FROM T H E OPHTHALMIC DEPARTMENT OF T H E FINSEA' INSTITUTE A N D RADIUM CENTER, COPENHAGEN.

CHIEF: OLAF BLEGVAD, M. D .

MYXOMA OF THE ORBIT.

By Olaf Blegvad.*)

While myxomatous areas in mixed orbital tumors are frequent, true myxomas of the orbit are exceedingly rare. In most textbooks they are not mentioned at all, and even in so large a work as Lubarsch & Henke's BHandbuch der speziellen pathologischen Anatomie und Histologic<, from 1931, myxomas are not mentioned among the many tumors of various kinds that may occur in the orbit. In a search through the available literature I have only succeeded in finding 5 cases of true myxoma. "he case which I shall report must therefore be said to be a rarity; and as it, moreover, illustrates how a tumor in the course of its growth may alter the refraction of the eye and, besides, may cause certain changes in the eyeground, in the form of foldings in the retina, I feel myself justified in presenting it here, and in making it the subject of a few observations.

The patient is a married woman, aged 29. According to state- ments, her general health has always been good. After a childbirth six years ago she noticed for the first time that the vision of her left eye was defective, and she consulted an oculist. A t the er- amination, the right eye was found to be normal, with V. = W6 and hypermetropia of 1.5 D. Also L. V. was = 6/6, but with hyper- metropia of 2 D. and slight astigmatism. The ophthalmoscopy was normal. In May, 1939, the patient returned. L. V. was still = 6/6; but the hypermetropia had increased to 4 D., and ophthalmoscopy now showed in the center a large area, fully 2 0. D. diameters

*) Received April, 3th, 1944.

wide, in which the retina was a little darker than normal, and in which there was seen a peculiar, rather coarse, stellate striation, extending all the way to the optic disk. The refraction was measured to + 6 D. in the center, 4- 4 D. on the disk. On the temporal side of the eyeball, the conjunctiva was somewhat thickened, with a number of dilated vessels. According to the patient's statement, this condition had developed in connexion with the childbirth just mentioned, when there had been conjunctival hemorrhages during the labors. The surroundings of the eye were normal, it was there- fore a little difficult to account for the increasing hypermetropia and the curious ophthalmoscopic picture. In June, 1939, she became the victim of a motor accident, in which she got severe lesions in the face; among others there is said to have been a large hematoma in the left orbit. She was in a provincial hospital for two months. I saw her again in February, 1940. She then complained of double vision, and of exophthalmus of the left eye. L. V. had now become reduced to < 6/9, mith + 4.00 sph. 2 -I- 0.75 cyl. axis 90". There was moderate exophthalmus of the left eye and double vision correspond- ing to a paralysis of the left lateral and superior recti muscles. The ophthalmoscopic observations were as before. The double images did not discommode the patient; and as the paralysis was believed to be the result of the hematoma in the orbit it was decided to await developments. In February, 1942, the patient had a second child-birth, and after this the exophthalmus distinctly increased: wherefore she again presented herself for examination in May, same year. The eyeball was now seen to have become deviated downwards and nasalwards, and a tumor could be felt upwards temporally in the orbit. Besides, there could faintly be seen large, tortuous vessels in the conjunctiva, which extended over the tumor. No pulsation could be felt, or any pulse sound heard, over the latter. The tumor felt soft and elastic to pressure, and did not empty itself when compressed. As the tortuous vessels suggested the possibility of an angioma, the patient was referred to the Radium Center, for roent - gen treatment. A roentgenogram of the orbit showed no signs of pathologic changes in the latter, and no blurring of the paranasal sinuses. In June, 1942, the patient was given 4 X 200 r to the left orbit, obliquely from the lateral commissure, with lead-shielding of the eyeball. There came rather strong reaction on the skin and in the conjunctiva after the treatments, but the tumor did not recede. In August, readings with Hertel's exophthalmometer were: right eye, 16 mm., left eye, 23 mm.. The vision had diminished to 6/60, the hypermetropia increased to 6 D.. There was still deviation of the eyeball and double images; which did not, however, discom- mode the patient, owing to the poor vision on the left eye. In Sep- tember, she was again given 3 roehgen treatments, and the same in January, 1943; all without noticeable effect. The exophthalmus

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remained unchanged, with 8-9 mm. difference in the two eyes. Its disfiguring effect was a great worry to the patient, as will per- haps be understandable when one looks at Fig. 4 . When the eye gradually became painful, she had reached a point where she felt that she rather would have it removed. It was proposed, however, that it should first be attempted to remove the tumor. In August, 1943, the vision on the left eye had diminished to < 3/36, and the hypermetropia increased to 8 D.. She was therefore entered for

Fig. 1.

operation, and on August 20th, 1943, a large canthotomy was done, which gave good room for reaching the tumor through an incision through the conjunctiva. It was then found that the growth was not, as it had been supposed, an angioma, but a fully walnut-sized, soft, myxomatous tumor, situated upwards and temporally, directly on the eyeball. It was easily removed entire, without much hemor- rhage. The eyeball was seen to have become greatly deformed by its pressure; there was an actual, deep dent in it where the tumor had been situated. The wound in the conjunctiva healed smoothly, the pain disappeared at once, the exophthalmus disappeared com- pletely; and, strange to say, the lateral rectus muscle seemed to have been preserved; for the eye returned nicely to parallel position, as seen in Fig. 2. The vision did not get better, though. It is now = 2/36, with hypermetropia of 5 D.. The field of vision is normal

Ada Ophthalrnol., Vol. 22. 11. 10

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Fig. 2.

for large objects. The eye can be moved naturally in all directions: only the abduction lacks a little in being complete.

Three months after the operation, ophthalmoscopy showed the following picture (Fig. 3): The striation in the fundus was still seen; hut it was much more delicate than before, and was seen not only in the center, but also above and below the optic disk and far out

Fig. 3.

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in the temporal side of the fundus, where there were also a number of minute pigmentations. The refraction 'was about f 4 D. on the disk and $. 5 D. in the center and temporal part of the eyeground. The stria were observed best with red-free light, and with Gul- stad's binocular ophthalmoscope it was seen that where they were situated the retinal tissue was clistinctly edematous. The micro- scopic diagnosis (Dr. Fiitjof Bang) was myxoma of the orbit.

Myxomas may be divided into true and false. The former are true tumors, the latter arise as the result of edemas or mucoid degeneration of other tissues. The myxomas contain mucin, which stains red with mucicarmine. *They arise from remnants of muciform embryonic tissue, which no doubt is the parent substance for several varieties of the connective tissue-group, espeuially for adipose, cartilagineous, osseous and ordinary connective tissues, and which is also found in the nerve sheaths, where it can give rise to multiple tumors ( Recklinghausen's disease). Myxomatous areas may therefore be found in tumors of very diverse character, - leaving aside the cases where it is a question of ,pseudomyxomasc<. Thus, they may be found also in the myxomas of the endocardium, some of which are no doubt true myxomas, while others are due to degenerative changes in thrombi, as recently shown, for instance, by Eskelund, in a paper published in #Nordisk Medicincc (vol. XIV, 1942).

True myxomas often arise from embryonal remnants of inyxomatous tissue left behind in the subcutaneous tissue and intramuscularly. They are, as a rule, poor in cells, but not always well delimited; are therefore said to have some tendency to local recidivation. They often occur combined with lipomas; and since the tumor I have just described was situated in the orbit, and embryonal mucous tissue is the parent substance of adipose and other tissues, it is reasonable to suppose that it in this case was a question of tumor tissue developed from a remnant of embryonal mucous tissue there. The tissue gave distinct positive reaction for mucicarmine. That it formed a growth which displaced the eyeball and caused proptosis is an absolute indication of its neoplastic character and negatives the supposition that it should have been a case of secondary changes and edema in the fatty tissue of the orbit or in a hematoma arisen as result of the

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trauma sustained. It cannot be supposed that a hematoma would go on growing for a considerable length of time and clinically manifest itself by tumor-like symptoms; and de- generative changes in such a hematoma are more likely to show themselves in the form of regression.

Another diagnosis, which must be’considered in this case,

Fig. 4.

is that of myxochondrosarcoma arisen from the bony wall of the orbit ; but neither histologically nor roentgenologically was there anything to indicate that it should have been a case of that very malignant tumor.

The tumor (Fig. 4) showed no signs of malignancy. In part, at least, it was well delimited by streaks of connective tissue. In several places there were groups of small vessels, and about these a more or less dense, mucofibrillar tissue with rather scattered, small, oblong cell-nuclei. The cytoplasni reacted positively to mucicarmine. In the vicinity of the blood vessels there were in many places a number of lymphocytes.

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Moreover, there were in a few places, especially near the peri- phery, cells with a yellow pigment which reacted positively to iron and thus was hemosiderin, indicating old hemorrhages in the neoplastic tissue. In some places the tissue stained poorly, indicating degeneration ; which may perhaps have been due to the roentgen treatment. There were no nuclear ab- normalities, no signs of neurogenetic or osteogenetic tumor formation; though a neuiogenetic origin cannot be excluded (Dr. Frifjof Bang).

If we now briefly summarise the history of the case, we find the following:

In a young woman there is shortly after a childbirth observed a diminution of the vision on the left eye, owing to a change in the refraction, at first amounting to moderate hypermetropia, but in the course of six years gradually in- creasing to 8 diopters. A t the same time, there developed, upward in the temporal side of the orbit, a tumor which pro- duced an exophthalmos increasing to 8 mm.. The eyeball be- came dislocated downwards, nasalwards ; the motility of the eye became considerably restricted, the vision fell from W6 to 2/60, and there came pain back of the eye. A t the same time, there was seen in the eyeground a curious folding of the retina, which showed itself as a stellate pattern of folds, with the macula as center. By operation, a fully walnut-sized tumor was removed, which was situated upwards on the tem- poral side, close against the eyeball, which it by its pressure had deformed, so that there was a large depression in it, cor- responding to the site of the growth. Though the lateral rectus muscle could not be visualised during the operation, it seemed to have been preserved; for the eye returned to parallel po- sition and almost completely recovered its motility. The dif- ference in refraction did not disappear entirely after the operation; and as late as five months after it the vision was only 2/36, and the folds in the retina still remained. Mi- croscopy showed true myxoma.

As already said, very little has been written about myxomas in the orbit. Altogether, these tumors are rather uncommon. They are oftenest found in the skin, the periosteum, the fasciae, the connective tissue of the nerves,

- on the whole i n loose connective tissue and where there is much adipose tissue. But also in such a peculiar site as the cornea, t rue myxomas have been found (Bussy; in Archives d'Ophthalmologie, vol. 42, p. 33; 1925).

I shall briefly mention the only five cases I have found in the literature.

The earliest seems to be a case reported in the Italien ))Archiyo di Ophthalmologiacc (rol. XXI, p. 300) by Mauricione, of which a brief summary is given in v. Michel's ))Jahresbericht((, 1913, p. 142. There is there described a non-capsulated epibulbar tumor composed of myxomatous tissue, - a true myxoma, arisen from the subcon- junctival connective tissue near the medial commissure. It had in the course of six months grown to a diameter of about 1 centimetey.

The second case was described in 1914, by Fuchs, in the Vienna Ophthalmologic Society, and later published in the ))Zeitschrift f . Augenheilkundecc, vol. XXXII, p. 192. The patient was a woman, aged 40, who for seven years had had an increasing exophthalmos; - eventually there was a difference of 20 mm. in the two eyes. The tumor, which was removed by operation, was 1% times as large as the eyeball. It was situated in the vicinity of the lacrymal gland, but not firmly adherent to any adjacent organ. It was surrounded by a firm, fibrous capsule, and microscopy showed it to be a true, unmixed myxoma. Fuchs said that whereas myxofibromas and myxosarcomas in the orbit are known, no case of this character had. to his knowledge, been described before.

The third case is reported in 1928 by H. D. Lamb (Archives of Ophthalmology, 1928, vol. LVII, p. 425). The patient was a girl, aged 16, who for three years had had a slowly growing tumor in the right orbit. There were occasional attacks of pain, and double vision, and there came slight esophthalmos of 1 mm.. The eyeball was dislocated down and outwards, the motility was restricted in all directions, most downwards. The disk projected forwards 0 diopters, and the vessels were tortuous. The vision was to counting fingers at 3.5 m.. Operation was performed and a tumor removed, which appeared to arise from the superior orbital fissure. It was fully 4 cm. in diameter, and had to be removed piecemeal. The vision became normal, but there remained a slight diplopia. The micro- scopy showed true myxoma, with positive m u c h staining.

The fourth case is from 1931 and was reported by J . Bistis, of Athens (Archiyes d'Ophthalmologie, vol. XLVIII, p. 440). The patient was a man, aged 29, whose right upper eyelid had for three years become increasingly ptosed. The eyeball was deviated downwards, but there was only very slight exophthalmos. Its motility was restricted, and the vision only 6/60. Ophthalmoscopic inspection showed optic disk atrophy. In the orbit could be felt a soft, displace-

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able, elastic tumor. It was removed by operation, and was found to be 1 X 2 cm. large, and extending all the way back in the orbit. It was lobulated, and the section surfaces yellow. Microscopy showed true myxoma; but it does not appear that staining for mucin was done. After the operation, the vision remained unchanged, 6/60; but, of course, there was also pressure atrophy of the optic nerve.

The f i f t h and last cuse is described by S. R. Gifford , also in 1931 (Archives of Ophthalmology, rol. V, p. 445). The patient was a woman, aged 25, from whose orbit he already in 1926 had removed seven small myxomas. Four years later, there was recurrence, with exophthalmos of 5 mm., and the eye was pushed down and out. Again a large number of separate tumor masses were removed, which on microscopy proved to be true myxomas. Stain for much was positive. Giffoi d points out that the recurrence is not an indica- tion of malignancy, but must be attributed only to incomplete re- moval of the tumors at the earlier operation.

It will be seen that most of these cases very much resemble my own. The myxoma is evidently a slowly growing tumor, which, although it is soft, pushes the eyeball forwards or towards the side, limits its motility and causes diminished vision and, sometimes, pain. The microscopic picture is typical and the same in all cases.

In my case, there were, however, two peculiar features; namely the increase of the refraction and the folds in the retina. That the pressure of the tumor from behind can cause flattening of the posterior pole of the eyeball and .hyper- metropia is in itself nothing strange, of course; yet it has not often been observed; and in many cases, even where it is a question of hard tumors in the orbit, it does not occur. The reason is perhaps that the eyeball deviates, - becomes either dislocated or proptosed. The site of the tumor, right on the eyeball, to which it has perhaps been held attached by a capsule, probably explains why there in my case came so large a depression in the wall of the eyeball. J . Kubilc states (Klinische Monatsblaetter f. Augenheilkunde, 1928 ; vol. LXXX, p. 513) that he has seen a hypermetropia of 8 diopters pro- duced by the pressure of an orbital osteoma, and in another case an astigmatism of 13 diopters caused by a mucocele which from above pressed the eyeball down against the inferior mar- gin of the orbit. Also myopia is sometimes seen to result from

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lateral pressure of a tumor on the eyeball; but in most cases there is no change in the refraction.

The same is the case with the folds in the retina. Loehlein has in the ,Klinische Monatsblaetter f . Augenheilkundecc (1927; vol. LXXIX, p. 769) published some photographs of an eyeground with retinal folds occasioned by pressure from a mucocele. In that case the pressure was, consequently, ex- erted from above and behind the eyeball, and t l e folds are therefore horizontal. Loehlein thinks that the diminution of the inner space of the eyeball may cause the inner membranes of the latter to become pushed together and thus become folded. The unilateral pression alters the tension in the retina, and produces creases in it. Loehlein believes that in his case only the inner retinal layer, and not the sensory epithelium itself, was folded; because the vessels did not seem to go a11 the way up into the folds. In my case, observation of the eye- ground through a binocular ophthalmoscope gave me the absolute impression that the retina in the places where it was folded was edematous, and I therefore believe that the edema may have been at least partly responsible for the fact that the retina could not find room in the area reduced as result of the indentation, and therefore had to fold up. It evidently requires a considerable time for the eyeball to re- sume it normal shape. Several authors mention that the folds are still seen a long time after the operation. In Loehlein's case they were present as long as ten weeks after; and in Graefe-Saemisch's handbook Birch-Hirschfeld speaks of a case in which they could still be seen even seven months after the operation. In my case they are still seen after five months; and the hypermetropia has not disappeared completely, either.