Situs Inversus of the Optic Disc

Situs Inversus of the Optic Disc

854 NOTES, CASES, INSTRUMENTS Fig. 3 (Givner). (a) Ghost cells in the subretinal fluid (low power), (b) Ghost cells in the subretinal fluid (high po...

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Fig. 3 (Givner). (a) Ghost cells in the subretinal fluid (low power), (b) Ghost cells in the subretinal fluid (high power). CONCLUSION

In considering a differential diagnosis in a young patient with a unilateral completely detached retina, one should bear in mind the

possibility of the condition being Coats' disease, 108 East 66th Street (21).


Coats, G.: Forms of retinal disease with massive exudation. Roy. London Ophth. Hosp. Rep., 17:440, 1907-1908. : Ueber Retinitis Exudativa (Retinitis Haemorrhagica externa). Arch. f. Ophth., 81:275, 1912. Elwyn, H.: Diseases of the Retina. Philadelphia, Blakiston, 1946, p. 148. Leber, T.: Die Krankheiten der Netzhaut. Graefe-Saemisch Handb. Gesam; Augenh., 7:1267, 1916.






Rochester, New York

' Situs inversus of the optic disc is that condition in which the disc appears to be in­ verted. The vessels arising from the disc come from the temporal side and pass nasally. The temporal half of the disc is full; it does not demonstrate the usual physiologic cup. After the vessels have left the disc, their distribution is normal. It has been theorized that this anomalous vascular pattern is due to a tilt of the disc which incorrectly de­ termines the order of the vessels.1

Limited reference to situs inversus has been made in the American literature. The fundus drawing that has probably been re­ ferred to more than any other is that by Hamblin. 2 ' 3 The legend identifies it as situs inversus of the left eye. It should be pointed out, however, that this can be correct only if the view is considered to be that obtained by indirect ophthalmoscopy. Photographs of the fundi in typical situs inversus are to be. found in articles by Rucker. 4 ' 5 In contrast to the American literature the German literature furnishes a detailed dis­ cussion of situs inversus. In 1882, in a classic paper concerning congenital anomalies of the optic nerve, E. Fuchs described for the first time the fre­ quent association of situs inversus with conus inferior.6



1953, with a presenting complaint of a tired feeling in her eyes on watching television. She had worn a correction for myopia for several years. Otherwise the ocular and sys­ temic histories were noncontributory. The uncorrected visual acuity was 18/200, O.D., and 19/200, O.S. With her glasses which had been prescribed two years pre­ viously ( - 1 . 7 5 D . sph., O.D.; —1.5D. sph., O.S.) the visual acuity was 20/40, O.D.; 20/30, O.S. With the addition of the mul­ tiple pinhole disc the visual acuity was 20/30, O.U. The near-point of accommodation was a normal 170 mm., O.U. External examination, including biomicroscopy, was normal. Cycloplegia was obtained by use of homatropine (five percent) and paredrine (one percent). Ophthalmoscopic findings, Ο.Ό., normal orange-red reflex. Media clear. Disc of normal color; no elevation; edges distinct. There was a well-defined inferior conus which was approximately one-third prism diopters in width and extended along the disc inferiorly from the 4- to 8-o'clock posi­ tion. The boundary between the conus and disc formed a horizontal line. The vessels arising from the disc came from the temporal side and passed nasally —the typical pattern of situs inversus. After leaving the disc, the vessels demonstrated a normal pattern. The macula was normal in appearance and showed a normal foveolar reflex. The supe­ CASE REPORT rior portion of the fundus was homogene­ The following case is submitted as a typical ously tessellated. However, that portion of example of this definite complex—situs in­ the fundus immediately inferior and nasal versus of the optic disc, inferior conus, de­ to the disc and extending for a distance of creased pigmentation inferior to the disc,, approximately three to four prism diopters different degrees of refraction in various from the disc showed a definite thinning of portions of the fundus, and tangent screen pigmentation. defects. No mention of this syndrome was O.S., similar to O.D.—that is, situs in­ found in a review of the standard ophthal­ versus, inferior conus, decreased pigmenta­ mic texts used by American ophthalmol­ tion inferior and nasal to the disc. However, ogists. the boundary between the inferior conus Mrs. C. A., a 37-year-old white woman, and the disc was the normally curved infe­ was first seen in the office on November 28, rior border of the disc.

In 1883, A. von Szily, Sr., brought at­ tention to two phenomena that frequently are found with conus inferior: (1) The lower half of the fundus has less pigmenta­ tion than that of the upper half and (2) the error of refraction of the lower half of the fundus is frequently greater than that of the macular region or temporal fundus.7 Myopia has been found in a majority of the cases. Conus inferior has also been re­ ferred to as Conus nach unten 7 - 8 and Fuchs Coloboma.9 It is located below and may ex­ tend either to the temporal or nasal side of the disc. Conus is believed to represent a congenital anomaly and is not progressive. It should not be confused with the more common myopic crescent, which is not congenital and usually is at least somewhat progressive. In addition, the myopic crescent is most fre­ quently located on the temporal side of the disc and in such cases there is no increased incidence, of situs inversus. ,In so-called myopia inversa,9 however, a myopic crescent does occur on the nasal side of the disc and in such cases there is a frequent occurrence of situs inversus. Rucker 4 - 5 has demonstrated the occur­ rence of temporal field defects for small targets on the tangent screen in both situs inversus alone and inferior conus alone. These defects have been attributed to dis­ tortion of the optic disc and possibly also defective choroid nasal to the disc.



The corrected retinoscopic findings were:

O.D, O.S.,

At Macula -2.2SD. -l.OOD.

In Area At Decreased Disc Pigmentation -4.S0D. - 8.2SD. -4.S0D. -10.00D.

Subjectively the patient accepted —2.5D. sph. for the right eye and — 1.5D. sph. for the left eye with a resultant corrected visual acuity of 20/30, O.U. Field studies revealed:

With the one-meter tangent screen and two-mm. and three-mm. white test objects there was a definite bitemporal field defect similar to that found by Rucker*· 5 in his cases. The isopters passed over the midline. without alteration of their course. The out­ line of the tangent screen defect rather closely resembled the pattern of the less pigmented area of each eye. 76 South Fitzhugh



1. Mann, I.: Developmental Abnormalities of the Eye. London, Cambridge Univ. Press, 1937, p. 123. 2. Ibid. 3. Walsh, F. B.: Clinical Neuro-ophthalmology. Baltimore, Williams & Wilkins, 1947, p. 417. 4. Rucker, C. W.: The interpretation of visual fields. Tr. Am. Acad. Ophth., 1948, p. 38. 5. Rucker, C. W.: Bitemporal defects in visual fields resulting from developmental anomalies of the optic disks. Arch. Ophth., 35 :S46-5S4, 1946. 6. Fuchs, E.: Beitrag zu den angeborenen Anomalien des Sehnerven. Arch. f. Ophth., 28:139, 1882. 7. von Szily, A.: Der Konus nach unten. Zentralbl. f. prak. Augenh., 7:3S8, 1883. 8. Duke-Elder, W. S.: Textbook of Ophthalmology, St. Louis, Mosby, 1938, v. 2, p. 134S. 9. Fuchs, A.: Myopia inversa. Arch. Ophth., 37:722-739, 1947.



Bronx, New York That ocular lesions exist in conditions as­ sociated with hypercalcemia has been ade­ quately recorded by many authors includ­ ing Cogan, Albright, and Barter, 1 Walsh and Howard, 2 and Walsh and Murray. 3 The mechanisms by which hypercalcemia is pro­ duced in hyperparathyroidism, Boeck's sarcoidosis, vitamin-D poisoning, chronic nephritis, and excessive calcium and alkali intake, are described at length in the monu­ mental work of Albright and Reifenstein.4 It would not seem worthwhile at this time to discuss them except to say that the ocular findings when noted in these conditions are a result of the hypercalcemia. Justification for making the present report * From the Ophthalmologic Service of the Leba­ non Hospital.

rests on the fact that (1) the globe was obtained for histologic study and (2) the interesting funduscopic picture was perhaps related to the other ocular conditions noted. The ocular findings repeatedly observed by many writers consist of conjunctival and corneal lesions—the former being minute glasslike nodules containing whitish flecks thought to be calcium phosphate; the latter taking the form of a band-shaped keratitis with the heaviest opacity at the limbus and shading off axialward. Howard and Meyer5 noted in a number of cases that the conjuctival lesions receded with improvement but that the keratopathy did not. Fleischner and Shalek6 were able to demonstrate calcific deposition on X-ray examination of the orbit in areas consistent with the clinical findings. Histologic studies described in the litera­ ture are meager and are confined to two biopsies of the nodular conjunctival lesions, and histologic examination of one globe from an individual who had taken milk and alkali in excess for many years for duodenal ucler. The globe had been removed because of an entirely incidental painful glaucoma.