ACUTE MACULAR NEURORETINOPATHY

IRA. A. PRILUCK, M.D., H E L M U T B U E T T N E R , M.D., AND

D E N N I S M. ROBERTSON, M.D. Rochester, Minnesota

In 1975, Bos and Deutman1 described a newly recognized macular disorder, called acute macular neuroretinopathy. It is characterized by mild visual impair-ment, paracentral scotomata correspond-ing to wedge-shaped parafoveal retinal lesions, and normal fluorescein angiogra-phy. A single case report of this disorder occurring in the United States was recent-ly published by Rush.2 We describe here-in another case of acute macular neurore-tinopathy.

C A S E REPORT

A 17-year-old boy came to the Ophthalmology Department of the Mayo Clinic with a two-day history of bilateral central "bluish-green shadows." During the previous week, he complained of flu-like symptoms, including a sore throat and fever. His past medical history was otherwise noncontributory. He had not taken any systemic medications.

Ophthalmic examination revealed visual acuity of 6/6 (20/20) in both eyes. The patient was able to reproduce his bilateral and relative scotomata on the Amsler grid (Fig. 1). External examination, intrao-cular pressures, and slit-lamp biomicroscopy of the anterior segments and vitreous were normal. Kinetic perimetry confirmed the paracentral defects and showed intact peripheral visual fields (Fig. 2, top). Static perimetry through two of the defects demon-strated dense scotomata (Fig. 2, bottom).

Ophthalmoscopic examination using contact lens biomicroscopy of the right fundus showed an irreg-ular dark appearing disturbance of the deep sensory retina just nasal to the fovea. The left fundus showed similar and more numerous lesions located in the deep sensory retina. These disturbances were best seen by using red-free light (Fig. 3). Except for a small intraretinal hemorrhage located inferior to the left macula, the retinal vasculature, retinal pig-

From the Department of Ophthalmology, Mayo Clinic, and the Mayo Medical School, Rochester, Minnesota. This study was supported in part by a Fight for Sight Research Fellowship (Dr. Priluck), Fight for Sight, Inc., New York, New York.

Reprint requests to Ira. A. Priluck, M.D., Creighton University School of Medicine, 601 N. 30th St., Omaha, NE 68131.

ment epithelium, and nerve fiber layer were normal in the areas of these lesions.

Fluorescein angiography revealed subtle areas of relative hypofluorescence corresponding to the le-sions of the deep sensory retina (Fig. 4). Electrore-

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Fig. 1 (Priluck, Buettner, and Robertson). Amsler grid demonstrating parafixational scotomata of right eye (top) and left eye (bottom).

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776 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER, 1978

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Fig. 2 (Friluck, Buettner, and Robertson) Tangent screen visual field testing (top) and static Goldmann perimetry (bottom) along the 105-degree meridian (left eye) and the 75-degree meridian (right eye) demonstrating dense paracentral scotomata. The dotted line represents the normal static perimetry through the respective meridians.

tinography, eleetro-oculography, dark adaptometry, and Farnsworth-Munsell color vision testing were all normal.

A follow-up examination three months later re-vealed persistent paracentral scotomata, greater in the left eye than the right eye. Visual acuity was 6/6 (20/20), both eyes, and defects on Amsler grid testing remained essentially unchanged. Ophthal-moscopie examination revealed the same subtle disturbances of the deep sensory retina, left eye greater than right eye. The previously noted para-foveal intraretinal hemorrhage of the left macula had resolved. Repeat fluorescein angiography again demonstrated the minimal parafoveal areas of hypo-fluorescence.

DISCUSSION

Our case exhibits all the characteristic ocular findings of acute macular neurore-tinopathy as previously described by Bos and Deutman,1 and Rush.2 Our patient had a recent history of an upper respira-tory infection, presumably of viral origin, and complained of minor visual impair-ment. Significant ocular findings were limited to wedge-shaped parafoveal le-sions radiating peripherally from the macular regions, which corresponded to reproducible paracentral scotomata on the Amsler grid. Both static and kinetic

Fig. 3 (Priluck, Buettner, and Robertson). Left eye, Stereoscopic photographs taken with red-free light. Posterior pole shows dark cuneate parafoveal lesions located in the deep sensory retina (viewed best with + 7.00 diopter lenses).

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Fig. 4 (Priluck, Buettner, and Robertson). Top, Arterial venous phase of fluorescein angiogram of left eye demonstrates subtle areas of hypofluores-cence, which correspond to the disturbance of the deep sensory retina. Bottom, Late venous phase of angiography shows persistence of these hypofluo-rescent areas (arrows).

perimetry confirmed the presence of these scotomata. Retinal function studies were otherwise normal.

The fluorescein angiographic findings are unlike those seen with acute disorders of the choriocapillaris or retinal pigment epithelium, such as acute posterior multi-focal placoid pigment epitheliopathy3 or acute retinal pigment epitheliitis. Defi-

nite but subtle findings of hypofluores-cence probably represent a blockage of the underlying choroidal fluorescence by the visible lesions located at the level of the deep sensory retina. We are not certain whether the small intraretinal hemorrhage observed in the left eye was a coincidental finding or indicative of small vessel disease. Bos and Deutman1 report-ed somewhat dilated perimacular capil-laries in two of their cases. It is not certain that an abnormality of the retinal capillar-ies is of significance in this disorder.

The retinal lesions were best seen by using red-free light and contact lens bio-microscopy. We believe the deep sensory retina, and specifically the photoreceptor cell layer, to be the principal site of involvement in this disease. Functional impairment as evidenced by dense para-central scotomata is consistent with the interpretation that the photoreceptor cell layer is of pathophysiologic significance. Contact lens biomicroscopy demonstrat-ing the defects to be at the level of the sensory retina also supports this supposi-tion.

All previously reported cases1'2 have been associated with a recent infectious process. Our case further supports this observation. Bos and Deutman1 reported six cases, five of which were in females, and of these, four were taking oral contra-ceptives. Additionally, the patient de-scribed by Rush2 was also using oral contraceptives. However, our patient and the one male described by Bos and Deutman1 make it difficult to implicate sex predilection or oral contraceptives with acute macular neuroretinopathy.

SUMMARY

A 17-year-old boy had minor visual impairment, paracentral scotomata, and parafoveal retinal lesions following a pre-sumed viral infection. Contact lens bio-microscopy demonstrated the retinal de-

778 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER, 1978

fects to be located at the level of the deep sensory retina. Fluorescein angiography revealed subtle areas of hypofluorescence which corresponded to the visible distur-bances of the sensory retina. Follow-up examination three months later revealed persistent complaints of paracentral sco-tomata and persistent, but less recogniza-ble retinal lesions. We believe the pri-mary site of involvement to be the deep sensory retina.

O P H T H A L M I C MINIATURE

The little children were in a pitiable condition—they all had sore eyes, and were otherwise afflicted in various ways. They say that hardly a native child in all the East is free from sore eyes, and that thousands of them go blind of one eye or both every year. I think this must be so, for I see plenty of blind people every day, and I do not remember seeing any children that hadn't sore eyes. And, would you suppose that an American mother could sit for an hour, with her child in her arms, and let a hundred flies roost upon its eyes all that time undisturbed? I see that every day. It makes my flesh creep. Yesterday we met a woman riding on a little jackass, and she had a little child in her arms; honestly, I thought the child had goggles on as we approached, and I wondered how its mother could afford so much style. But when we drew near, we saw that the goggles were nothing but a camp-meeting of flies assembled around each of the child's eyes, and at the same time there was a detachment prospect-ing its nose. The flies were happy, the child was contented, and so the mother did not interfere.

Mark Twain, Innocents Abroad New York, Harper and Harper, 1911

REFERENCES

1. Bos, P. J. M. and Deutman, A. F.: Acute macu-lar neuroretinopathy. Am. J. Ophthalmol. 80:573, 1975.

2. Rush, J. A.: Acute macular neuroretinopathy. Am. J. Ophthalmol. 83:490, 1977.

3. Gass, J. D. M.: Acute posterior multifocal plac-oid pigment epitheliopathy. Arch. Ophthalmol. 80: 177, 1968.

4. Krill, A. E., and Deutman, A. F.: Acute retinal pigment epitheliitis. Am. J. Ophthalmol. 74:193, 1972.