and restoration of a foveal depression(Figure 3).

Figure 3.
Postoperative horizontal opticalcoherence tomography results show resolution of the macular detachment withrestoration of normal macular anatomy.

Postoperative horizontal opticalcoherence tomography results show resolution of the macular detachment withrestoration of normal macular anatomy.

Comment

The recent use of optical coherence tomography has demonstrated thatvisual loss in various vitreoretinal syndromes may result from a subclinicalphenomenon. Such conditions include poor vision associated with choroidalnevi, delayed visual recovery after retinal detachment repair, and posteriorhyaloidal traction syndrome associated with diabetic macular edema.35 Optical coherencetomography findings in these cases show the presence of subretinal fluid withmacular detachment not appreciable by clinical examination or with fluoresceinangiography. Similarly, in our patient here, with otherwise unremarkable clinicaland fluorescein angiographic findings, we were able to document the etiologyof her visual loss as subclinical retinal detachment only through opticalcoherence tomography and not through any other technique.

As mentioned previously, abnormal vitreous traction plays a causativerole in both neonatal and adult manifestations of retinopathy of prematurity.When present, vitrectomy is indicated to relieve vitreoretinal traction andrestore the normal anatomic configuration of the macula. Plasmin enzyme maybe useful in these patients in particular because hyaloidal separation isroutinely difficult in young adults and especially in patients with retinopathyof prematurity who have an abnormal vitreoretinal interface.6 Theposterior hyaloid in these cases is uncommonly adherent and when contractedcan result in tractional detachment of the retina and even vascular stasis.7 In our case, contraction of the vitreous and hyaloidas noted by organization of the vitreous overlying the optic nerve presumedlyresulted in macular detachment and visual loss. Plasmin-assisted vitrectomywas successful in reestablishing the patient's normal macular anatomy andrestoring her baseline visual acuity. When present and confirmed by carefulclinical examination, including adjunct methods such as optical coherencetomography, subclinical tractional retinal detachment in patients with adultretinopathy of prematurity with otherwise unexplained visual loss can be successfullytreated by vitrectomy. The results from using optical coherence tomographyin our case explain a circumstance that for many years had been observed yetwas inadequately understood by physicians managing adult patients with retinopathyof prematurity.

The authors have no relevant financial interest in this article.

Dr Shaikh is an AOS-Knapp fellow.

Corresponding author: Michael T. Trese, MD, 3535 W 13 Mile Rd, Suite632, Royal Oak, MI 48073 (e-mail: mgjt46@aol.com)

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