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Intraocular Growths

Intraocular Growths
By WARD A. HOLDEN, A. M., M. D.,

Tumors of the Iris. Strictly speaking, tumors of the iris include cyst, sarcoma, simple granuloma, pigmented granuloma or melanoma, and angioma, although besides these primary tumors there are the nodules of tuberculosis and lepra, the condylomata and gummata of syphilis, and the lymphomata of general leukemia, which will not be treated of in this article.

1. Cysts of the Iris. (1) Cyst of the stroma of the iris usually follows a perforating wound of the cornea, and appears, some months or years after the trauma, as a smooth, round tumor, translucent and non inflammatory, projecting from the surface of the iris and distorting the pupil.

In color the cyst ranges from bluish gray to yellow according to its size, the thickness of its walls, and the consistency of its contents.

The cyst as it grows preserves its globular form until it impinges on the cornea, when it flattens and moulds itself to the shape of the anterior chamber. At the outset it is not accompanied by signs of inflammation, but as it increases in size, particularly if the increase is rapid, there appear evidences of irritation, soon followed by true irido cyclitis. The latter, which is associated often with glaucoma or even with sympathetic disturbance, destroys the sight and at length necessitates enucleation.

Since the growth, if neglected, is fatal to the eye, an early attempt at removal should be made, but, owing to the impracticability of extirpating the cyst entire, recurrence is usual, although cures are reported.

These cysts may be either serous or epithelial. The former are true cysts, having a wall lined with one or more layers of epithelium (or rarely endothelium), and enclosing liquid content. When the wall is thin and the liquid clear, such a cyst may be perfectly transparent (Fig.312).

The epithelial cysts, on the contrary, are composed in the periphery of stratified epithelium, which toward the center of the tumor gradually passes over into an atheromatous mass of broken down epithelium, fat, and cholesterin. From their appearance when cut these epithelial cysts have been called pearl tumors, and, from their pathogenesis, epithelial implantation tumors.

The theory now accepted as adequate to explain the genesis of most of these tumors, and certainly of all those lined with epithelium, is that epithelial particles from the cornea, lashes, or lids are carried by the penetrating body into the eye, and, proliferating there, form a cyst. Cysts may readily be produced experimentally in this way.

For the rare cases in which there is no history of perforation of the cornea Schmidt Rimpler has advanced the plausible theory that the mouth of one of the crypts in the anterior surface of the iris becomes occluded, thus forming a sac lined with the endothelium that normally covers the surface of the iris. This sac, undergoing a progressive distention with liquid, becomes a serous cyst.
(2) Cysts of the pigment layer of the iris occur in eyes with broad posterior synechie, and are usually not discovered until the eye is cut open, although this condition has been diagnosed twice in life, the cyst presenting in the pupil a; a pigmented, vibrating, translucent tumor

These cysts are due to the drawing apart of the two strata of cells making up the posterior pigment layer of the iris, and the filling with liquid of the cavity so produced. Although usually small, they may involve the pigment layer in its entire extent (Fig. 313).

Sarcoma of the Iris. This is usually an extension of sarcoma from the ciliary body, which, passing through the bead of the ciliary body, presents in the angle of the anterior chamber (Fig. 314, D).

Sarcoma may, however, be primary in the iris, and it then appears in middle life as a very vascular tumor, soon leading to iritis and glaucoma. It is more common in women than in men.

If pigmented, as it usually is, it can only be confounded with melanoma, which is non vascular and non progressive! If not pigmented, sarcoma may be mistaken for the irregular non vascular nodules of tuberculosis, which develop with a chronic iritis in young persons (see page 339).

Treatment. In the early stages, when the growth is circumscribed, favorable results follow excision of the diseased portion of the iris by means of a broad iridectomy. It should be remembered, however, that there may have been extension into the ciliarv body, even at a time when the growth still seems localized in the iris. If this point should be positively ascertained, or if extension should have taken place, thorough enucleation is the only remedy.

Tumors of the Ciliary Body. These are sarcoma, myo sarcoma, primary and metastatic carcinoma, adenoma, nevus, and cyst. Sarcoma is the most common, and only a few cases of each of the others have been reported. Myoma and Myo sarcoma of the Ciliary Body. These are names given several times to tumors composed of long fusiform cells which were taken to be smooth muscle cells springing front the ciliary muscle. The differentiation between smooth muscle cells and the long fusiform cells of sarcoma is difficult, and it is not improbable that in some of. the reported cases the tumor was an ordinary sarcoma.

Primary carcinoma and adenoma, of the ciliary body may arise from the proliferation of the cells of the pars ciliaris retina, which is of epithelial origin. The new structure is likely to be of a glandular type. Theoretically, similar growths could arise from the posterior pigment layer of the iris, and such a case has been reported by Hirschberg, but lie admits that the character of the growth was questionable,

Cysts may be formed in the ciliary body or choroid, or there may be detachment of the choroid with rotation inward of the ciliary body such conditions are readily mistaken for sarcoma of the ciliary body. Oblique illumination of the sclera in the ciliary region will show translucency in the former case, but opaqueness if a tumor is present. The tension is also of importance.

Tumors of the Choroid. These are sarcoma, which is the most common; metastatic carcinoma, which is seen occasionally; and cyst and nevus, which are rare.

1. Sarcoma of the Choroid and Sarcoma of the Ciliary Body, These growths may be described together. The course of the disease has been divided by Knapp into four stages.

Symptoms. In the first stage, that of latency, the patient, who is usually past middle life, complains simply of a defect in the visual field. The media are clear, and there is seen a smooth, rounded elevation of the retina, without folds, not undulating with movement of the eye, not extending in most cases to the ora serrata, and with an overhanging margin in all or most of its extent. If the sarcoma is unpigmented, its vessels may be recognized beneath the retinal vessels. Sarcoma of the choroid usually appears of a reddish color and sarcoma of the ciliary body black. The tension is normal and the eye is otherwise healthy.

While this condition lasts and it may persist for years the disease usually can be distinguished easily from spontaneous detachment of the retina and from detachment of the choroid, the two conditions that resemble it.

Spontaneous detachment of the retina is preceded by the perception of musce volitantes, and comes on suddenly. It extends to the ora serrata, and the folds into which the retina is thrown undulate with every movement of the eye. The vitreous is cloudy, signs of choroiditis are usually found in the affected eye or the other, and the tension is reduced (see page 428).

Detachment of the choroid is a very rare condition, of sudden onset, and caused, as a rule, by hemorrhage, and more rarely by exudation. Tension may be increased. The characteristic vessels of the choroid, however, can usually be recognized beneath the vessels of the retina, thus establishing the diagnosis (see page 357).

Toward the end of the first stage of the course of sarcoma the vitreous grows cloudy and a general detachment of the retina ensues, producing complete blindness. Detachment, however, is longer delayed when the tumor is in the ciliary body or near the posterior pole of the eye. The tension may still be normal for a time, and the diagnosis will then be exceedingly difficult. This is true particularly of those rare cases in which the tumor is flat, for such a growth will sometimes perforate the globe posteriorly before it presents much of a tumor in the interior of the eye. The opaque tumor can, however, sometimes be made out beneath the floating retina by using intense illumination, and its plastic features may be recognized by means of Bellarminof's device of pressing a moistened Plane glass upon the cornea, thus eliminating the refraction of the cornea and permitting objects in the interior of the eye to be seen more nearly in their natural size and relief. The final test of tumor is puncture. If a sarcoma is present, blood will be withdrawn, but if the condition is merely one of simple detachment of the retina, only a serous liquid will appear.

Soon after the general detachment of the retina has occurred the second stage of the disease is ushered in, that of glaucoma. The anterior ciliarv veins are now dilated, more particularly on the side corresponding to the tumor, the anterior chamber is shallow, the media are cloudy, the tension is increased, and the eye is painful. There is occasionally hemorrhage into the eye, and at times the glaucomatous symptoms may mask every sign of tumor. Then the fact that the patient was blind before the glaucoma will arouse suspicion of tumor, and the coexistence of increased tension and detachment of the retina is almost pathognomonic. Cyclitis may supervene in this stage, or sarcoma may develop in an eye already shrunken from cyclitis; but these cases will be distinguished from those of uncomplicated cyclitis by the increased tension.

In the third stage, that of local extension, the growth spreads to parts outside of the eyeball. When the tumor is located in the anterior portion of the ball, it extends into the ciliary body, presenting in the angle of the anterior chamber, and thence passes out along the anterior ciliary vessels to form nodules in the episcleral tissue. When it is located posteriorly, the growth passes out along the vene vorticose or the posterior ciliary vessels and nerves, or the optic nerve, extending in the substance of the latter or between its sheaths, and then forms nodules in the orbit which cause exophthalmos.

In the fourth stage metastatic tumors develop in other organs, notably the liver. Even when the eye has been enucleated early, metastases occur in from 20 to 40 per cent. of the cases, and death then follows, usually within three years.

Pathological Anatomy. The shape of the sarcomatous tumor varies with the relations of the inner layers of the choroid, which overlie it like a capsule. Rarely the tumor is diffuse and only slightly elevated, but, as a rule, it preserves a spheroidal form as long as the choroidal capsule is intact (Fig. 314, A). When the capsule is ruptured, however, the tumor assumes the shape of a sphere springing from a flatter base (Fig. 314, B), and later the entire mass may again become spheroidal (Fig. 314, C).

second nodule developing near the first may remain permanently separated from the other by its capsule (Fig. 314, B). When the tumor is in the ciliary body, the anterior portion breaks through the capsule early and impinges on the lens, dislocating and distorting it (Fig. 314, D). The retina, which in the normal state is but loosely attached to the choroid, may readily undergo a total funnel shaped detachment while the capsule is still intact (Fig. 314, A). But when the growth perforates the capsule the retina becomes adherent at the point of perforation, and remains attached there, although it may otherwise be detached entirely (Fig. 314, B and C).

The consistency of sarcoma is generally firm, although the tumor may be gelatinous, and it may undergo fatty, myxomatous, cartilaginous, or osseous degeneration.

The pigmented variety (melano sarcoma) is much more frequent than the unpigmented (leuko sarcoma). The pigment of melano sarcoma may lie only in a few cells along the vessels, or may color single tracts of cells, or every cell in the. tumor may be black with pigment. The pigmentation is usually denser in the periphery of the tumor than in the center.

Sarcoma of the uveal tract may occur in many of the protean forms in which sarcoma is found elsewhere, and more than one type of structure May be represented in the same tumor. The cells are usually small, and the spindle cell is commoner than the round. There are all degrees of vascularity, from the type in which the tumor is made up of thin walled vessels, each surrounded by a sheath of epithelioid cells arranged in concentric layers (~late 8, Fig. 1.) to the type in which tracts of spindle cells run in various directions, and often in a considerable field the only trace of a vessel to be seen is a spot of pigment in the center of a tract cui transversely, representing the remains of a previously existing vessel about which the tract developed (Plate 8, Fig, II.). Alveolar forms of sarcoma are also found occasionally, and these in former days were sometimes described as carcinoma.

Prognosis. If an eye with sarcoma of the uveal tract is enucleated before there are visible evidences of extension, the chances of local return are slight; the prognosis as regards metastasis, however, is grave. If we take the average of the statistical tables that have been published, it appears that there is eventually a fatal result in about 30 per cent., of cases.

Treatment. The treatment is prompt enucleation as soon as the diagnosis of sarcoma is made. The optic nerve is to be resected far back, and evidences of extension are to be looked for, since the presence of nodules outside of the eyeball usually calls for evisceration of the orbit.

11. Metastatic Carcinoma of the Choroid. This growth has been seen a score of times at the posterior pole of the eye as a broad, flat patch of dull yellow mottled with white and some spots of pigment, with fine vessels running through it, elevated some millimeters in its central portion, and at its periphery passing over into the healthy choroid without a sharp line of demarcation. Not infrequently more than one patch is present, and the patches then tend to coalesce and surround the optic disk.

At first glance carcinoma might be mistaken for an exudation in the choroid, but the details of the growth are too clearly defined for this, and there are wanting the congestion and edema of the disk and retina that would accompany an inflammatory exudation. Carcinoma has a slow progressive course, first elevating the retina and producing hyperopia, then interfering with its function and causing a scotoma. Later, the retina is detached. In nearly every case the primary carcinoma has been located in the breast, and in a number of cases both eyes have been affected.'

The epithelial cells from the primary growth are carried into the eye through the posterior ciliary arteries, and, lodging in the chorio capillaris, they proliferate and invade all the layers of the choroid (Plate 8, Fig. 111.).

As with metastatic tumors elsewhere, nothing can be gained by operative interference, although in the glaucomatous stage enucleation has been done for the relief of pain.

Flat tumors of the choroid have proved in a few instances to have the character of angioma or cavernoma, and the designation nevus seems fitting.

Tumors of the Retina. These are cyst and glioma.

1. Cysts are found occasionally in the detached retinas of degenerated eyes, but since the media in such eyes are cloudy, the cysts are rarely discovired until after enucleation. It may happen, however, if the cysts lie far forward and the lens is not entirely opaque, that they may be indistinctly seen in life, as they were in the eye represented in Fig. 315 ; and the cysts might then be mistaken for tumors of the ciliary body did not the reduced tension and the clinical history oppose that diagnosis.

These cysts are due to disturbances of circulation whereby a liquid transuded from tached retina in an eve with plastic the retinal vessels collects in little cysts,until cyclitis from a non perforating injury. at length the retina, for some distance, is split into two layers.

11. Glioma of the Retina. This is the most malignant tumor of the eye, and is sometimes present at birth, but usually appears within the first two years of life and never later than the eleventh year. In one fifth of the cases the disease affects both eyes.

Symptoms. The clinical course of glioma may be divided into four stages, like that of sarcoma:
In the first stage the attention of the parents is attracted by a dilated pupil and a whitish reflex from the interior of the eye. If the glioma has grown from the posterior surface of the retina backward (glioma exophytum), the commoner form, it will push the retina forward, so that the latter will be seen, with its characteristic vessels, forming the nodular and uneven surface of the tumor. Portions of the retina not involved in the growth may be detached and undulating. The color of the tumor is bright pale yellow or pink, with scattered spots of white. If the glioma has grown from the anterior surface of the retina forward (glioma endophylum), a much rarer form, there will be seen a number of light colored nodules projecting forward into the vitreous in front of the retina, which is thickened and uneven.

When the growth has reached a considerable size the glaucomatous stage comes on, with injection of the eyeball, shallow anterior chamber, cloudiness of the media, and increased tension. In this stage cyclitis may supervene, causing a temporary shrinking of the ball and masking the presence of the growths condition known as crypto glioma.

In the third stage there is extension, usually first along the optic nerve, and then through the cornea, which is destroyed. The orbit thus becomes filled with a fungoid mass, and at the same time the tumor attacks the glands of the bead, and scattered nodules form on the bones of the skull.

Finally, in the fourth stage, metastases develop in other organs.

Diagnosis. This is often exceedingly difficult, but, owing to the malignaney of the growth, enucleation is usually done when there is a reasonable assurance that the disease is glioma; consequently, many of the eyes enucleated with this diagnosis are found on examination not to contain a glioma, but to represent one of the several conditions called pseudo glioma.

Pseudo glioma may consist in a malformation of the anterior portion of the vitreous, with pe~sistence of the embryonic hyaloid artery and vascular sheath of the lens a condition whose nature can usually be recognized. It may be solitary tubercle, and then, as in glioma, enucleation is indicated if the tuberculosis is limited to the eye and sight has been destroyed. But in the great majority of cases pseudo glioma is an exudation into the vitreous chamber following meningitis.

An infant has fever with meningeal symptom,,, and shortly afterward a whitish reflex is noticed from the pupil. The iris is normal or only atrophied in spots, but its ciliary margin is retracted by cyclitic membranes, so that the periphery of the anterior chamber is very deep, while the pupillary margin of the iris is pushed forward by the lens, rendering the center of the anterior chamber shallow. The pupil is usually small, and the iris, as a whole, has the peculiar appearance of a truncated cone, which is characteristic of membranous cyclitis.

In the vitreous chamber a smooth exudation will be found, dull yellow or gray in color, and without visible blood vessels. The tension is generally reduced. Later small vessels may appear in the exudation, the retina may become detached, and the eyeball may even shrink. This condition is brought about by a metastatic uveitis or retinitis due, as a rule, to meningitis, but also coming on in pyemia and various other infectious diseases. Syphilis and penetrating wounds may also give rise to a similar exudation.

Glioma, however, is distinguished from these conditions by the normal or increased tension, the dilated pupil, the normal or uniformly shallow anterior chamber, and by the nodular surface of the growth with its characteristic retinal vessels (see also pages 3,56 and 400).

Pathological Anatomy. Glioma of the retina is a soft vascular tumor, composed of small cells with a large nucleus, imbedded in a delicate mesh work of cell proeesses and fibers. It readily undergoes fatty and even calcareous degeneration. In the hardened specimen thick sheaths of healthy cells are seen surrounding the thick walled and often degenerated blood vessels, while the cells farther from the nutritive supply are degenerated and do not take the nuclear stains.

Virchow first thought glioma to be a proliferation of neuroglia tissue; others have put it in the category of sarcoma ; and there has been much discussion as to what layer of the retina glioma really springs from, and what is its true nature, some contending that a simple proliferation of neuroglia tissue could not have the extreme malignaney of glioma, which spares no tissue in the body.

Sections of glioma stained by the Golgi Cajal silver impregnation method have recently shown FIG, .He,. Glioma exophytum us that glioma is composed of neuroglia tissue and a few nerve cells of various sizes (Fig. 317). Glioma in rare instances contains tubules composed of a thin elastic membrane surrounded by long cylindrical cells, each sending a process through the membrane into the lumen of the tubule. The elements of these tubules are histological analogous to the cone nucleus, membrana limitans external and cone body of the normal retina ' and such tumors have been called neueo epitheliama.

The prognosis of glioma is very bad, only about 10 per cent., of the patients being permanently cured by operation, the others dying mostly within a year, from local recurrence.

Treatment. Euucleation should be done early and the optic nerve resected far back. If the disease has extended into the orbital tissues behind the eyeball, only complete evisceration of the orbit with removal of the periosteum can be of any avail.

Tumors of the Intraocular 1~ud of the Optic Nerve. These include hyaline bodies and sarcoma.

1. Hyaline bodies are found in the optic disks of young persons with eyes otherwise healthy and having normal vision, and also in eyes with optic neuritis or with pigmentary or albuminuric retinitis In most cases a few discrete, lustrous, pearly globules are seen in the disk, but these globules may be present in such number as to cover the disk, and even spread beyond it in a congruent mass like frog spawn (see Fig. 265).

Microscopically, we find laminated hyaline masses lying among the fiber bundles. The exact pathogenesis of these bodies is unknown, the old view, that they are products of the like the so called colloid excrescences on the lamina vitrea, now being given tip, since the bodies are often present in the disk when the pigment epithelium is healthy (Fig. 318), and they are never surrounded by pigment like the others (see also page 453).

II. Sarcoma of the optic disk has been seen a few times as a hemispherical tumor involving the adjacent retina and projecting forward into the vitreous. It is always an extension from sarcoma farther back in the optic nerve, and it can be differentiated from a tumor of the choroid overlapping or involving the disk by the fact that the retro bulbar tumor from which it extended must have caused an exophthalmos before the tumor appeared in the eye.

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