102 E V E R E T T L. GOAR

R E F E R E N C E S

1. Reese, A. B . : Herniation of the anterior hyaloid membrane following uncomplicated cataract extrac­tion. Am. J. Ophth., 32:933 (July) 1949.

2. Leahey, B. D . : Bullous keratitis from vitreous contact. Arch. Ophth., 46:23 (July) 1951. 3. Irvine, S. R.: A newly defined vitreous syndrome following cataract surgery: Interpreted according

to recent concepts of the vitreous. Am. J. Ophth., 36:599-619 (May) 1953. 4. Villaseca, A. : Late emptying of anterior chamber and choroidal detachment in cataract operation.

Arch. Ophth., 52:250 (Feb.) 1954. 5. Weisel, J., and Swan, K. C.: Mydriatic treatment of shallow chamber after cataract extraction.

Arch. Ophth., 58:126 (July) 1957.

A CONGENITAL ANOMALY SIMULATING TUMOR

PARKER HEATH, M.D. Sullivan Harbor, Maine

The developmental complexes formed in the anterior segment separately and jointly by the iris, ciliary body, lens, and retina are of great interest and frequently of practical importance. The causes lie in two fields of pathology—the less common developmental anomalies and postnatal acquired processes, especially from physical injury. Clinical manifestations usually take courses peculiar to each origin, but exceptions are encoun­tered. Considerable difficulty concerning diagnosis and management may arise in the individual case.

The following report tells the story of an iris-ciliary-body-retina complex within the developmental anomaly field. Only hindsight provided the correct diagnosis and showed therapeutic possibilities.

CASE REPORT HISTORY

The history was supplied by the referring physi­cian. A five-year-old girl (full-term baby) pre­sented a convergent left eye which had always been thought sightless. About six weeks before the initial examination the patient noticed she could see with the left eye—an event thought to be a miracle. No history of injury, inflammation, pain, or familial eye disorder was established.

EXAMINATION

Examination of the left eye ( E 53-25) showed in a five-year-old otherwise healthy girl a con­vergence of about 30 degrees, Priestly-Smith method. A brownish to black mass mesially behind and possibly in the iris applied to the lens. The lens was cataractous and displaced toward the temporal, 3-o'clock position. Fundus details were not supplied.

Intraocular pressure was not elevated. Transillumi-nation was inconclusive. Following several exami­nations the left eye was suspected of harboring a tumor in the anterior segment. The right eye func­tioned normally and appeared to be normal in every way.

LABORATORY EXAMINATIONS

Gross examinations showed: volume, six cm.; weight of globe, 4.6 gm.; measurements: horizontal, 20.5 mm., vertical, 21.5 mm., anterior-posterior, 21.5 mm. The globe was well proportioned and appeared to be that of a child except for a bulge of seven-mm. in diameter in the region of the attachment of the inferior oblique. The pupil measured four mm. and was somewhat off round and eccentric as the temporal half of the iris was somewhat displaced forward. Anterior chamber deep. Within the tem­poral half of the pupil the lens appeared tilted and the mesial edge rotated posteriorly as well as pushed temporally. An optic nerve present, meas­ured two mm. in length.

The eye was fixed in formol and later opened horizontally. The cornea was normally thick and the anterior chamber appeared deep. The anterior surface of the iris appeared uniformly brown and the temporal side bulged because of a yellowish opaque lens about five-mm. thick in an-teroposterior diameter, and displaced as noted. The ciliary body-lens angle was deeply pigmented. The lens appeared to be adherent to the temporal iris and ciliary processes. The vitreous was mostly fluid and supported a few strands extending from the posterior retina to the region of the lens. The retina was in position throughout. Vessels on the disc appeared dilated; the macula area was not re­markable.

The macroscopic appearance of the specimen on the slide showed the lens displacement and ex­traneous tissue behind the iris on the mesial side.

Microscopic findings may be somewhat briefly summarized: The cornea showed a pannus and a few deep stromal vessels. The angle was open and contained a few cells and considerable pigment in the trabecula. Schlemm's canal was open and con-

CONGENITAL ANOMALY SIMULATING TUMOR 103

Fig. 1 (Heath). Whole eye ( x 2 ) . (L) Lens displaced. (I) Iris-ciliary-body-retina complex. (S) Scleral staphyloma.

Fig. 2 (Heath). Mesial angle (Xl8) . (R) Reti­nal tissue with cysts. (I) Iris pigment layer with cysts. (CB) Cystic ciliary body. (Z) Interrupted zonule. (V) Vitreous boundary.

tained some pigment, apparently of the melanin variety. The root of the iris extended from the ciliary body, contained considerable pigment and a few clump cells, and a few intra-epithelial cysts.

A few balls of pigment and flocculi and cells lay behind the iris near or in the pupillary space. A lens-iris synechia was well developed. The mesial root of the iris was attached to a long peninsula of anomalous cystic retinal and ciliary body tissues extending into the pupillary zone, during life, no doubt, pushing and tilting the lens toward the temporal side.

The intra-epithelial cysts contained fine granular debris, red discoids, and rare blood cells. The cysts formed by the cilioretinal epithelium contained numerous blood vessels, some large capillaries, vitreal strands, few red blood cells, and amorphous debris. A fenestrated membrane on the inner aspect of the ciliary body was continuous with that from the pigment epithelium of the retina and coupled with Bruch's membrane. The choriocapillaris as it approached the ciliary body appeared on both sides of Bruch's membrane. Larger vessels were closely applied to the outer side. The retinal pigment layer

Fig. 3 (Heath). Detail of iris (I) and ciliary body (CB) cyst formations (x70).

104 PARKER HEATH

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Fig. 4 (Heath). Detail of retinal component (x300). (FM) Fenestrated membrane.

made the transition to unpigmented ciliary epithe­lium on the tongue or peninsula complex.

The ciliary body appeared to be rather small and partly differentiated and loose and contained pig­ment scattered in the anterior aspects. Large ves­sels here showed margination. The pars plana was nearly nonexistent on the mesial side, and appar­ently had contributed heavily to the aberrant tissue effecting a displaced lens. The mesial side of the choroid showed more vessel content than the tem­poral. The lens thinned out somewhat mesially and the posterior surface in some sections showed some dishing inward.

Posterior synechia were rather broadly attached to the lens capsule. The whole lens was displaced to the temporal side; fluid clefts were present; bow cell nuclei sharply reduced; epithelial nuclei ex­tended abnormally posteriorly. The lens stained in a diffuse cataractous manner. The vitreous boundary occupied its usual position behind the lens.

A hyaloid remnant could be traced from the mesial aspect of the lens to the mesial side of the nerve head. The posterior retina apparently con­tained the usual number of ganglion cells in the macula, but these rapidly tapered off, especially mesially. Some ganglion cells appeared over the ciliary body. The nerve head was reduced in di­ameter and glial columns were irregular. The sclera was thin on the entire temporal side, and contained an area of staphylomatous bulging near the insertion of the inferior oblique muscle.

DISCUSSION

The iris contribution to the cystic aber­rant tissue was mostly pigment epithelium. Cyst formations from iris, ciliary, or retinal types of tissue increase in size as the tongue reaches toward the axis of the globe. It is possible that atypical formations of ciliary processes which normally begin at the 60-to 70-mm. stage held and dragged the iris pigment inward. The displaced iris epi­thelium formed flocculi but not ectodermal muscle. The ciliary processes at about the 75-mm. stage are on the lens and should have withdrawn safely away by the fifth month and this gives us some idea of timing. Or if held by atypical persistent tunica vasculosa lentis the ciliary-iris structures would by growth follow closely and become attached to the lens, and years later exert pressure by developing cyst formations. The per­sistent myaloid artery which does not carry blood and extensive lens attachments if pres­ent early had now, five years later, disap-

CONGENITAL ANOMALY SIMULATING TUMOR 105

peared. The suspensory zonule of the lens and the ciliary muscle appear during the third month normally, therefore, interacting defects in the former could contribute toward attachments to the lens. When the margin of the optic cup is growing forward, at the 48-to 50-mm. stage, the retina is redundant and by position could become incorporated into atypical differentiations with the ciliary proc­esses at larger stages.

That the anomaly we see is developmental and not a metaplasia is indicated by collateral findings of persistent hyaloid artery, the nature of the lens changes, and the defects not limited to the anterior segment which include defective development of the poste­rior sclera in the region of the inferior oblique (abortive coloboma).

We can only speculate upon the exact mechanisms initiating the abnormality. The interval of five years since birth has been long enough to mask and resolve the actual interplay of obstruction and adhesive forces causing the display we have described above. Certainly the changes are developmental as opposed to inflammatory origins, and prob­ably extend over the organic, neofetal, and fetal periods.

Because of the well-developed posterior segment, especially the retina, the question of therapy and clinical procedure are im­portant. The first consideration is diagnosis. An anterior segment anomaly was suggested by the nature of the cataract, size of eye, muscular imbalance, and absence of inflam­mation, and the rate of growth. The cystic nature of the growth would be difficult to establish by inspection, but should be sus­pected from the displaced lens. The age of the patient is against malignant melanoma

Fig. S (Heath). Posterior segment (X8) shows good differentiation.

and also retinoblastoma. In consequence of hindsight, the surgical procedure would seem to be iridectomy and attempt to remove the underlying cystic structure. Not only would this offer the best avenue of attack, but it also would provide material to clinch the diagnosis.

SUMMARY

The convergent left eye of a girl, aged five years, was thought to be blind from cataract of congenital type. Spontaneous re­storation of partial vision occurred. Ex­amination showed a pigmented mass pushing the cataractous lens toward the temporal side partly uncovering the pupil space. Labora­tory study of the eye showed an iris-ciliary body-retinal complex evolving from a devel­opmental anomaly. Expansion of the aber­rant tissue which lay behind the mesial iris was by growth and multiple cystic forma­tions.