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Show ~', 1'ffl5 R.wen Press, Nl'W York Pseudohemangioma of the Optic Disc Following Ischemic Optic Neuropathy J. LAWTON SMITH, M.D. A b,.'l·V"'M-llld Wl,"l.m with .m l'!t'\'.\kd m.l$S oc("upyin~ tht' t'nlin' uppl'r tt'mpl.lr.ll qu.ldr.lnt of the It'ft \lptic di:'C W.\S t'\..lOlint'd in Dt'Ct"mbE'r 19i-l, Tht' l"'sil," r"'st'mbled .In .lngiom.l llf tilt' optic nt'n't' by ~lth llphth.11ml1scopit": .md {JUtlrt'sct'in-., n~ill~r.\phil· crilt'ri.l. Thre'" mtmths latt'r. tht' It'sillll Shll\\'",d no chdn~t'. Howt"vt'r, dt a fllutine fllllllw-up in J.muary 1976 tht" mdSS hild di$<lppt:>drl.'d. This occurr",d with ml change in svmpll1ms or \'isu.lI function at .111. The patit'n't hild prt'\'il1usly sufft'red classic ~uts of nonilrt",ritk jscht"mic optic neuropathy In both eves. She rt"maint"d c1inicillly stilblt' thert'dfter o'n serial t"xaminations for the next 5 years. This lesion rna\' be called a "pseudohemdngiomd" of the optiC' disc. It has since 1:leen observed i.n other patients with previous bouts of optiC nen'e ischemia, The process may also be called "pseudoangiomatous hyperplasiil" of the optic disc. or the "proud flesh syndrome." This report documents this interesting ophthalmo' scopic observation to point out the Imp.ortance of differentiating it from a true hemangIOma ?f the optic disc, to comment on th.e pathog~n~sls of its development, and to consIder the t.lml~g of its appearance and subsequent resolutIOn In a few other cases. Case 1 A 63·year-old right-handed white woman was first seen December 13. 1974, through the courtesy of Dr, Valberg of Ottawa. Canad~. Her chief complaint was difficulty with viSion In the right eye since 1968 and wi~h vision in the left eye since July 1974. Present Illness revealed thai she had had no visual problems until june 1968. when she noted the onset of blurred vision in the right eye and was foun.d t~ have a .swollen optic disc and a lower alhtudl.nal optIC nerve fiber bundle visual Cield defect III that eye. The From the Depilrtment of Ophthalmology. 8.J!j(l"m r~[.m ...r Eye Institute. University of M"lmi Schoul of M~·dl("H"" Miami. Aoricb. t>HllO Wrltt for rrprmls IQ: J. L. Smith. M.D., r.o. B...~ 01 . Mi.lmi. FL )3101. U.S.A. .utt.'rillit's Wt'rt.' narrowl'd un the disc. the disc rapidly r.t.'Camt· pale, and, although the field deft'Ct pt'rsistcd. she was had 20120 acuity in the t'yt' and no subsequent change thereafter. She did well for the next 5 years until July 1974 (6 months bt"fore presentation). when she suddenly developed a superior altitudin~l fi.eld d:ft.' ct in the left eye. She was hospltahzed.1Il Canada and was examined thoroughly. 1Ileluding roentgenography of the skull. chest. and optic foramina, a brain scan, and a lumbar puncture. The impression was of ischemic ophC neuropathy. A short course of oral steroids at a modest dosage produced questionable improvement in the left eye, She had no other neurologic complaints. Her .past histo~y w~s otherwise unremarkable, while her family hiStory was positive for diabetes mellitus in her mother. An examination in December 1974 revealed a corrected acuity of 20115 - 1 in the right eye and 20/25 + 1 in the left eye. Blood pressure was 170/100 in both arms, The visual fields revealed a dense, upper altitudinal defect in the left eye and a dense, lower nasal. field defect ~n the right eye. Applanation tensIon was 12 In both eyes. Ophthalmoscopy revealed diffuse pallor of the right disc, without. any particular atrophic cupping, and the artenoles were very narrow (Fig. I). The left disc was pale, and there was an elevated mass un the llpper temporal aspect between 12 and 3 u'elock (Fig. 2). On fluorescein angiography of tht' left disc. the mass resemblt'd an angioma of the disc, filling in the arterial phase (Fig. 3) and stainin~ in later phases (Fig, 4). The impression was ot nonarterilic ischemic optic neuropathy with a pseudoangioma of the left optic disc. At a follow-up ex.lmination 3 months later. the It'lt disc appt>art'tJ unchanged. Hm.......ver. at a fulluw-up t>xamination on January 20. 1976, although no interval symptoms had occurred and acuity and fields were unchanged. the mass on thl.' left disc hdd disappeared (Figs. 5-7). The patient was seen again in January 1977 and January 1978. with no further changes noted. Dr. Weint'r saw her in January 1979 and found visual acuities of 20/20 in the right eye and 81 Pseudohemangioma of Optic Disc Figure 1. Case 1. Right eye on December 13, 1974. showing changes of nonarteritic ischemic optic neuropathy that had occurred 6 years earlier. 20/25 in the left eye and noted that the fields and discs were unchanged. Comment A 63-year-old woman was seen in 1974. She had developed typical ischemic optic neuropathy in the right eye 6 years earlier and in the left eye 6 months prior to examination. She had excellent acuity but a lower-field defect in the right eye and an upper-field defect in the left Figure 2. Case 1. Left eye on December 13, 1974, showing changes of nonarteritic ischemic optic neuropathy that had occurred 6 months earlier. Note the elevated reddish mass on upper temporal margin of this disc (arrowheads). 82 Figure 3. Case 1. Fluorescein angiogram of left eye on December 13, 1974. Note that the mass on upper temporal disc fills in the arterial phase (arrowheads). eye. Routine examination 6 months after the episode in the left eye revealed an elevated mass lesion on the left disc that closely resembled a hemangioma of the optic disc by both ophthalmoscopy and fluorescein angiography. The lesion was unchanged 3 months later, but 13 months after the first examination the lesion was found to have spontaneously regressed to such a point that it had effectively disappeared. No subsequent change occurred in acuity, fields, or discs over the next 5 years. This was the author's first and most dramatic Figure 4. Case 1. Venous-phase fluorescein angiogram shows late staining of this vascular mass (arrowheads). Journal of Clinical Neuro-ophtha!mo!ogy Figure 5. Case 1. Leit e~'e on January 20, 1976. Note that the mass on upper temporal aspect of this disc has essentiall\" diSdppeared. obseryation of pseudoangiomatous hyperplasia of the optic disc following ischemic optic neuropathy. A few milder cases are documented below to illustrate other points about this finding. Case 2 A 62-year-old black man had been taking oral hypoglycemic drugs for diabetes mellitus for 5 years. On September 8, 1984, he noted the onset of a painless "shadow" before the left eye. Examination at the Bascom Palmer Eye Institute Figure 6. Case 1. 2 x fundus photograph of left eye on lJecember 13, 1974, showing large, elevated pseudohemangioma of the disc (arrowheads). June 1985 Smith Figure 7. Case 1. 2 x fundus photograph of left eye on January 20, 1976, showing that the mass has effectively disappeared. Only a few "ghost vessels" remain (arrowheads). that day revealed a corrected vision of 20/25 + in the right eye and 20/60 + in the left eye. A lower altitudinal visual field defect was noted in the left eye. A 3 + Marcus Gunn pupil was present on the left. The right disc was within normal limits (Fig. 8). The left disc showed diffuse swelling of the entire upper temporal margin, with abnormal small vessels in that distribution and two small linear hemorrhages on the disc margin (Fig. 9). Exudative and lipid deposits were seen in the adjacent retina. On September 13, 1984, the corrected vision was 20/30 in the right eye and 20/100 in the left eye. Blood pressure was 164190, sedimentation rate was 58, serum fluorescent treponemal antibody absorp- Figure 8. Case 2. Right eye in September 1984, showing a normal disc. 83 Pscudohcmangil1ma of Optic Disc Figure 9. Case 2. Left eye in September 1984, showing pseudoangiomatous hyperplasia of upper temporal quadrant of the disc. hon (FTA-ABS) was nonreactive, and the blood glucose level was 269 mg%. The impression was of ischemic optic neuropathy in the left eye. Fluorescein angiography confirmed the presence of the pseudoangiomatous hyperplasia of the entire upper temporal quadrant of the left disc (Figs. 10 and 11). On September 21, 1984, Dr. Darnley noted that the tuft of new vessels on the left disc above was still present but was starting to resolve. It should be noted that the patient had had a routine eye examination at the Bascom Palmer Eye Institute on June 21, 1983, when he had lost his glasses. Corrected acuity was 20/25 in the right eye and 20/20 in the left eye; a dilated Figure 10. Case 2. Left eye in September 19H4, showing pseudoangiomatous hyperplasia of upper tempor.ll quadrant of disc and partial macular star figurl>. Two small l1l'morrhages are seen at 12:45 and 2 o'clock on thl' disc. Nlltt. abnormal vessels in this area (arrow). 84 Figure 11. Case 2. Fluorescein angiogram of left eye in September 1984, showing abnormal vessles and late staining of disc in this quadrant (arrowheads). fundus examination at that time revealed healthy discs and no background retinopathy in either eye. Comment A 62-year-old diabetic man had normal optic discs in 1983. In September 1984, he developed acute ischemic optic neuropathy in the left eye, and the disc acutely showed pseudoangiomatous hyperplasia of the upper temporal quadrant that was confirmed on fluorescein angiography. This case illustrates that the process under consideration can develop very rapidly in an ischemic disc. Case 3 A 46-year-old right-handed white man had 20/20 vision in both eyes until November 23, 1975, when he awoke ~ith blurred vision in the lower field of the left eye. This was painless, and it progressed over the next few days. He had no other complaints. He was examined at the Bascom Palmer Eye Institute on December 17, 1975, by Dr. J. W. Klein. Visual acuity was 20/15 in the right eye and 20/60 in the left eye. A 2+ Marcus Gunn pupil was present on the left. The visual field was normal in the right eye, but there was a lower altitudinal defect in the left eye. The right disc was normal (Fig. 12), but the left disc showed classic changes of acute nonarteritic ischemic optic neuropathy (Fig. 13). Fluorescein angiography showed some focal pseudoangiomatous hyperplasia of left disc (Figs. 14 and 15) but was within normal limits of the right disc (Fig. 16). He was treated with a subtenon's Kenalog injection, placed on Coumadin for 6 weeks, and he then took aspirin, Journal of Clinical Neuro-ophthalmology Figure 12. Cast.' 3. N,'rmal right optic disc on Dt.'ct.'mbt.'r 17. 1975. two per day, for the following year. Visual function remained stable for the next 7-8 years. On Februarv 24, 1984, when he was 55 years old, he noted 'an abrupt painless blurring of vision in his right eye while driving. Within a few days this became stable. An examination on September 17, 1984, revealed a corrected acuity of 10/200 in the right eye and 20/40 in the left eve, Altitudinal field defects were now present in both eyes. The optic discs in both eyes showed the late changes of ischemic optic neuropathy (Figs. 17 and 18). Comment The patient showed only mild pseudoangiomatous hyperplasia of the left disc at onset, Figure 13. Case 3. Left eye on December 17, 1975, showing changes of acute ischemic optic neuropathy. June 1985 Smith Figure 14. Cast.' 3. Arterial-phase fluorescein angiogram of left optic disc on Decembt.'r 17, 1975. Note poor filling of nasal vt.'ssels on disc surface. confirmed by fluorescein angiography, but his case is described here because it documents the presence of a normal disc 8 years before onset of ischemic optic neuropathy in the right eye. It demonstrates the observation reported by Felt et al. 1 and Beck et al. 2 of a small, or absent, physiologic cup in an eye pr.ior to adocumented occurrence of nonarteritic IschemIC optic neuropathy. Figure 15. Clse 3. Lll,'-pi',IS" 11uor,'sn'lIl angiogram at" tt.'ti optic disc on lJel"l'lllb,'r 17. 1'175. N,.tt.' I"t" staining and som,' "It'v,llion of lower lelllf'oral ,ISf','.-t ot the disc. 85 Pseudohemangioma of Optic Disc Figure 16. Case 3. Normal fluorescein angiogram of right eye in 1975. Case 4 A 56-year-old white man was examined on July 13, 1982, by the author along with Dr. E. W. D. Norton. The patient's chief complaint was photophobia for 2 months and decreasing near vision for 2 weeks. The best corrected vision was 20/20 (]-1) in both eyes. However, lower nasal arcuate scotomas were noted in both eyes. His blood pressure was 125/75 and the applanation tension was 13 in the right eye and 12 in the left eye. The optic discs in both Figure 17. Case 3. Right eye on September 17, 1984, showing changes late after ischemic optic neuropathy. Compare with Fig. 12, which shows that this eye was quite normal 9 years before. 86 Figure 18. Case 3. Left eye on September 17, 1984, showing late changes of ischemic optic neuropathy. Note that the swelling seen 9 years before (Fig. 13) has cleared. The upper part of the disc is pale and the lower pole of the disc has some residual normal (pink) color. eyes showed changes consistent with ischemic optic neuropathy. The right eye is shown in Figs. 19-21 and the left eye in Figs. 22-24. Comment A 56-year-old man presented with bilateral nonarteritic ischemic optic neuropathy of recent onset in both eyes. The focal disc changes under consideration were present in mild degree in both eyes. This case is described to show that the process can be simultaneous in both eyes. tigure 19. Case 4. Right eye on July 13, 1982. Note swelling of lower temporal aspect of the disc (arrowhead). Journal of Clinical Neuro-ophthalmology Figure 20. Cast' 4. Artt'rial-phase fluroesct'in angiogram of right t'yt' on July 13. 1982. Note that the focal swelling is tilled with d,'t' (arrowhead). Case 5 A 73-vear-old white man was examined on April 23. 1984, by the author for Dr. Klingele. In September 1983, the patient awoke with painless blurring of vision in the left eye. Corrected acuity was 20/30 in the right eye and 201 50 in the left eye. An afferent pupil was noted on the left, altitudinal field defects were noted in both eyes, and both discs were swollen. He was admitted to Barnes Hospital on November 30, 1983, and treated with intravenous bolus steroids in high doses. He also had very narrow angles, for which a laser iridotomy was performed in the left eye. On the next day, the tension increased to 45 in the left eye and was controlled medically. Figure 21. Case 4. Late venous-phase fluorescein angiogram of right eye on July 13, 1982. Note that the lower temporal swelling fills and stains late (arrowhead). June 1985 Smith Figure 22. Case 4. Lt'ft eye on July 13, 1982. Note swelling of two areas of the disc (4-5:30 and 8-9 o'clock) (arrows). An examination in April 1984 revealed a corrected acuity of 20/30 in the right eye and 20/40 in the left eye. The blood pressure was 120178. The tension was 19 in both eyes by applanahon, and was 24 in both eyes after dilatation. The fundi revealed interesting changes thought to be consistent with pigmented paravenous chorioretinal atrophy in both eyes. There were also changes on the lower tempora~ margin. of the right disc thought to be consistent With the proud flesh syndrome; ~his was inte~preted as having followed recent IschemiC optic neuropathy in the right eye. The patient also had shallow anterior chambers and was status postlaser iridotomy in the left eye, which was considered probably not patent. Photographs of the Figure 23. Case 4. Earlier venous-phase fluorescein angiogram of left eye on July 13, 1982. Note that the two areas of staining of the disc are easier to see than in Fig. 23 (arrowheads). 87 Pseudohl.'mangioma of Optic Disc Figure 24. Case 4. Later-phase fluorescein angiogram of left eye on July 13, 1982. Note that the two areas of disc staining are easier to see than in earlier views (arrowheads). right disc and its fluorescein-angiographic appearance are seen in Fig. 23; the left eye is shown in Fig. 24. COlli III ell t A 73-year-old man presented with a complicated eye problem, having pigmented paravenous chorioretinal atrophy3 in both eyes and then experiencing an attack of ischemic optic neuropathy in the right eye, with a tendency to angle closure glaucoma as well. The author has seen a patient who suffered a classic bout of nonarteritic ischemic optic neuropathy during an acute attack of angle closure glaucoma, who was later treated by bilateral peripheral iridectomies. It is important to recognize that ischemic optic neuropathy can rarely be caused by angle closure glaucoma, as iridectomy may prevent the occurrence of the problem in the second eye in such cases. This case illustrates the fact that pseudoangiomatous hyperplasia can occur in patients who have other types of concomitant eye disease; its description can be helpful in establishing the diagnosis of ischemic optic neuropathy in similar clinical settings. Discussion I~ch~mic optic neuropathy is a very common entIty m neuro-ophthalmological practice and has become well recognized since it was first reported in 1966. 4 The form not associated with temporal arteritis is the most common. It is marked by the abrupt onset of painless blurring of vision in one eye in a middle-aged, ~ealthy adult. Central vision is often spared initIally, but there t~pically is a ratlwr profound altJtudmal vIsual tleld detl'ct, which usually is permanent. After a delay of as much as several years, the second eye often becomes symmetrically involved in as many as perhaps half of cases. Although the patients are usually in excellent general health, about one-half will haye mild hypertension and/or a family history of diabetes. It is important to reassure the patients that a second attack virtually never occurs in the same eye once the disease has stabilized (usually within 3 months of onset). The author may have seen the extremely rare exception to this rule in a recent patient of Dr. James Mitchell, but it remains a helpful clinical point. Hypertensive optic neuropathy5 may recur in the same eye and has some clinical similarities. The ophthalmoscopic criteria of ischemic optic neuropathy are of interest. Initially, a typical pallid swelling, originally termed "pseudopapillitis vasculaires" by Francois et al.,6 is seen, but this rapidly progresses into an atrophic disc, often showing narrow arterioles and segmental or altitudinal pallor. The sharply altitudinal atrophy and its related field defect have not, to my mind, been adequately explained, despite numerous investigations of blood supply of the optic nerve head. However, the point under discussion here relates to focal changes in the disc both early and late in the evolution of the acute disc swelling in these patients. One might consider the process simply as "luxury perfusion" of the disc. However, with careful Hruby lens study there are other aspects that need to be considered. What is being luxuriously perfused? One can see optic nerve disease presenting with simple hyperemia (i.e., va~ o?ilatation and prominence of normally preexIstIng small vessels on the disc margin). One can also see neovascularization on the disc ~argin or surface, which can be a proliferation ot rather normal-appearing small vessels or obviously abnormal-appearing microtelangiectatic or aneurysmal small vessels. One can also see c~mbined pr.olifera~ion of capillaries and glial tIssue, causmg a tocal swelling of the disc margin, as seen in some of the cases described here, and, uncommonly, the process can produce a notably elevated mass on the disc that can cIosel~ mimic a true hemangioma of the optic disc. I There are interesting questions that can be ask.ed ~bo.ut the pseudohemangioma of the optIc diSC tollowing ischemic optic neuropathy. Was the lesion in case 1, seen 6 months after the disc ischemia, a residual area of swelling from the original event, or had the swelling resolved and this tumefaction subsequently appeared? Interval observations were not available in case 1 to answer this question. The presump- Journal of Clinical Neuro-ophthalmology tion would be that this was truly a residual swelling from the original ischemic event, but why did the rest of the disc margin dear so rapidly and yet leave this impressive mass 11 months later? What is the nwchanism of production of this degree of proliferation of capillaries and glial tissue? Is it due to axoplasmic tlow stagnation? Is there a local action of angiogenesis induction factor operatin~ on the optic nerve in such cases? The cause of pseudohemangioma of the optic disc is unknown at this time, at least to the author. It is impl)rtant tl) examine these discs with the Hrubv lens to see the changes discussed, for they usuall\' are totallv missed by indirect ophtha·lmoscl)~"vand can be overlooked on routine fundus photographs unless 2 x magnification views of the discs are ordered. It is evident that one can differentiate the pseudohemangiomas l)f the optic disc from true capillary hemangiomas of the disc not only by a history of previl)us ischemia but also by the spontaneous disappearance of the pseudohemangioma over time. It is hoped that further study of the nature of these focal disc changes will help elucidate the pathogenesis of the rare in- June 1985 Smith stances in which the process can mimic a hemangioma. References 1. Fcit, R. H., TomsJk, R. L., and Ellenberger, c.: Structural f,lCtors in the pathogenesis of ischemic optic Ill'uropathy. Alii. I. Ophtha/mo/. 98: 105108, I'.1M. 2. Beck, R. W., Savino, P. J., Repka, M. X., Schatz, N. J., Jnd Sergol\, R. C. Optic disc structure in anterior ischemic optic neuropathy. Ophtha/I/ Ill/ogy 91: 1334-1337, 1984. 3. Skalka, H. W.: Hereditary pigmented paravenous rL'tinochoroidal atrophy. Alii I. Ophtha/ mol. 87: 286-291, 1979. 4. Miller, G. R., and Smith, J. L.: Ischemic optic neuropathy. Am. I. 0l'ht!ltl/mol. 62: 103- 115, 1966. 5. Smith, J. L., and Goldhammer, Y.: Hypertensive optic neuropathy. TrailS. AI/I. Acad. aphtha/mol. 0101. 79: 520-523, 1975. 6. Francois, J., Verriest, G., Neetens, A., De Rouck, A., and Hanssens, M. Pseudopapillitis vasculaires. AIIII. Owl. 195: 830-885, 1962. 7. Takahashi, T., Wada, H., lani, E., Nakamura, A., and Hiramatsu, K. Capillary hemangioma of the optic disc. I. Clill. Neliro-ophlha/l1Io/. 4: 159162, 1984. 89 |
