Indian Journal of Ophthalmology

: 1967  |  Volume : 15  |  Issue : 3  |  Page : 114--116

Essential atrophy of iris

MS Nirankari, SS Sangha 
 Department of Ophthalmology, Medical College, Amritsar, India

Correspondence Address:
M S Nirankari
Department of Ophthalmology, Medical College, Amritsar

How to cite this article:
Nirankari M S, Sangha S S. Essential atrophy of iris.Indian J Ophthalmol 1967;15:114-116

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Nirankari M S, Sangha S S. Essential atrophy of iris. Indian J Ophthalmol [serial online] 1967 [cited 2022 Aug 13 ];15:114-116
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Full Text

This rare disease has been known in the past by various names such as co­rectopia, polycoria, diplocoria, tricoria, coloboma of iris, unilateral pupil doubling etc. The modern names like progressive essential iris atrophy Ga­zala (1960) or essential iris atrophy, hardly need any comments.

This condition was first described by Harms, who also included the cases of Johnson (1886) and Hess (1892). Duke Elder with whom many other authors agree think that the disease effects only one eye. But the fact that Czukrasz (1947) and Gazala (1960) have reported bilateral cases each, go to disprove this hypothesis. In the present communica­tion, the authors report a case of bilate­ral essential iris atrophy.

Case Report

S.S. 25 M. unmarried reported in the outpatient department on 6-7-64 with the complaints of painless, progressive loss of vision from the left eye for the last 8 years and right eye for 4 years. There was no history of redness in the eyes, no trauma, no family history suggestive of such a disease, no mus­cular weakness.

General Examination

The patient was well nourished and well built, without any muscular wast­ing. He had sparse hair in the region of the beard, whereas axillary and pubic hair were normal.

Nothing abnormal in the systemic examination was detected.

Examination of nose showed deflect­ed nasal septum and there was no other abnormality.

Examination of Eyes

RIGHT EYE: Vision Finger counting at 3 feet. The adnexa and lids were normal. Conjunctiva show­ed scarring in the palpebral re­gion. The corneal-diameter was 13.5 mm. No K.Ps, no tears in Descemet's membrane were visible. The anterior chamber was deep with no aqueous flare. Pupil was irregular and pulled towards 10 O'clock position See [Figure 1],[Figure 2]. No reaction to light or ac­commodation could be elicited.


It showed patches of atrophy at 1, 3, 7-O'clock positions and the atrophy involved the pigment as well as the stromal layers. At 7-O'clock position the atrophy resulted in a complete hole, about 2-3 mm. diameter, irregular in shape through which the lens as well as the fundus could be seen. No sphincter pupillae was present around this hole. Gonioscopy revealed peri­pheral anterior synechiae at 1, 3 & 7 O'clock positions. The rest of the angle was open, no pigment seen. Intraocular tension was 500 mm Hg Schiotz. The lens was normal. No synechia or pig­ment dispersal on the anterior surface was present.

Fundus-Disc showed glaucomatous cupping and atrophy, slight narrowing of the blood vessels and marked cho­roidal sclerosis.

LEFT EYE: There was a divergent squint of about 30˚-35˚ Adnexa, lids were normal, conjunctiva showed tra­chomatous scarring. Cornea was 13 mm in diameter and showed no pig­ment deposit on its back, no tears in the Descemet's membrane. Anterior chamber was deep with no aqueous flare; pupil was irregular and pulled towards 10 O'clock position See [Figure 1],[Figure 2]. Light and accommodation reactions were absent.


There were three atrophic patches at 11, 2 and 5 O'clock positions. At these places the pigment as well as the stroma were degenerated with com­plete hole formations through which the lens and the fundus could be seen. At the periphery of the 2 and 5 O'clock holes, the pigment layer was partially absent. He could not perceive light. Intraocular tension was 60 mm Schiotz.

Fundus : Almost the same picture as in the right eye. Gonioscopy: The angle was closed with peripheral anterior synechia adjacent to the holes. The rest of it was partially open. No pig­ment was seen.


E.S.R. 10 mm 1st hour Westergreen. Total leucocytic count: 7500, Poly­morphs 56%, lymphocytes 40%, mo­nonuclears 2% and Eosinophils 2%. Serological tests were negative. Blood Sugar: 80 mg%. Examinations of urine, stools and chest X-Rays were of no contributive value.


Cyclodialysis, iridectomy and punch trephining were done in both eyes.

The patient reported for check up on 22-12-64 and intraocular tension was 18 mm in both eyes. Again on 15-4-65, and 14-8-65 the tension was 20 mm Hg. in both eyes on each occa­sion. The vision in his right eye had been reduced to perception of hand movements.


Such divergent factors have been mentioned as the cause of such atro­phy that the etiology remains as ob­scure as ever. Among the causes men­tioned are: (1) some vascular change in the circulus iridis minor, (2) glau­coma, primary and secondary, (3) con­genital anomaly with corectopia, (4) abiotrophy, (5) reactivation of the pro­cess responsible for absorption of pu­pillary membrane, (6) Mechanical stretching of the blood vessels on op­posite sides. (7) Association with sym­pathetic disturbance.

There is no evidence in the present case that he had any disturbance like myotonia atrophica. We agree with Gazala (1960) that the association of essential iris atrophy and myotonia atrophica must have been coincidental. The etiology remains as obscure as ever.

In the present case there is nothing like pigment deposit over the anterior surface of the lens, posterior synechia, pigment in the angle, K.P.'s, aqueous flare to suggest that the disease could be secondary. In the absence of any stigma of inflammation of the anterior segment of the eye it can be presumed that the disease was of primary type.

The gonioscopic findings in our case, agree with those of the earlier workers (Troncoso, 1937; Sugar, 1945; Daily and Daily 1957; Lohlein, 1951). There were broad peripheral anterior syne­chia at the trabeculee, most marked ad­jacent to the site of the displacement of the pupil and atrophic patches. Rest of the angle was open and pigment was conspicuous by its absence. Slightly larger size of the cornea and eye ball appears to be due to the onset of glau­coma at an earlier age.

Regarding treatment, medical treat­ment cannot be trusted for long. Sclerotomy done early is said to be very useful and delays the progress of the disease. Waite (1938); Daily and Daily (1957) advocated simple iridec­tomy. Clarke (1960) reported failure with iridectomy. Although one year of observation is too short to draw any conclusion, our case is doing very well so far as maintenance of intraocular tension goes. It is hoped that the com­bination of iridectomy, cycloidialysis and punch trephining which was per­formed in this case will surely prove better for normalizing the tension.


A case showing bilateral essential atrophy of the iris is reported and a brief resume of the literature is given.[17]


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