Essential Progressive Iris Atrophy : Case of Bilateral Occurrence

Abstract

While reports of this unusual condition have become more frequent in the past few years, its occurrence bilaterally is so unique as to merit special mention. \Ve have been able to assemble from the literature twenty-three cases of the type of iris atrophy here described. Of these, the three earliest, although their descriptions suggest a strong similarity to the disease under consideration and although they are generally included in bibliographies as authentic cases, probably do not fall into the essential group. The first of these is an adequate report by johnson' in 1886, of a case of progressive iris atrophy in one eye following two years after injury and enucleation of the fellow eye. Hess (1892) provides the only report of a bilateral progressive atrophy of the iris, with hole formation and secondary glaucoma, which occurred in a boy of fourteen years. He commented, however, that there had been a chronic iridocyclitis following scarlet fever at the age of eight years, and described deposits on the lens and anterior synechiae to confirm such a view. The iris atrophy he believed to be the result of a chronic inflammation. Harms (1903) described a case which was closely analogous to that of Hess, except that it was unilateral. There was a progressive iris atrophy, hole formation, and hypertension; many fine brown deposits on the surface of the cornea, the author believed, pointed to a low-grade iridocyclitis, similar to that in Hess's case. The articles by de Schweinitz' and Waite 6 afford the most complete descrip*From the Department of Ophthalmology, Stanford University Medical School tions of the disease. The unilateral occurrence of progressive iris atrophy is cited as one of the characteristics. The disease occurs twice as often in females as in males. The age incidence is from five years (in our patient) to fifty-four years (Griscom'), but the majority of the cases are in early adult life. Most commonly there is early a progressive atrophy of the peripheral portions of the iris, with exposure of the underlying pigment layer. Later, formation of holes occurs and the fibers of the sphincter are involved. The pupil may become eccentric before the formation of any hole. Finally there ensues a secondary glaucoma which fails to respond to any of the usual operative procedures. In the few instances in which anatomic examination was performed, the secondary glaucoma was referred to the dense peripheral anterior synechiae which were found and to the liberation of large quantities of pigment (Rochat and Mulder/o Ellett/ Wood Licsko).8 Waite dissents from this opinion and expresses the belief that the hypertension is directly related to the iris atrophy, with reduction of the capillary bed, and not to the anterior synechiae. Our patient is a boy of nine years who at the age of five years was first examined in the Eye Clinic of the Stanford University Hospitals in June, 1933, because of a deformity of the pupil which the parents had observed for three or four months previously. Examination. Vision in the right eye was 15/100---1, in the left eye 15/30-3. Normal iris markings were absent, the stroma was very thin, and in places almost transparent. Polycoria was present.