Bilateral Essential Atrophy of the Iris

Abstract

ESSENTIAL atrophy of the iris is a rare clinico-pathological entity; its aetiology is ill-understood, almost all cases reported have been unilateral, and treatment is of no avail. We report this case not only because it is rare, but also because bilateral cases are rarer still. This case, which appears to be closely allied to buphthalmos, like that of Barkan which was also bilateral (Duke-Elder, 1940), also appears to shed some light on the probable aetiology of this condition. Case ReportA male farmer, aged 26, was admitted to hospital on July 20, 1954, with a history of progressive blindness. The left eye had been blind for 3 years and the right for 3 months. Neither redness of the eyes nor ocuiar pain was a featuie of his illness. A slowly progressing baldness was the only extra-ocular complaint-this he had noticed for 5 years.Family History.-Two brothers and parents healthy. Examination.-A healthy muscular individual whose only extra-ocular defect was baldness of the frontal area of the scalp with inroads to the vertex. The skin over these areas was of normal texture; the hair, which was predominantiy black, was brownish over the vertex, and was greying at the sides. The Wassermann reaction was negative.Ophthalmic Investigation.-Both eyes were buphthalmic and executed a series of fine nystagmoid movements in the horizontal direction. Vision in each was perception of light. The corneal diameters were: Right horizontal 14 mm., vertical 13mm.; left hoiizontal 14 mm., vertical 14 mm.The ocular tension was 26 mm. Hg (Schiotz) in the right eye with pressule of 7 5 and 10 mg., and 44 and 37 mm. Hg (Schiotz) in the left eye. The left cornea was cloudy because of oedema of the epithelium and substantia propria. The anterior chambers were deep. The irides (Figs 1 and 2, overleaf) showed two shades of brown pigmentation, the paler areas having a washed-out appearance; there were multiple holes-pseudopolycoria. The pupils were eccentiic and ectropion uveae delineated their borders. Strands of atrophic iris tissue showing lines of traction radiated from the pupils. No contraction furrows, crypts or collarettes were seen. The irides looked as though they had been ironed out. The left iris was tremulous opposite the three holes below the pupil (Fig. 2). The pupils reacted sluggishly to light. There were no keratic precipitates or deposits of iris pigment on the back of the cornea.Through the slight dilatation that could be effected with homatropine 2 per cent. drops, the lenses were seen to be sclerosed but the fundi could not be seen.

Le glaucome par atrophie essentielle progressive de l’iris

“…Modifications générales: dystrophie myotonique (Lohlein, 1951), hypopituitarisme (de Ferrari, 1954;Lotin, 1939), troubles endocrins ou neuroendocrins (Larmande, 1953;Vancea, 1932), probablement syndrome Vogt-Koyanagi (Sivasubramanian et Hoole, 1955).…”