Coloboma of the Iris, Polycoria, 
AND 
Primary Glaucoma. 
By G. E. de SCHWEIN1TZ. M.D., 
Of Philadelphia. 
[Reprinted from American Ophthalmological Society Transactions, 1891.] [Reprinted from American Ophthalmological Society Transactions, 1891. 
COLOBOMA OF THE IRIS, POLYCORIA, 
AND 
PRIMARY GLAUCOMA. 
By G. E. de SCHWEINITZ, M.D., 
OF PHILADELPHIA. 
The occasional association of primary glaucoma with com- 
plete or partial congenital absence of the iris, has been noted in 
several instances. The most recent paper on the subject is one 
by E. Treacher Collins {Ophthalmic Review, April, 1891), in 
which he reports a case of primary glaucoma with apparently 
complete aniridia, and refers to the case of congenital coloboma 
of the iris and primary glaucoma recorded by Mr. Lang (Trans. 
Ophth. Soc., U. K., Vol. X, page 106), the instance of double 
microphthalmos and glaucoma, with partial absence of the iris, 
reported by Brailey {Ibid., page 139), and an example of glau- 
coma secondary to dislocation of the lens in a patient with 
nearly complete aniridia, described by Armaignac (Memoires 
et Observations d' Ophtalmologie pratique, page 239). To 2 
Schweinitz : Coloboma of the Iris, 
this list I will add a case of coloboma of the iris, polycoria, 
and primary glaucoma. The history is as follows : 
Mrs. M. M., aged 52, an American by birth, presented her- 
self for treatment January 26, 1891, with the following state- 
ment: For several years she had noted with her left eye iri- 
descent circles surrounding lights, most marked, according to 
her statement, when looking at the moon, but had experienced 
no pain. In the spring of 1890, the vision in the left eye be- 
came very poor, with intermitting pain in the peri-orbital and 
temporal regions. Two weeks before her visit for treatment, 
she suffered violent pain in the left eye and almost complete 
suppression of sight, the attack lasting about forty-eight hours. 
Since then she has had a number of similar attacks of less 
severity, and characterized chiefly by temporary obscurations 
in vision. The patient is a well-preserved woman, in good 
general health, and with a clean family history. Her previous 
illnesses have been rheumatism and malaria, neither of violent 
type. Her children are healthy, and have normal eyes and 
natural vision. There is no instance of blindness or congenital 
ocular defect in her ancestors. The following is the record of 
the examination of the eyes : 
O. D. with - i.25c axis 15 ; + 2.50s. D = 0.50 p. 
p. 22 cm. 
O. S. unimproved by glasses ; with + 2.50 spells D 
1 with difficulty. 
O. D. Oval disc, gray red ; small central excavation ; sev- 
eral spots of disseminated choroiditis. 
O. S. Oval disc, excavated to the scleral border, the center 
of the excavation measuring -2D; strong arterial pulse; be- 
low the disc a large irregular patch of retino-choroiditis, at first 
yellowish-white in appearance and farther towards the periph- 
ery presenting pigment dots. A smaller similar patch in the 
macular region. Coarse injection of the episcleral vessels ; 
steaminess of cornea ; shallow anterior chamber ; T -I- 3. The 
iris of this eye presented a congenital coloboma down and in 
its axis at 75 degrees. Up and out from the coloboma were Polycoria, and Primary Glaucoma. 
3 
three supernumerary pupils, the upper being crossed by two 
fibres. In addition to these defects of the iris there were con- 
siderable patches of atrophy, the largest being adjacent to the 
uppermost supernumerary pupil, lying to its inner side. (See 
colored drawing.) In the center of the pupil lying on the cap- 
sule there was a small, irregular, brownish, faintly granular 
tag. Figure 1 represents the field of vision. The outer bound- 
ary marks the limits of the normal field ; the transverse shading 
where vision was lost. It will be noticed that the greater por- 
tion of the nasal field is wanting, together with a small strip 
on the temporal side. Iridectomy was declined ; eserine was 
Fig. i. 
ordered. The next day the vision of the affected eye had risen 
to . In the fields of vision for white, blue, and red, in addition 
to the loss of the greater portion of the nasal field, there 
was slight increase in the shrinking of the field below. Both 
the blue and red fields were contracted, the greater proportional 
contraction being present in the red field. Two days later, in 
spite of eserine, the vision was and the day succeeding this, 
2OXL, fingers being poorly counted at one foot ; T + 2. The pa- 
tient was not seen after this until the 13th of February, having 
used the eserine continuously, and also having taken iodide of 
potash. She then reported one attack of general head pain, 
with some redness of the eye. The vision was barely the 4 
Schweinitz : Coloboma of the Iris, 
cornea very steamy, and the tension 4- 2. The lens was slightly 
opaque, and no view could be obtained of the eyeground. Iri- 
dectomy was urged, and the following day when the tension 
was unchanged, but the steaminess of the cornea and the pain 
had materially increased, and the central vision had sunken to 
counting fingers; this was permitted. This was performed 
by inserting the keratome at the position of the congenital 
coloboma, seizing the rim of iris and a portion of the ad- 
jacent tissue and excising it. A sudden movement of the 
patient caused the point of the keratome to come in contact 
with the capsule of the lens. Reduction of tension, and free- 
dom from pain were the immediate results of the operation, 
but the accident just noted caused an opacity of the lens. No 
glaucomatous attack has occurred since the operation, and the 
patient has been free from pain. An extraction of the opaque 
lens may restore vision to the eye. 
The first point of interest in this case and in all cases anal- 
ogous to it, evidently is the relation of the congenital defect of 
the iris to the development of primary glaucoma. This vice of 
conformation apparently ought to interfere with the substanti- 
ation of the idea that closure of the filtration angle and increased 
tension bear to each other the relation of cause and effect 
Fortunately, Mr. Collins has found an opportunity of making 
pathological and microscopical examinations in two cases which 
throw light upon this subject. The first case was one of con- 
genital aniridia in which the cornea ulcerated, the eye became 
glaucomatous and a staphyloma formed in the ciliary region. 
After the removal of this eye it was found that the ciliary body, 
with its processes, was present, and ended anteriorly in a small 
projection which proved to be the representative of the iris. 
The microscope revealed that the filtration area of the cornea 
was occluded by a close adhesion to it of the stump in which 
the ciliary body terminated. Mr. Collins's second case was one 
of traumatic aniridia, the eye having become blind and glauco- 
matous, the nerve being deeply cupped. On section the follow- 
ing lesions were found : round celled infiltration of the anterior 
part of the cornea; avulsion of the iris at its extreme root; 
atrophy of the ciliary body; and intimate adhesion of the 
most anterior ciliary processes to the posterior surface of the Polycoria, and Primary Glaucoma. 
5 
cornea at its periphery, in the region of the ligamentum pecti- 
natum. Illustrating the same point from the clinical standpoint 
the case of A. W. Natanson (Abstract in Centralblatt f. prak. 
Augenheilkun.dey Supplementheft zum Yahrgang, 1890, page 
489) may be quoted. This observer studied an example of 
aniridia and traumatic aphakia, with elevation of the intra-ocu- 
lar tension (T4-1). As a result of a blow with a piece of wood, 
the left eye of the patient was deprived of the iris and lens, and 
none the less secondary glaucoma with increased tension, devel- 
oped twenty-seven days after the accident. From the cases ex- 
amined by Mr. Collins, it fairly may be concluded, as he points 
out, that examples of partial and even complete aniridia form 
no exceptions to the rule that development of glaucoma is asso- 
ciated with blocking of the filtration area of the cornea. 
The second point of interest in this case is the character of 
the congenital defect itself. The coloboma does not extend 
entirely across the iris, but stops a short distance from the cil- 
iary margin and in this sense is incomplete. This fact, added 
to the others quoted from Collins's researches, serves to explain 
the existence of the glaucomatous tension. As is usual with 
unilateral defects of this character, the left eye is affected, and 
the fissure is directed downward and inward, inclination from 
the perpendicular being about 150. In addition, three distinct 
apertures or supernumerary pupils are present, occupying the 
upper and outer part of the iris, the uppermost of the three in a 
line with the coloboma, being crossed by two thin bands, proba- 
bly remains of the pupillary membrane. The remainder of the 
iris is considerably atrophied, especially in thcee patches, the 
largest one of which occupies the upper and inner quadrant, and 
the smallest* oval in shape, is adjacent to the lowest aperture 
from which it is separated by a band of nearly normal iris tis- 
sue. This patch of atrophy and the aperture near which it is 
situated, have almost exactly the same shape and size. Had 
the iris tissue in the three atrophic areas completely absorbed, 
six apertures, in addition to the coloboma, would have existed. 
As is well known, the number of so-called supernumerary pupils 
may be considerable. Mittendorf (Trans. Amer. Oph. Soc., 
1884) has reported one case in which five distinct openings were 6 
Schweinitz : Coloboma of the Iris, 
present. It seems probable, at least in human eyes, that no 
true instance of doubling of a normal pupil has been observed, 
and that the term of polycoria describes the existence of sev- 
eral openings in one iris.* In the majority of cases, in addition 
to the central pupil, one or more apertures are placed eccen- 
trically which do not represent malformations due to arrested 
development, but either are defects in the iris tissue arising 
from an incomplete evolution of the choroid, or are dialyses, 
i. e., separations of the attachment of the iris. The patches of 
atrophy in the present case appear to be imperfect apertures, 
the defects not having gone on to complete absorption of the 
structure. The small granular tag lying in the pupillary space 
(not shown in the colored drawing), undoubtedly repre- 
sented the remains of the capsulo-pupillary membrane. The 
association of capsulo-pupillary membrane and coloboma of the 
iris, and the etiological questions which this raises are interest- 
ing points which have received considerable attention in the 
papers of O. Plauge (Archiv. f. Augenheilk, Bd. xxi, page 
194) and Seggel {Klin. Monatsbl. f. Augenheilk, August, 1890, 
page 299), the former observer concluding that in laterally 
placed iris colobomas, in contradistinction from those which 
are situated below, anomalies of the pupillary membrane of the 
embryonal eye should be regarded as the most important etio- 
logical factor. The patches of retino-choroiditis present in both 
eyes deserve notice. In the left, or glaucomatous eye, the 
large area below the disc at the first glance suggested the possi- 
bility of coloboma of the choroid, an anomaly reasonably to be 
expected owing Jo its frequent association with a similar defect 
in the iris. The lesion, however, was an ordinary area of exu- 
dative retino-choroiditis, and not a developmental deficiency. 
Jn this connection it is interesting to recall the theory that 
many cases of arrested development are due to intra-uterine 
sclero-choroido-retinitis (Deutschmann), and the fact that cases 
of polycoria have been placed upon record (Seeley), the subjects 
of which have become blind from choroiditis. Finally, the cho- 
roiditis may have been the primary cause of the glaucoma. In 
one of Mr. Collins's cases, this was attributed to a hemorrhage 
into the lymph space between the choroid and sclerotic. 
*Franke E. Ueber Angeborene Polykorie Klin, Monaisbl. f Augenheilk, August, 1889, s. 386.