764 AMERICAN JOURNAL OF OPHTHALMOLOGY OCTOBER, 1966

small degree at a later date, but the disease usually remains stationary. The inheritance is usually sex-linked recessive.10

Behr's complicated form was first de­scribed in 1909. Varying degrees of atrophy are associated with complications such' as ataxia, increased deep tendon reflexes, men­tal deficiency, bladder disturbance, hyper-tonicity, spastic gait, nystagmus, poor co­ordination, and club foot (Friedreich's type).1'2 The optic atrophy is always bilater­al and rarely complete. Both sexes are equally affected and it is transmitted as simple recessive.11

The late developing hereditary optic atro­phy is transmitted as a dominant gene and only a few cases have been reported.

Congenital or infantile hereditary optic atrophy has a severe recessive and milder dominant form. The recessive form occurs at birth or shortly thereafter. The optic atrophy is complete and the visual acuity is always bad.

Our family exhibited the dominant form (fig. 2) . This is a milder variety and de­velops gradually from infancy onward. The peripheral field is always normal and there is usually a scotoma that is central, paracen-tral or cecocentral. Our cases almost uni­formly showed a central scotoma without peripheral changes. Central vision varies greatly from hardly being affected to an acuity as low as 20/200. No hemeralopia is to be expected and this was true of our cases. The transmission is direct and equal in the two sexes.

SUMMARY

A family with infantile hereditary optic atrophy is presented. A brief discussion of this and allied entities is included.

Proctor Foundation University of California

Medical Center (94122)

REFERENCES 1. Francois, J., Heredity in Ophthalmology,

St. Louis, Mosby, 1961, pp. 497-512.

2. Cordes, F. C : Optic atrophy in infancy, childhood and adolescence, Amer. J. Ophth. 35:1272, 1952.

3. Mooney, A.: The color of the optic disc and its relations to various field defects. Tr. Ophth. Soc. U.K. 227, 1964.

4. Krauss, W.: Kongenitale hochgradige Verand-erungen an der Sehnerveneintrittsstelle. Klin. Mbl. Augenh. 63:125, 1920.

5. Scheie, H., and Adler, F. : Aplasia of the optic nerve. Arch. Ophth. 28 :61, 1941.

6. Jerome, B., and Forrester, H., Jr.: Congeni­tal hypoplasia and partial aplasia of the optic nerve. Arch. Ophth. 39 :669, 1948.

7. Henderson, J. W.: Present status of optic atrophy, Nat. Soc. Prev. Blindness, 1956, p. 44.

8. Ford, F. R.: Diseases of the Nervous Sys­tem in Infancy, 'Childhood and Adolescence. Springfield, 111., Thomas, I960, ed. 4, pp. 399-401.

9. Scott, J. G.: Hereditary optic atrophy with dominant transmission and early onset. Brit. J. Ophth. 25:461, 1941.

10. Bell, J.: Hereditary optic atrophy. Treasury of Human Inheritance. Cambridge, Engl, Univ. Press, 1931, v. 2, Part 4, p. 188.

11. Franceschetti, A.: Infantile optic atrophy associated with general disorders syndrome of Behr. Schw. Med. Wehnschr. 70 :285, 1940.

FUSION IN APHAKIC ANISOMETROPIA

W. S. MUENZLER, M.D. Oklahoma City, Oklahoma

Because its optical state is altered by aphakia, the eye is about 12 diopters hy-peropic, the same as if, near the center of the crystalline lens of the normal eye, an infinitely thin lens had been inserted, which had the power to just neutralize the crystal­line lens.1 Parallel rays of light would now focus about 31 mm posterior to the cornea, while the average anterior-posterior diame­ter of the eye is only 22.8 mm.2 Correction of the aphakic, previously emmetropic, eye with spectacle lenses results in an enlarge­ment of the retinal image of about 36%. As a convex lens is moved closer to the eye, the size of the retinal image is reduced and, with a contact lens is only about 11% en­larged (9%, Ogle, Burian and Bannon3;

From the Department of Ophthalmology, The University of Oklahoma Medical Center.

VOL. 62, NO. 4 NOTES, CASES, INSTRUMENTS 765

5.5%-9%, Dyer and Ogle4; 6.99%, Girard and associates5). However, in order to make the image size equal to that of a normal eye, the lens would have to be brought within the eye.

In 1906, Deloge6 reported two patients in whom binocular single vision was attained in monocular aphakia corrected by glasses. Berens, Connolly and Kern7 reported that they had been able to obtain binocular single vision in some of their young patients (two in their 20's were presented) by giving the strongest possible plus lens for the normal eye and slightly undercorrecting the aphakic eye, carefully adjusting the lenses. They mentioned that they had not been equally fortunate with the treatment of their adult patients. Recently,8 unilateral aphakia has been corrected by means of spectacle lenses adjusted in such a way that the distance to the back vertex of the lenses is different on the right and left.

Linksz9 mentions that spectacle correction of monocular aphakia with some 30% image size difference usually causes diplopia, con­fusion and serious visual difficulties but does not cause the type of eyestrain that an-iseikonia does. He also mentioned that Ed­ward Jackson corrected two patients with unilateral aphakia with regular spectacle lenses and they had no discomfort because they suppressed the image.

It is the purpose of this paper to present a patient with monocular aphakia following surgery for senile cataract who was found to have stereopsis and remarkable fusional amplitudes with spectacle lenses.

C A S E REPORT

M. S., a 63-year-old white woman, was seen initially on November 15, 1962, with the complaint of gradual, painless loss of vision in both eyes for the past few years. Her best corrected vision was R.E., 20/100 (+1.75D sph Z +1.0D cyl ax 30°) and L.E. 20/40 (+5.SD sph). Slitlamp revealed bilateral nuclear cataracts. A year later the vision, R.E., was light perception and projection. Exami­nation revealed an intumescent lens with a shallow anterior chamber. The vision L.E. was 20/40 with correction.

On Feburary 11, 1964, an intracapsular, round-pupil, lens extraction with peripheral iridectomy was performed without complication. Subsequently vision was R.E., 20/20 (+12D sph C +1.5D cyl ax 35°). Vision remained 20/40, L.E.

Three months later she returned wearing the correction just mentioned. She fused the Worth 4-dot near and far, and the second row of animals on the Titmus Stereotest (30% stereopsis). Or­thoptic examination revealed third-grade fusion (stereopsis) with the following amplitudes on the amblyoscope: —10/—5 to +40/+28 (distance)* without suppression.

Testing on the space eikonometer could not be evaluated because of her lack of understanding. When last seen, amplitudes on the amblyoscope were —6/+2 to +25/+14 (distance) and —10/—8 to +22/+12 (near).

DISCUSSION

Carlton and Madigan10 state that each 0.25 diopter difference between the refrac­tion of the two eyes causes a 0.5% difference in the size of the retinal images, and that a difference of 5% is probably the limit which can be tolerated with ease. The adult generally has difficulty wearing ordi­nary spectacles when the difference in the two eyes is greater than two diopters; how­ever, children with good fusion may tolerate differences much greater than this.

In the case presented a 14% difference in the image size is calculated. Dyer and Ogle3

point out that when the difference in retinal image size is greater than 5%, the periphery is suppressed and binocular vision is present only in the macular areas. Linksz8 refers to "instrument stereopsis" and does not believe that showing the faculty of binocular vision in office tests specifically designed to test fu­sion means that the patient has compulsive binocular vision under everyday circum­stances. It is known2 that, if both eyes are axially hyperopic by 5.0 diopters, the chance to fuse in monocular aphakia is increased. Perhaps then under ordinary surroundings this patient alternately suppresses; although, if this is true, of what value are our "in-

* Measured on the major amblyoscope using standard second-grade targets. The divergence amplitude was measured first.

766 AMERICAN JOURNAL OF OPHTHALMOLOGY OCTOBER, 1966

strument" estimations of fusion. On the other hand, this patient may be one of those rare individuals with a tremendous fusion faculty and the ability, at the perceptual level, to overcome great obstacles to stereop-sis.

SUMMARY

The case of a monocular aphakic patient who obtained fusion with ordinary spectacle lenses is reported. A brief discussion of the relationship of anisometropia to retinal image size is presented.

3434 N. W. 56th Street (73112)

ACKNOWLEDGMENT I wish to thank Dr. James E. Lebensohn, Chi­

cago, for his critical review of this paper.

REFERENCES 1. Ogle, K. N.: Optics. Springfield, 111., Thom­

as, 1961, p. 211.

2. Duke-Elder, W. S.: Textbook of Ophthalmol­ogy. St. Louis, Mosby, 1949, v. 4, p. 4368.

3. Ogle, K. N., Burian, H. M., and Bannon, R. E.: On the correction of unilaterial aphakia with , contact lenses. Am. J. Ophth. 59:639, 19S8.

4. Dyer, J. A., and Ogle, K. N.: correction of unilateral aphakia with contact lenses. Am. J. Ophth. 50:11, 1960.

5. Girard, L. J., Friedman, B., Moore, C. D., Blau, R. I., Binkhorst, C. D., and Gobin, M. H.: Intraocular implants and contact lenses: Monocu-larly aphakic patients. Arch. Ophth. 68:762, 1962. *

6. Deloge, C : These. Anisometropie et Vision Binoculaire. Paris, Libraire Medicale et Scien-tifique, 1906.

7. Bernes, C, Connolly, P. T., and Kern, D.: Certain motor anomalies of the eye in relation to prescribing lenses. Am. J. Ophth. 16:199, 1933.

8. Zoubeck, F., Polasek, J., and Hamak, V.: Anequidistant spectacles: Correction for binocular * vision in unilateral aphakia. Sborn. ved. Praklek. Fak. Hradci Kralove. 6:327 (Part II) , 1963.

9. Linksz, A.: Anisekonia. Am. J. Ophth. 48:441, 1959.

10. Carlton, E. H., and Madigan, L. F.: ' Size and shape of ocular images : II. Clinical significance. Arch. Ophth. 7 :720, 1932.

OPHTHALMIC MINIATURE

Keratoconus. Von Graefe, German ophthalmologist, 1828-1870, de­scribed a cone-shaped deformity of the cornea, known as keratoconus, in 1868.

Keratoplasty. Arthur von Hippel, German ophthalmologist, 1841-1917, developed a technique for keratoplasty which is the basis of the modern operation.

Keratoscope. Antonio Placido da Costa, Portuguese ophthalmologist, 1848-1916, introduced the use of the keratoscope. Reported in 1882. Known as Placido's disk

Schmidt, J. E.: Medical Discoveries Springfield, Illinois, Charles C Thomas, 1959, pp. 249-250