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AMERICAN JOURNAL OF OPHTHALMOLOGY VOLUME 80 OCTOBER, 1975 NUMBER 4

ACUTE MACULAR NEURORETINOPATHY P I E R R E J . M. BOS, M.D.

Amsterdam, The Netherlands

AND

A U G U S T F . D E U T M A N , M.D.

Rotterdam, The Netherlands

Recently we observed four women with a peculiar acute macular affection, character-ized by slight depression of visual acuity and nararentral scotomas correfip™"ti"g w i t n

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The macular pigment epithelium appeared normal and the retinal vessels did not show gross abnormalities. The optic disk was un-affected and there were no nerve fiber de-fects. Visual recovery, when present, pro-gressed slowly over many months.

Since no similar disease process is known to us, we called this entity acute macular neuroretinopathy (neuroepitheliopathy)1 be-cause of the acute onset and the localization of the lesions in the more superficial retinal layers at the macula.

Two patients were seen in the Amsterdam Eye Clinic, one patient was seen in the Rotterdam Eye Hospital, and the fourth was examined at both hospitals.

Routine ophthalmoscopic examination, fundus photography, and fluorescein angiog-raphy were done in all patients. The photo-graphic methods have been described.2 Ki-netic visual fields were recorded with the Goldmann perimeter and static perimetry with the Tubinger perimeter.

From the Department of Ophthalmology, Univer-sity of Amsterdam (Dr. Bos), and the Eye Hospital, Erasmus, University of Rotterdam (Dr. Deutman), The Netherlands.

Reprint requests to A. F. Deutman, M.D., De-partment of Ophthalmology, University of Nij-megen, Nijmegen, The Netherlands.

573

We performed electroretinography and electro-oculography in two patients.2 All pa-tients underwent general physical examina-tion and laboratory tests.

CASE REPORTS

Case 1—A 29-year-old white woman had noticed black dots and scotomas in both central visual fields since April 15, 1971. The onset of the com-plaints was sudden. She was first seen on April 20, 1971, when corrected visual acuity in both eyes was 20/20.

Ophthalmologic examination showed no abnor-malities outside the macular regions. There were irregular reflexes and dark, brown-reddish flecks in the right macula; some had a triangular configura-tion with the sharp point of the triangle directed toward the fovea (Fig. 1, left). Some polymorphic spots were present elsewhere in the macula. In her left eye (Fig. 1, right), she demonstrated similar irregular reflexes and some dark red-brownish dots, like those in her right eye.

Biomicroscopy revealed that these spots were not located in the pigment epithelium but in the more superficial retinal structures. Fluorescein angio-graphy was normal (Fig. 2). Kinetic perimetry showed that the peripheral boundaries of the visual fields were intact, while static perimetry demon-strated dense scotomas in the 2- to 5-degree area of both eyes and a slight decrease in central light sensi-tivity (Fig. 3).

There were no systemic complaints. In her youth the patient had had a splenectomy because of famil-ial thrombocytopenia, but otherwise she had a non-contributory medical history. She was taking the oral contraceptive lynestrenol (Lyndiol).

No subjective changes were observed during fol-low-up and static visual fields were unchanged. The fundus changes were visible, although the lesions were less obvious.

Case 2—A 33-year-old white woman noticed a scotoma in her left eye of sudden onset on Sept. 18, 1972. Her right eye was completely normal. She had not been ill before the ocular complaints. She had been using lynestrenol for eight years.

574 AMERICAN JOURNAL OF OPHTHALMOLOGY OCTOBER, 1975

Fig. 1 (Bos and Deutman). Case 1. Right macula (left) and left macula (right) showing darkish wedge-shaped and irregular flecks, localized in the neuroretina.

Fig. 2 (Bos and Deutman). Case 1. Fluorescein angiography of the right macula showing a normal fluorescence pattern in the arteriovenous phase.

Two days after the onset of symptoms, she had normal central vision in both eyes. There was a dark brown-reddish spot in the macular region of the left eye close to the fovea (Fig. 4, left). Other-

wise her eyes were normal. Fluorescein angiography revealed some question-

ably dilated perimacular capillaries (Fig. 4, right). There was, however, no leakage of dye recorded in the late stages of the angiogram, such as seen in cystoid macular edema.3

Meticulous static perimetry revealed a scotoma in the 1- to 3-degree area from the center of the left eye (Fig. S). This scotoma was not as dense as the scotoma recorded in Case 1.

The patient was last seen in October 1973, when the symptoms were unaltered. Careful ophthal-moscopy showed a faint discoloration located where the dark spot was seen originally. Static perimetry still showed a scotoma that had decreased slightly in depth (Fig. 6).

Case 3—A 24-year-old white woman noticed dis-turbance of her central vision after visiting a game reserve where she had had a bout of enteritis. She claimed she had not been looking at the sun.

On March 2, 1972, she was seen in the Amster-dam University Clinic. Visual acuity in both eyes was 20/20. Ophthalmologic examination was nor-mal outside the macular regions, which had a swollen glossy appearance with increased reflexes and vague, darkish red, wedge-shaped lesions around the center of the macula. The lesions pointed toward the fovea with a butterfly-shaped appearance (Fig. 7>-Fluorescein angiography revealed no abnormali-

Fig. 3 (Bos and Deutman). Case 1. Static perimetry (top and bottom) performed with the Tiibinger pe-rimeter showing slightly decreased light sensitivity centrally and paracentrally in the right eye (top) and dense paracentral scotomas in the left eye (bottom). These scotomas were also seen with kinetic perimetry (center) in the left eye.


Fig. 4 (Bos and Deutman). Case 2, left eye. Macula showing one darkish, wedge-shaped dot supero-nasally to the fovea. Fluorescein angiography of this macula (right) showed no clear abnormalities. There is only questionable dilatation of some of the perimacular capillaries.

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Fig. 5 (Bos and Deutman). Case 2, left eye. September 1972. There is a dense, well-delineated, paracen-tral scotoma corresponding to the side of the ophthalmoscopically visible dot. This scotoma was re-corded with the Tubinger perimeter after painstaking care in static perimetry.

Fig. 6 (Bos and Deutman). Case 2, left eye. May 1973. Left macula showing some remnants of the para-central scotoma shown in Figure 5.

VOL. 80, NO. 4 ACUTE MACULAR NEURORETINOPATHY 577

Fig. 7 (Bos and Deutman). Case 3. March 2, 1972. Right macula (left) and left macula (right) dem-onstrating wedge-shaped lesions not unlike the shape of a butterfly. However, these lesions are localized more superficially in the retina than in butterfly-shaped pigment dystrophy and they are not pigmented.

ties (Fig. 8). Static perimetry, however, showed a paracentral scotoma for maximal luminance above the center in the right eye (Fig. 9). No scotoma was seen in the left eye.

On March 29, she was seen at the Rotterdam Eye Hospital. Visual acuity in both eyes was 20/20. The Amsler grid test showed three small scotomas, two temporal to the fixation point in the left eye. Ophthalmologic examination revealed abnormalities at the macula that appeared swollen and glossy, with increased reflexes and vague, darkish red, wedge-shaped lesions around the center of the macula. The lesions pointed toward the fovea with a butterfly-like appearance. The ophthalmoscopically abnormal areas appeared to correspond almost com-pletely with the scotomas demonstrated on the Amsler grid.

On April 4, 1973, there was little subjective change (Fig. 10). Electroretinography and electro-oculography were performed and appeared to be completely normal. The electroretinogram (ERG) responses were normal. Scotopic and photopic a and b waves were well above the lower limits of normal. The electro-oculogram (EOG) showed a normal light/dark ratio of 2.74 in the right eye and 2.85 in the left eye.

There were no systemic complaints. This patient was taking the oral contraceptive norgestrel (Eugynon).

In October 1973, there were no subjective changes. The same characteristic fimdus changes were observed, although they were more faint. Angiography was performed, and was normal. Peri-metric details were the same as in March 1972 (Fig. 9).

Case 4—A 32-year-old white woman noticed scotomas in both eyes during a bout of influenza

and high fever in the middle of December 1972. When seen on Jan. 2, 1973, visual acuity was R.E.: 20/25, and L.E.: 20/66. Visual acuity in the left eye had always been slightly worse than in her right eye, probably due to amblyopia.

On Jan. 9, her visual acuity was unchanged. The Amsler test demonstrated a paracentral scotoma on the temporal side of the fixation point in the right eye and a rather large scotoma temporal to the center in the left eye. Both macular areas appeared

Fig. 8 (Bos and Deutman). Case 3. Fluorescein angiogram of the right macula revealing no ab-normalities in the arteriovenous phase.


Fig. 9 (Bos and Deutman). Case 3, right eye. Absolute paracentral scotoma in the 45- to 225-degree axis with kinetic perimetry (top) and static perimetry (bottom).

edematous with increased reflexes. There was one dark-reddish, triangular zone in the right macula, just nasal to the foveola (Fig. 11, left), and at least three wedge-shaped darkish-red lesions point-ing to the center in the left eye (Fig. 11, right).

The nerve fiber layer was more pronounced nasal to the center. Otherwise the ocular examination showed no pathologic findings. Vessels, disk, and retinal periphery were normal in both eyes and the media were clear.

Fluorescein angiography demonstrated an intact retinal pigment epithelium. There were some ques-

tionably dilated perimacular capillaries above the fovea (Fig. 12, top left), but there were no fluor-escein leaks (Fig. 12, top right and bottom left).

With the Goldmann contact lens, the retinal swelling appeared to be localized to the inner portion of the sensory retina. The patient was hos-pitalized on Jan. 16, when vision had not improved. A general physical and laboratory examination re-vealed a slightly abnormal glucose tolerance test. There was no glycosuria. She had hypercholes-terolemia (9.9 mM/1; normal, 6.4 mM/1). She had used contraceptives for many years. The patient


Fig. 10 (Bos and Deutman). Case 3. April 4, 1973. Right macula (left) and left macula (right) with the wedge-shaped paracentral dots that are more visible in tht right eye than in the left eye.

received one tablet of xanthinol niacinate (Com-plamine), twice daily, carbon dioxide inhalation, clofibrate capsules, and a diet. A neurologic ex-amination, including x-ray films of the sella turcica, was normal.

On Jan. 18, central visual fields showed a small paracentral scotoma on the superotemporal side of the fixation (Fig. 13). On Jan. 29, visual fields were unchanged, but by May 11, there was a small but definite decrease in the size and depth of the sco-tomas (Fig. 14).

Electroretinograms performed on Jaa 17, with diffuse and macular stimulation, were normal. The visually evoked cortical potentials were also normal.

On May 4, visual acuity was R.E.: 20/25, and L.E.: 20/50. Subjectively there was not much change, although the left eye had improved slightly.

On June 27, visual acuity was R.E.: 20/22, and L.E.: 20/40. She complained of seeing flecks close to the center in the right eye and paracentral and temporal flecks in the left eye. The maculae ap-peared swollen although the swelling had subsided.

Fig. 11 (Bos and Deutman). Case 4. Acute macular neuroretinopathy. Clearly visible darkish wedge-shaped lesions are in the superficial part of the retina. In the left eye (right), these lesions are doverleaf shaped and the nerve fiber layer demonstrates an increased visibility.


On Nov. 19, visual acuity was R.E.: 20/20, and L.E.: 20/33. The paracentral scotomas were present and caused considerable disability.

DISCUSSION

This disease appears to be a distinct entity that, to our knowledge, was described only in a short communication.1

It is different from the many clinical dis-eases affecting the central f undus, recognized in recent years and differentiated from one another based on the nature of the disease and the accompanying morphologic changes.

Central serous choroidopathy4 is one such

Fig. 12 (Bos and Deutman). Case 4. Fluorescein angiography of the left macula (top) shows ques-tionably dilated perimacular capillaries on top oi the macula. However, there is no sign of fluorescein leakage in the late phases of angiography. Bottorr left, The right macula also has a normal fluores-cence pattern.

entity that is well known but poorly under-stood. It is probably caused by dilation anc profusely leaking capillaries of the centra choriocapillaris.

Acute posterior multifocal placoid pig ment epitheliopathy is another of these enti ties.5 Although some authors5 think this dis ease process is due to pigment epithelia disease, there are arguments in favor of ; primary affection of the choriocapillaris. In both diseases the morphologic change occurring in the pigment epithelium ar probably secondary.

The changes in acute retinal pigment epi


theliitis7*8 appear to be nearly completely restricted to the pigment epithelium and this disease has a more favorable course, leading to complete recovery in six to ten weeks. There are no cells in the vitreous body or in the anterior chamber. A viral inflammation probably causes this self-limiting condition.

Serpiginous (geographic) choroiditis1'9

presents lesions at different stages of evolu-tion. In early lesions there is a definite pale

swelling of the pigment epithelium while older lesions demonstrate atrophy of the pigment epithelium and choriocapillaris sur-rounded by an edematous retinal zone. This inflammatory disease is characterized by relentless progression, presents in patients in their fourth decade and, in our experience, responds well to corticosteroids.

The so-called presumed histoplasmic cho-roiditis represents a distinct clinical entity

Fig. 13 (Bos and Deutman). Case 4. January 1973. Central visual fields demonstrating dense paracentral scotomas on the superonasal side of the center. Top, right eye; bottom, left eye.

Fig. 14 (Bos and Deutman). Case 4. May 1973. Central visual fields demonstrating some improve-ment. The scotomas are slightly smaller and less dense than in January 1973. Top, right eye; bottom, left eye.

582 AMERICAN JOURNAL OF OPHTHALMOLOGY OCTOBER, 197S

Fig. IS (Bos and Deutman). Case 4. Wedge-shaped lesions, shaped not unlike the lesion in acute macular neuroretinopathy, in a patient with intra-retinal hemorrhages due to hypertensive retinopathy.

affecting adults, characterized by subretinal neovascularization at the fovea. We saw this ocular syndrome in patients without evidence of previous infection by Histoplasma capsu-latum.1

The disease we present appears to be local-ized to the more superficial retinal layers, shown well on binocular slit-lamp examina-tion, while fluorescein angiography displayed no choroidal or retinal pigment epithelial abnormalities. The retinal vessels do not show definite abnormalities and do not leak fluorescein. The nerve fiber layer is not obviously affected since no nerve fiber layer defects were disclosed even after extensive perimetric examination.

There was no diffuse retinal disturbance since, in those patients tested, the ERGs and EOGs were normal. Static perimetry dem-onstrated a well-localized paracentral retinal disease with dense scotomas. The lesions were located predominantly on the nasal side of the fovea. The wedge-shaped lesions were shaped not unlike certain intraretinal hemorrhages (Fig. 15) and, therefore, may be localized at the same level in the neuro-retina.

As in all macular diseases without striking ophthalmoscopic changes, optic neuropathy has to be differentiated. However, there are no macular changes in optic neuropathy and partial or complete optic atrophy occurs in time. The visual fields and the macular changes together with the normal appearance of the optic disk indicated macular pathology in all four cases. In Case 4, optic nerve pathology was excluded by normal, visually evoked cortical potentials and by a normal neurologic examination.

Addendum—Recently one of us (PJ.M.B.) examined a 30-year-old white woman who developed acute macular neuro-retinopathy a few weeks after an influenza-like disease. Visual acuity was R.E.: 20/20, and L.E.: 20/100. The maculae showed wedge-shaped lesions in the superficial retinal layers (Fig. 16). Paracentral scotomas were elicited. Color vision was normal. After a few weeks visual acuity was 20/20 in both eyes. However, four months after the visual symptoms started, she still had paracentral scotomas. No systemic abnormalities were found. This patient used the oral contracep-tive ethinyl estradiol (Neogynon).

Another patient, a 23-year-old white man who developed visual problems in his right eye after a bout of influenza, presented with a mild form of acute macular neuroepithe-liopathy. Visual acuity was R.E.: 20/25, W L.E.: 20/20. Nasally to the right fovea there was a darkish dot in the superficial retinal layers (Fig. 17). Fluorescein angiog-raphy did not show any abnormality. Color vision was also normal. Perimetry, however, demonstrated a small and dense visual field defect at the site of the ophthalmoscopically visual lesion. The left eye was completely normal. The macular picture did not change during two months of observation.

SUMMARY

An unrecognized acute macular affection occurred in four women, 24 to 35 years old, using oral contraceptives who com-

VOL. 80, NO. 4 ACUTE MACULAR NEURORETINOPATHY

plained of a sudden decrease of visual acuity or paracentral scotomas.

Three patients had bilateral lesions and one patient had unilateral lesions. These lesions consisted of darkish brown-red, wedge-shaped dots in the macula pointing to the fovea. These dots were located mostly on the nasal side of the macula.

Biomicroscopy showed these lesions were located in the superficial layers of the retina.

Fig. 16 (Bos and Deutman). Right and left mac-ula (top and bottom, respectively) of a 30-year-old white woman shows the typical superficial wedge-shaped lesions of acute macular neuroretinopathy. The wedges point to the center of the fovea.

Fig. 17 (Bos and Deutman). A small darkish dot on the nasal side of the right fovea corresponding with a deep paracentral scotoma as the only mani-festation of acute macular neuroretinopathy.

The retinal vessels, pigment epithelium, and optic disk showed no distinct pathologic features. Fluorescein angiography, per-formed repeatedly, showed some question-ably dilated perimacular capillaries without leakage in two cases.

Static perimetry delineated dense para-central scotomas. Recovery was slow or absent, confirmed by perimetric observation.

Ophthalmoscopic, fluorescein angiography, and perimetric details excluded an affection of the pigment epithelium, the nerve fiber layer, and the optic disk. Since the affection appears to be localized superficially in the retina, we called this specific entity acute macular neuroretinopathy.

ACKNOWLEDGMENTS

H. H. A. H6tte, M.D., and R. A. Crone, Ph.D., referred Case 3, and H. E. Henkes, Ph.D., referred Case 4.

REFERENCES

1. Deutman, A. F.: Fluorescein angiography in macular diseases. In Henkes, H. E. (ed.) : Pho-tography, Electro-Ophthalmology and Echo-Ophthalmology in Ophthalmic Practice, vol. 3. The Hague, W. Junk, 1973, pp. 208-209.

2. : The Hereditary Dystrophies of the Posterior Pole of the Eye, thesis. Assen, The Netherlands, Van Gorcum and Co., 1971.


3. Gass, J. D. M., and Norton, E. W. D.: Cystoid macular edema and papilledema following cataract extraction. Arch. Ophthalmol. 76:646, 1966.

4. Gass, J. D. M.: Pathogenesis of disciform de-tachment of the neuroepithelium. 1. General con-cepts and classification. Am. J. Ophthalmol. 63:573, 1967.

5. : Acute posterior multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 80:177, 1968.

6. Deutman, A. F., Oosterhuis, J. A., Boen-Tan, T. N., and Aan de Kerk, A. L.: Acute posterior multifocal placoid pigment epitheliopathy. Br. J. Ophthalmol. 56:863, 1972.

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

8. Deutman, A. F.: Acute retinal pigment epitheliitis. Am. J. Ophthalmol. 78:571, 1974.

9. Schlaegel, T. F., Jr.: Essentials of Uveitis. London, Churchill, 1969, pp. 101-103.

OPHTHALMIC MINIATURE

He had only one good eye. The left distinguished only light and shade. But the good eye was dark-bright, full of observation through the over-hanging hair of the brow as in some breeds of dog. For his height he had a small face. The combination made him conspicuous.

Saul Bellow, Mister Sammler's Planet New York, Viking Press, 1970

ajopht 1975 bos

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