Journal of Neuro-Ophthalmology, September 1985, Volume 5, Issue 3

Optic disc edema with aphakic cystoid maculopathy masquerading as ischemic optic neuropathy.

Two patients with visual loss after cataract extraction were found to have prominent pallid optic disc edema, peripapillary hemorrhage, and cystoid maculopathy. A diagnosis of anterior ischemic optic neuropathy, with coincidental aphakic cystoid macular edema, was initially considered. The clinical course, however, favored a diagnosis of visual loss secondary to maculopathy. In both cases, fluorescein angiography disclosed leakage of fluorescein dye from macula, optic disc, and peripapillary foci. Fluorescein angiography and stereo color photographs of 51 patients with aphakic cystoid maculopathy were reviewed to determine the incidence of optic disc edema. Although optic disc swelling was rarely recognized on fundus photography, optic disc hyperfluorescence was observed in 75% of cases.

J. Clill. NClIro-0l'hthallllol. 5: 1HO-1H4, 1':1H5 © 1985 Raven Press, New York Optic Disc Edema with Aphakic . Cystoid Maculopathy MasqueradIng as Ischemic Optic Neuropathy MICHAEL L. SLAVIN, M.D. RONALD J. LOPINTO, M.D. ARNOLD S. PRYWES, M.D. DAVID A. ROSEN, M.D. Abstract Two patients with visual loss after cataract extrac­tion were found to have prominent pallid optic disc edema, peripapillary hemorrhage, and cystoid maculopathy. A diagnosis of anterior ischemic optic neuropathy, with coincidental aphakic cys­toid macular edema, was initially considered. The clinical course, however, favored a diagnosis of vi­suaI loss secondary to maculopathy. In both cases, fluorescein angiography disclosed leakage of fluo­rescein dye from macula, optic disc, and peripapil­lary foci. Fluorescein angiography and stereo color photographs of 51 patients with aphakic cystoid maculopathy were reviewed to determine the inci­dence of optic disc edema. Although optic disc swelling was rarely recognized on fundus photog­raphy, optic disc hyperfluorescence was observed in 75% of cases. Visual loss after cataract surgery with optic disc edema and peripapillary hemorrhages most frequently is attributed to anterior ischemic optic neuropathy. Typically, anterior ischemic optic neuropathy is characterized by specific vi­sual field disturbances, afferent pupillary de­fect, and pallid edema of the optic nerve. Post­operative cystoid macular edema may be asso­ciated with ophthalmoscopic evidence of optic disc edema but only occasionally with marked optic disc swelling and peripapillary hemor­rhage. In this report, two postoperative cataract pa- From the Department of Ophthalmology, Division of Neuro-ophthalmology, Long Island Jewish Medical Center. New Hyde Park, New York; and SUNY at Stony Brook, Health Sciences Center, School of Medicine, Stony Brook, New York. Write for reprints to: M. L. Slavin, M.D., 270-05 76th Av­enue, New Hyde Park, NY 11042, U.S.A. 180 tients with visual loss are described in whom optic disc swelling and peripapillary hemor­rhages overshadowed the macular edema and whose subsequent clinical course was entirely consistent with typical aphakic cystoid macu­lopathy. Both patients were initially felt to have anterior ischemic optic neuropathy with coin­cidental cystoid macular edema. In addition, we reviewed color photographs and fluorescein an­giograms of recent cases of postoperative cys­toid macular edema to determine the incidence of concomitant optic disc edema. Case Reports Case 1 A 70-year-old woman underwent uneventful intracapsular cataract extraction in the right eye. Six weeks postoperatively, best corrected visual acuity was 20/40 in the right eye and 20/60 in the left eye (secondary to lens changes). The pupils were 5 mm on each side, with a 3+ re­sponse to light. No afferent pupillary defect was seen. Slit-lamp examination was normal except for the presence of 1+ flare and cells in the anterior chamber on the right. Goldmann pe­rimetry showed minimal generalized constric­tion with no focal scotomata. Ophthalmoscopy revealed marked pallid swelling of the right optic nerve with peripapillary hemorrhages and slight arteriolar narrowing (Fig. 1, top). The macular reflex was diminished and cystoid mac­ular edema was suspected, although no distinct intraretinal cysts were seen by Hruby lens ex­amination. Peripheral retinal hemorrhages and venous distension were not observed. fluores­cein angiography disclosed prominent disc and peripapillary dye leakage and evident cystoid macular edema (Fig. 1, center). Follow-up examination 7 months later re­vealed a visual acuity of 20130 in the right eye, Journal of Clinical Neuro-ophthalmology with a normal-appearing optic nerve and macula (Fig. 1, bottom). Case 2 A 70-year-old woman underwent right cata­ract extraction complicated by vitreous loss in August 1983. Anterior vitrectomy was per­formed and an anterior chamber lens was im­planted. The postoperative course included cil­iochoroidal effusion, anterior segment inflam­mation (with mutton-felt precipit.ltt'S on the implanted lens), and intraocular pressure ele­vation; visual acuitv was 20/30 1 month later. Prednisone, 1'i(-, alid timoloI. 0.5'ic, were pre­scribed, and the patient was lost to follow-up for approximatel~' 1 ~·ear. When the patient was examined in July 1984, visual acuity was 20/200 in the right eye. Mul­tiple precipitates were again seen on the im­plant, and the intraocular pressure was 30 mm Hg. Vision was 3:200 in the left eye because of a dense cataract. A mild afferent pupillary de­fect was detected in the right eye. Goldmann perimetry study disclosed an enlarged blind spot and small central scotoma to the I13e isopter. Ophthalmoscopy revealed pallid swelling of the right optic disc with superficial peripapillary hemorrhages, and cystoid macular edema. Fluorescein angiography showed dye leakage from the optic disc, macula, and peri­papillary sites. Topical corticosteroid and antiglaucoma agent therapy was reinstituted, and over the next sev­eral weeks, visual acuity improved to 20/50, with diminished cystoid maculopathy and optic disc edema. Materials and Methods Stereo color photographs and fluorescein an­giograms of 68 patients who were diagnosed as having aphakic cystoid macular edema (from 1980 to 1984) were selected from the ophthal­mology photographic files at Long Island Jewish Medical Center. Additional history in these cases was obtained from the requisition sheets initially submitted by the referring ophthalmol­ogists. Cases with photographic evidence of di· abetic retinopathy (12 patients) or occlusive vas­cular disease (three patients) were eliminated from consideration. None of the patients had ocular hypotony or chronic uveitis. Two pa­tients with suspected clinical cystoid macular edema had disc leakage only on fluorescein an­giography. No information on visual fields, pupil examination, or intraocular pressure wa.s provided in those cases, and they were elimI­nated from the study. Thus, stereo color pho­tographs and fluorescein angiograms of 51 pa- September 1985 Slavin et al. Figure 1. Top: Milr!-.t·d p,lllid "ptic' disc' edema with p,'ri­p" pill"rv Iwnwrrh,lgl". Center: Fundus f1ulHl'S(l'in 'lJ1gi­ogro1phy sllllwing dl'l' 1<-001-.'lhl' lnllll "pti( disc', Ill,lc'ul". "nd pl'rip.lpill,lrl' IllLi. Bottom: N'Hnl,ll of'tk Ill'r\"l' Sl'\','r,,1 months I,lter. tients were reviewed by one of us (M.L.S.). Late photographs on fluorescein angiograms (ap­proximately 5 min after injection) were exam­ined and categorized as follows (Fig. 2): 181 Optic Disc Eclcm.l with Aphakic Cystoid MilculoPilthy Figure 2. Classification of optic disc edema with aphakic cI'stoid macu!L'pathY on tluores(t,'in angiographl': Grade I-no relative hyperfluorescence (top left); Grade II-relatil'e hl'pertluorescence cLlntined tL' the Llptic disc (smooth-edged pattern) (top right); Grade IIl-hyperfluorescence extending beyond the optic disc (ieathered-t'dge pattt'rn) (bottom left); Grade IV- marked hyperfluorescenct' from optic disc and peripapillary iLKi (bottom rightl. Grade I-no hyperfluorescence of optic disc rel­ative to the fellow eye. Grade II-relative hyperfluorescence of optic disc contained within the disc's anatomical borders (smooth-edged pattern), Grade III-optic disc hypertluorescence beyond the disc's borders (feathered-edge pattern). Grade IV - marked optic disc hyperfluores­cence beyond the disc's borders, with distinct foci of peripapillary tluorescein leakage. Results As assessed by stereo color photographs, the optic nerve was deemed normal in 41 cases, hy- 182 peremic in h\'o, slightl~' edematous in two, and markedly edematous with peripapillary super­ficial hemorrhages in two (case nos. 1 and 2 in this report). In four cases, the media or quality of the stereo color photographs precluded eval­uation of l)ptiC disc edema. In 38 of 51 cases (75'i(). relative fluorescein leakage from the optic disc was detected. A designation of Grade 1 was made in 13 cases, Grade II in 17 cases, Grade 1II in 13 cases, and Grade IV in eight cases, Of the last eight cases, two had marked disc edema and hemorrhage on stereo color photographs, one had disc hyperemia, one had minimal disc edema, three had normal optic discs, and the photograph for one case was un­readable because of lens opacity. Journal of Clinical Neuro-ophthalmology Discussion Visuallo~s afte.r ca.taract extraction with l)ph­thalmoscoplC optic dIsc edema is l)ften asnibl'd ~o ante~ior i~chemic optic neuropathy. Antl'rior IschemIc optic neuropathv mav occur in this sL'l­ting eit~er. in a ca~~se-~nli-dfect rl'lationship or as a cOinCIdental tll1dlng. In till' latter circulll­stance, symptoms often begin wl'l'ks or months after surgery. The association bL'lween cat.Had su~gery and optic neuropath\' \\',lS iirst de­scnbed by Townes and associ,ltl'sl in IlJ:il. four eyes desc~bed.in their rl'port developed poor vIsual aCUIty WIth edematous optic nerves -l-l:i months subsequent to cataract l'xtraction. All dev~~oped optic atroph~'. ViSll,ll field abnor­malIties were not mentioned. Since that article th.ere ha~'e been several reports of this entity: WIth OptiC neuropathy occurring as early as 4 weeks' and as late as 7 vears" after cataract ex­tr~ ctio~ .. In 1980, Hayreh4 described 13 eyes ~Ith chmcal ~nterior ischemic optic neuropathy In the I~medlate postoperative period. None of the. pa~ents .had a documented postoperative penod In whIch visual function was normal be­fore the onset of anterior ischemic optic neurop­athy. Raised intraocular pressure as docu­mented by ocular pain, tonometry, or corneal edema was noted in 11 of the patients soon after s~rgery. Because of a suspected high risk of s~ilar developments in the fellow eye, prophy­laXIS for postoperative intraocular hypertension was strongly advised. . Irrespective of its clinical setting, anterior IschemIC optic neuropathv has characteristic op~thal~c features. As in' other optic neurop­athIes, vls.u~1 dysfu.nction as demonstrated by abnormalItIes of VIsual aCUIty, visual fields, pupil examination, or color vision is always ev­Ident. Inferior altitudinal defects make up the typICal pattern of visual field loss occurring in 70-8OCk of cases in most_series. 5-? In the study by Boghen and Glaser,~ central field defects were seen in 12.5% of cases. While pallid edema of the optic nerve (and retinal arteriolar nar­rowing) is the hallmark of this disorder, hyper­emIa of the optic disc is occasionally observed. Sectoral or diffuse optic atrophy ensues rapidly and may be apparent within 2 weeks of visual loss, as compared with atrophy secondary to optic neuritis and compressive optic neurop­athy. Recovery of visual acuity or visual fields is infrequent. Symptomatic nonsimultaneous bilateral involvement in nonarteritic anterior ischemic optic neuropathy has been reported in 15-40% of cases in most series. 5•H.'/ The second eye is often involved within 1 year. Four pa­tients with classical anterior ischemic optic neu­ropathy in one eye and concomitant optic disc September 1985 Slavin et al. l'dema as the only sign in the fellow eye were dl'scnbed by Hilyreh III in 1981. Each patient, howewr, developed iln inferonasal visual field dl'lect on that side within 3 months. The presence oi optic disc edema with cystoid 1ll,1cular l'liema, illthough detected in 75't? of our cases by fluorescein angiography, was not a common hndlng m the color photographic as­sessments [two (4%) cases had florid edema, two (4%) had minimal edema, and two (4%) had hyperemic discs]. Gass and Norton ll described 14 oi 44 patients as having optic disc edema (on clImcal examination) in their series of patients With cystOid macular edema. Attendant optic dISC hemorrhages in that study were "occasion­ally seen," whereas we noted this sign in only two cases. Case 1 is unusual in that marked optic disc edema and superficial peripapillary hemor­rha~ es wer~ the. predominant ophthalmoscopic findings. DIminIshed VIsual acuity but absence of an afferent pupillary defect and visual field abnormality made optic neuropathy an unlikely dIagnOSIs. The optic disc edema was noted, on fluorescein angiography, to be part of a gener­alIzed posterior pole edema (from macular, optic disc, and distinct peripapillary foci). While the coincidental occurrence of cystoid macular edema and subclinical ischemic papillopathv (see above) cannot be ruled out, the subsequent Improvement of visual function and the normal appearance of the optic nerve make it unlikelv. Case 2 had evidence of decreased visu'al acuit~, central scotoma, afferent pupil defect, cystOId macular edema, and optic disc edema, ~uggesting a concurrent optic neuropathy. lim­Ited central scotomas and subtle afferent pupil defects, however, may be seen with maculop­athy. 12 Fluo~escein angiograms (as in case 1) and the chmcaI course again suggest that the optic diSC edema was the result of diffuse break­down of the blood - retina barrier in the poste­rior pole. The presence oi optic disc edema and visual loss in an aphakic patient with clinical signs un­characteristic of optic neuropathv should, there­fore, suggest the svndrome oi cvstoid macular edema. Fluorescei~ angiograph\; is the confir-mative diagnostic procedure. . References 1. Townes, C. D., Moran, C. T., Pfingst, H. A.: Complications of cataract surgerv. Tral/s. Alii. 0l'hilltlfllloi. SPt'. 49: 91-107, 1951. - 2. Carroll, F. D.: Optic nerve complications of cat­aract extraction. Tral/s. Alii. Acad. Ophihalllloi. OlolarYI/S01. 77: 623-629, 1973. 3. Welch, R. B., and Cooper, J. c.: Macular edema, 183 Optic Disc Edl'nhl with Aph,lkic Cyst'.1id Maculopathy p,lpilledel11a ,1I1d llptic atrophy aiter cataract ex­tr, lCtilln. 11,.<11. Opltt/III/lIIol. 59: 665-675, IY5H. 4. H,wrL'h, S. S.: Anterior ischemic optic neurop­, lthv: IV. <..knlrrenCl'S after cataract extraction. /I,..';/. Ol/lttllllllllo/. 98: 1410- 1416, IYHO. 5. Bllghl'n, D. R., and Clasl'r, J. 5.: Ischemic optic l1l'umpathy. Hrail/ 98: 6HY-70H, IY75. 6. Ellenbergl'r, c., Jr.: Ischemic optic neuropathy as a possible early complication uf vascular hyper­tension. Alii. /. Oplttllll/II/O/. 88: 1045-1051, 1979. 7. H,lyreh, S. 5., and Podhajsky, P.: Visual field dc­fects in anterior ischemic IIp'tiC neuropathy. Doc. OplttlIllIIllO/. Proc. Sa. 19: 53-71, 1979. 8. Cullen, J. F.: Ischemic optic neuropathy. Trans. Ophtha/mol. Soc. U.K. 87: 759-774, 1967a. 184 9. Geogiades, G., Konstas, P., and Stangos, N.: Re­flexions issues de I'etude de nombreus cas de pseudo-papillite vasculaire. Bull. Soc. Opiha/. Fr. 79: 505-536, 1966. 10. Hayreh, S. S.: Anterior ischemic optic neurop­athy: V. Optic disk edema an early sign. Arch. Ophtha/mo/. 99: 1030-1040, 1981. 11. Gass, J. D. M., and Norton, E. W. D.: Cystoid macular edema and papilledema follOWing cata­ract extraction. A fluorescein, funduscopic and angiographic study. Arch. aphtha/mol. 76: 646­661, 1966. 12. Walsh, F. B., and Hoyt, W. F.: Clinical Neuro-oph­thalm% gy. Williams & Wilkins, Baltimore, 1969, pp. 612-613. Journal of Clinical Neuro-ophthalmology