CASE NEUROMA OF THE OPTIC NERVE, WITH REMARKS AND ILLUSTRATIONS. BY JOHN A. LIDELL, M.D., SDRQ. U. S. VOL., PROP. OF ANATOMY IN THE NATIONAL MEDICAL COLLEGE. Second Edition. NEW YORK: HOLMAN, BOOK AND JOB PRINTER, CORNER OF CENTRE AND WHITE STREETS. 1 186 3 A CASE NEUROMA OF THE OPTIC NERVE, WITH REMARKS AND ILLUSTRATIONS. f BY JOHN A. LIDELL, M.D., SURG. C. S. VOL., PROF. OF ANATOMY IN THE NATIONAL MEDICAL COLLEGE. Second Edition. ,«^ 7/H. uk'iifs NEW YORK: HOLMAN, BOOK AND JOB PRINTER, CORNER OF CENTRE AND WHITE STREETS. 1863. A CASE OF NEUROMA OF THE OPTIC NERVE. Neuroma (from vevqov, a nerve) signifies a benign tumor de- veloped in connection with a nerve. ^This disease is not often met with ; and it is only within a very recent period that the general character and relations of these growths have been commonly understood, and that they have been arranged together in a special group or class, denominated neuroma. By the older surgeons, all tumors of nerves were considered to partake of a malignant character, and even Chelius, in his great work on surgery, although he distinctly recognizes the non-malignancy of the nerve-tumors under consideration, still describes them under the head of, and in connection with, cancer of the nerves. The excellent monograph of Mr. Robert W. Smith, of Dublin, gives the fullest, most recent, and best account of neuroma which we have seen, and, as Mr. Erichsen well remarks, it contains "the principal part of our knowledge of this subject." Neuromata are not confined to any one part of the body, to the exclusion of all other parts. They are, however, more fre- quently met with in the extremities than in the trunk; and, again, found oftener in the lower than in the upper extremity. Their principal seat is the cerebrospinal system of nerves; though Bdrard has once met with this morbid growth on a ganglionic nerve. The case about to be described is the only one I have been able to find where this disease involved the optic nerve, and almost the only one involving a nerve of special sense. 6 ture. 2. It never contaminates the neighboring lymphatic ganglia ; carcinoma does. 3. While-it impairs the patient's general health by pain and distress, causing pallor, emaciation, and debility, it never produces the true cancerous cachexy by specific infection of the system ; carcinoma does produce such a result. 4. It does not return after excision ; carci- noma does. 5. The pain of neuroma is neuralgic (electrical and paroxysmal) in character ; the pain of carcinoma js not* The comparative rarity of the neuromatous disease, its entire benignity, the liability to mistake a bad case of it in an ad- vanced stage for malignant disease, the fact that it is not amenable to medical treatment, that it can not be cured short of extirpation, and that it is cured by operation with great cer- tainty, according to the testimony of all observers, and, finally, the fact that I have not been able to find the history or the description of any other case of neuroma, involving the optic nerve, have induced me to place the following case on record, as a contribution to the current surgical literature of the day. It is barely possible that individual cases of neuroma of the optic nerve have passed hitherto for cancer of the eye, or orbit, or both, to which, indeed, in an advanced stage, it bears some resemblance, and thus have either not been extirpated, or, if extirpation has been practiced, the disease has borne another name. Case.—In the latter part of July, 1858, a pale and rather spare young woman, of twenty years, named J. L., applied to me for advice concerning a large, red, fleshy-looking, elon- gated, and rounded mass, which filled the left orbit, and pro- truded therefrom, so as to hang over the cheek almost as far as the level of the nostril [vide plate]. She and her mother together gave the following history : Her health had been good till the spring of 1850, when she was twelve years old. Previously, she had had measles, scarlet fever, and hooping-cough, but had them so light, as not to produce any difficulty worth mentioning. In the spring of 1850, she, along with other children, took the mumps (paro- titis), which involved the gland on both sides of the neck. The difficulty in the right gland followed the usual course, which was not the case, however, with the one on the left side. The swelling in this gland did not readily subside, but it re- mained enlarged and troublesome for nearly a year. In the latter part of the same summer (1850) the left eyelids became swelled. At the same tims the right eyelids were natural. This palpebral tumefaction lasted two or three months, or till * Vide Smith's monograph. 7 the last of autumn. At first her mother supposed it was pro- duced by poisoning with ivy (rhus radicans—they lived in the country), but this conjecture was negatived by the fact that the swelling was confined to the left eyelids, and lasted for so long a time. Four or five months after the palpebral swelling had disappeared, that is, in the spring of 1851, she found the sight of the left eye was becoming dim. Objects, when seen through this eye alone, appeared to be in a mist, or haze. ^ She could, however, distinguish the scholars, as they sat in their places in school, so as to recognize them. At the same time the sight in the right*eye was unimpaired. The left par- otid gland still continued to be enlarged. Through the remainder of spring, and the following summer, the dimness of sight (amblyopia) increased so rapidly that in the fall (1858) she could only distinguish light from darkness. During all this time she did not suffer any pain, nor muscae volitantes, nor colored spots, nor flashes of light in the affected eye, so far as she remembers. About this period, i. e., some time in the fall of 1851, she noticed that this same left eye began to protrude (exophthalmos). After this, the perception of light rapidly became fainter, and soon disappeared ; the eye gradually pro- truded more, until, twenty months afterward (June, 1853), the eye protruded so much that the lid could not be closed over it. Her mother says that, at this time, the eyeball itself did not seem to be much enlarged ; also, that the pupil was widely dilated, and that every thing behind the pupil appeared to be dark-colored. She was positive she never saw any yellowish or shining metallic appearance behind the pupil. The patient says that at this time the eye was distinctly movable in front of and upon the substance, whatever its nature might be, which caused the protrusion, and had been thus movable for some time previously. In this statement she was very positive. Up to this time she had not suffered any pain, except about a year before, when a compress was applied by the late Dr. White, of Cherry Valley. Having tried various remedies without benefit, he resorted to compression, for the purpose of arresting the protrusion, but, after continuing it about two weeks, was forced to abandon it, on account of the severe pain it occasioned. After the compress was removed, the pain ceased. Up to this time her general health had not suffered. In the latter part of June, 1853, her symptoms underwent a material change. On the day subsequent to a long walk (five miles) under a hot and bright sun, she had a good deal of pain in the affected eye, which became red, and speedily swelled to a great size (conjunctivitis). The eyelids also were much swelled, and purplish in color. During the following fortnight 8 she suffered very much from the severity of the inflammation; at the end of this period the eye broke, discharging water (aqueous humor) and matter. After this the pain abated, and the inflammation gradually subsided, till it entirely disappeared by the following October. In the mean time, the patient's suf- ferings had been so severe as to wear her down very much, affecting her general health, and making her thin, and pale, and weak. Much of the ophthalmic enlargement became perma- nent ; that is, the original swelling increased very rapidly during the late period of activity. During the following win- ter she got on tolerably well; her'general health improved, but she was occasionally troubled with paroxysms of pain, neu- ralgic in character, in the left eyebrow, left half of forehead, and the left cheek. The morbid growth, had probably attained such a size as to press considerably on the supra and infra- orbital nerves. In the winter of 1856 and '57, she had fever which lasted six weeks, and left her very weak for a long time. This did not seem to effect the local disease one way or the other. The ophthalmic swelling slowly increased in size, and the neuralgic pains in the left eyebrow, forehead, and cheek, gradually increased in severity until I first saw her (July, 1858). For some time before this, she says she had suffered great distress, the paroxysms of pain being frequent and severe. She also says that any considerable amount of pressure upon the protruding mass, or the handling incident to a physical examination of it, produces, besides the supra and infra-orbital neuralgia (by which I mean pains intermittent, and like elec- trical shocks), an aching pain in the mass itself, so severe as to deprive her of sleep for the following thirty-six or forty-eight hours. A misstep in walking, whereby the diseased parts are jarred, produces a similar distress, varying only in intensity. She is not subject to any other pains in the head than those mentioned ; except, perhaps, more or less of a sense of disten- tion in the orbit. She has partial deafness in the left ear, but was not aware of it till I ascertained it during the examina- tion. She menstruated at 14, and has always been regular. There is no hereditary disease in the family. Objective Symptoms.—The patient is pale, and rather thin ; the features are sharpened, and the habitual expression of coun- tenance is that of much distress ; the eye (sound one) is grayish- blue in color, and the hair brown. The patient sits with her head dropping partially forward, apparently from the weight of the tumor. On close inspection, the protruding mass is seen to be regu- lar in shape, rounded, elongated, and somewhat conoidic at the end, and to hang down almost to the level of the nostril; the 9 upper eyelid is thrust forward and stretched down over it to the breadth of two inches; the tarsus and ciliae are sound ; the skin is natural in color ; and the eyelid, notwithstanding it is stretched so much, is every where movable, in some degree, over the morbid growth ; and near the tarsal border is a vein enlarged, blue, and tortuous. At the opposite, or conoidic end of the tumor is a pearl-colored, nearly circular spot, three fourths to seven eighths of an inch in diameter, evidently the opaque remains of the cornea, which had been destroyed in 1853. Between this pearl-colored, opaque spot, and the tarsal border of the lid, the tumor is dark red in color, and is covered with a granular membrane, which secretes a muco-purulent fluid; this is evidently the ocular and palpebral mucous membrane, pushed down from its natural situation, and stretched over the morbid mass ; this dark-red, granular, and thickened conjunct- iva is not reflected beneath the "eyelid, but terminates abruptly at the tarsal border thereof; no ulcerations are present, and this mucous membrane is, in some degree, movable (non-adher- ent) throughout its whole extent. Beneath the middle of the upper eyelid, there is a semi-fluctuating sensation imparted to the touch (deep-seated fluctuation). In every other part the tumor has a tense, fleshy, and elastic feel. The orbit is com- pletely filled by it; the orbitary margin is well defined, but the size of the orbit itself is considerably increased in the direction of both its perpendicular and horizontal diameters ; indeed, the orbit is so much enlarged, that the portions of the malar and temporal bones, which form the external boundary of the orbit, bulge out so much as to produce quite an angular appearance of that side of the head and face. The tumor also seems to be slightly movable in the orbit, but of this I am not entirely sure. I can not discover any disease in the walls of the orbit besides the enlargement above mentioned, which has apparently been produced by the long-continued pressure of the morbid growth. Diagnosis.—The object of diagnosis is the discrimination of diseases. "The science of diagnosis," says Louis, "is the most %portant, most useful, and most difficult of all the divi- sions S medical science. The discrimination of the peculiar nature V each kind of disease, and of its different species, is the source of curative indications. Without an accurate and precise diagnosis, theory is ever at fault and practice often unfaithful."* The object of diagnosis as applied to the case in hand, is to determine whether the morbid growth we have been describing * Vide Chomel's Pathology, p. 273. 10 is cancerous or benign in character ; whether it is developed from the eye itself, destroying that organ by its progressive enlargement, or from the soft parts behind the eye, occasioning ophthalmic protrusion (exophthalmos) and subsequent destruc- tion of the organ, by mechanical pressure : or, again, whether it is developed from the osseous wall of the orbit, or from parts still deeper seated, e. g., the brain ; in fine, whether excision of the tumor be possible, and, if possible, whether such excision would be beneficial to the patient. If the disease is benign, then its extirpation can be attempted with a reasonable expectation of curing the patient: if, on the other hand, it is carcinomatous, no such encouragement should be offered. The disease resembles carcinoma in several respects. The patient's general health is impaired, and she is thin, and pale, and weak. The swelling also is painful, and located in a situ- ation where most swellings of large size are at once strongly suspected to be cancerous; indeed, the disease had already been diagnosticated as carcinoma by more than one respectable medical man, and the case pronounced to be utterly hopeless. But the case presented other symptoms, which do not belong to carcinoma, and it was my appreciation of them which led me to a conclusion different from that arrived at by others who had seen the patient. It seemed to me that even the pallor and emaciation might be ascribed to the constant muco-purulent discharge from ^the conjunctiva, and to the severe neuralgic pain already mentioned, with fully as much propriety as to the constitutional infection of carcinoma; indeed, the patient's countenance, though pale, did not, as it appeared to me, present that peculiar hue and peculiar expression which are character- istic of the cancerous cachexy. Again : there was nothing about the pain characteristic of malignancy. No pain was occasioned by the tumor, until it became so large as to press upon the oph- thalmic branches of the fifth pair that run along the walls of the orbit, and the larger the tumor became, and the greater its pressure upon these nerves, the severer became the pain. The pain did not seem to be in the tumor itself, so much as in the neighboring parts, which were invaded mechanically by the morbid growth. Again : this tumor did not appear to affect the surrounding tissues, otherwise than by pressure. One of the most important characteristics of malignant disease is, as already stated, that it is prone to invade the surrounding tis- sues by infiltrating them with its own peculiar substancefand imposing upon them its own peculiar structure, thus contract- ing adhesions to surrounding parts, and, as it were, fusing them all together. The tumor under consideration has done nothing of the kind ; for, notwithstanding its great size, even the skin 11 covering it is healthy and non-adherent to it. A«-ain- mali