3
854 NOTES, CASES, INSTRUMENTS Fig. 3 (Givner). (a) Ghost cells in the subretinal fluid (low power), (b) Ghost cells in the subretinal fluid (high power). CONCLUSION possibility of the condition being Coats' In considering a differential diagnosis in a disease, young patient with a unilateral completely detached retina, one should bear in mind the 108 East 66th Street (21). REFERENCES 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. SITUS INVERSUS OF THE OPTIC DISC WITH INFERIOR CONUS AND VARIABLE MYOPIA : A CASE REPORT WILLIAM CHARLES CACCAMISE,, M.D. 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

Situs Inversus of the Optic Disc

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Page 1: 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 power).

CONCLUSION possibility of the condition being Coats' In considering a differential diagnosis in a disease,

young patient with a unilateral completely detached retina, one should bear in mind the 108 East 66th Street (21).

REFERENCES

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.

S I T U S I N V E R S U S O F T H E O P T I C D I S C

W I T H INFERIOR CONUS AND VARIABLE

MYOPIA : A CASE REPORT

W I L L I A M CHARLES CACCAMISE,, M.D. Rochester, New York

' Situs inversus of the optic disc is that condition in which the disc appears to be in­verted. 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 de­termines 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 re­ferred 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 dis­cussion of situs inversus.

In 1882, in a classic paper concerning congenital anomalies of the optic nerve, E. Fuchs described for the first time the fre­quent association of situs inversus with conus inferior.6

Page 2: Situs Inversus of the Optic Disc

NOTES, CASES, INSTRUMENTS 855

In 1883, A. von Szily, Sr., brought at­tention to two phenomena that frequently are found with conus inferior: (1) The lower half of the fundus has less pigmenta­tion 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 re­ferred to as Conus nach unten7-8 and Fuchs Coloboma.9 It is located below and may ex­tend 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 fre­quently 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.

Rucker4-5 has demonstrated the occur­rence 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 dis­tortion of the optic disc and possibly also defective choroid nasal to the disc.

CASE REPORT

The following case is submitted as a typical example of this definite complex—situs in­versus of the optic disc, inferior conus, de­creased pigmentation inferior to the disc,, different degrees of refraction in various portions of the fundus, and tangent screen defects. No mention of this syndrome was found in a review of the standard ophthal­mic texts used by American ophthalmol­ogists.

Mrs. C. A., a 37-year-old white woman, was first seen in the office on November 28,

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 sys­temic 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 pre­viously ( -1 .75D. 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 mul­tiple 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 biomi-croscopy, was normal.

Cycloplegia was obtained by use of ho-matropine (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 posi­tion. 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 supe­rior portion of the fundus was homogene­ously tessellated. However, that portion of the fundus immediately inferior and nasal to the disc and extending for a distance of approximately three to four prism diopters from the disc showed a definite thinning of pigmentation.

O.S., similar to O.D.—that is, situs in­versus, inferior conus, decreased pigmenta­tion inferior and nasal to the disc. However, the boundary between the inferior conus and the disc was the normally curved infe­rior border of the disc.

Page 3: Situs Inversus of the Optic Disc

856 NOTES, CASES, INSTRUMENTS

The corrected retinoscopic findings were: In Area

At At Decreased Macula Disc Pigmentation

O.D, -2.2SD. -4.S0D. - 8.2SD. O.S., -l.OOD. -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:

OCULAR FINDINGS IN HYPERCALCEMIA*

A CLINICAL AND HISTOLOGIC STUDY

VICTOR GOODSIDE, M.D. Bronx, New York

That ocular lesions exist in conditions as­sociated with hypercalcemia has been ade­quately recorded by many authors includ­ing Cogan, Albright, and Barter,1 Walsh and Howard,2 and Walsh and Murray.3 The mechanisms by which hypercalcemia is pro­duced in hyperparathyroidism, Boeck's sar-coidosis, vitamin-D poisoning, chronic nephritis, and excessive calcium and alkali intake, are described at length in the monu­mental 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 Leba­non Hospital.

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 out­line of the tangent screen defect rather closely resembled the pattern of the less pigmented area of each eye.

76 South Fitzhugh Street.

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 conjuc-tival 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 litera­ture 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.

REFERENCES

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.