HARLAN: Thrombosis and Peri- Vasculitis.

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THROMBOSIS AND PERI-VASCULITIS OF THE RETINAL VESSELS. BY GEORGE C. HARLAN, M.D., PHILADELPHIA, PA.

WITHOUT being at all sure that this view is the correct one, I use the term Thrombosis as suggesting a probable explanation of a condition of the retinal vessels whose pathology seems to be involved in much obscurity. I refer to that condition in which they appear, in the ophthalmoscopic examination, as snow-white bands of the same, or nearly the same, width as the normal vessels. This rather rare ophthalmoscopic appearance is variously attributed by authors who have reported cases to thrombosis, embolism, ischaemia, peri-vasculitis, and inflammatory or degenerative opacity of the vessel-walls. It is very probable, of course, that the cause may be different in different cAses, and I will confine myself to the discussion of the following case, which is, at least, unusually well marked, and is, so far as I have been able to learn, unique in its completeness. The ophthalmoscopic picture that most nearly approaches it is a chromo-lithograph in Knapp's article on blindness in facial erysipelas. (Arch. of Ophth., vol. xiii.) In that, however, several of the larger vessels are still partially or completely filled with red blood. F. V., a healthy looking married woman, thirty-three years of age, applied at the Eye and Ear Department'of the Pennsylvania Hospital, June 5th, i886, with the following history. Her father and a younger sister died of Bright's disease, and her mother of paralysis. Her own general health had been good, except that for the last two years she had suffered a good deal with headache, which at times had been severe but was much less so of late. She had a miscarriag-e in July, i885, and was very ill for ten weeks, but recovered completely,

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HARLAN: Thrombosis and Peri- Vasculitis

She again miscarried in January, i886, but suffered little and was well in a few days. She had never had any trouble with her eyes, except that she was always short-sighted, until a few weeks before. On May 6th she read the paper in the evening with usual comfort, and there was nothing to call attention to her eyes. On rising the next morning she noticed a dimness of vision in the left eye, but, on testing it, found that she could still read large print with the right eye closed. The dimness increased during the day, and the next morning there was not even light perception in the left eye. There was no pain or other symptom than blindness, and the other eye was not affected. She had no competent advice at the time, but was examined two weeks afterwards by a well-known ophthalmic surgeon, who pronounced her case to be one of embolism. There was no indication of specific disease; her child, three years old, who accompanied her, was a picture of robust health, and her husband was a healthy-looking man. Dr. Richardson, the gynecologist of the out-door service, made an examination and pronounced her free from uterine disease. Dr. Fisher, who referred her from the medical clinic, reported that the heart sounds were normal. The sp. gr. of the urine was IOO9; there was some albumen, but no casts could be found. There was no light perception in the left eye. The media were clear. The ophthalmoscope showed hemorrhages scattered all over the retina; none of them, with two or three exceptions, at all dense. Some were striated and extended in long streaks by the sides of the vessels, were found at all the bifurcations and in a few spots concealed the vessels. Others were in large, rounded, faint patches, with difficulty distinguished from the surrounding fundus. At the macula there were three groups of white spots embedded in dark pigment. The disc was opaque-looking and snow white, its margins slightly blurred. One thread-like arteriole, arising independently near the centre of the disc, and passing upward about two disc diameters, contained red blood. With this exception, all the retinal vessels, both arteries and veins, were converted into white bands and lines, appearing as if marked

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out with chalk. Four or five experienced ophthalmoscopists searched repeatedly but in vain for a single red vessel. The vessels were nearly, if not quite, of normal size, possibly the arteries a little increased in width, and their margins were sharply defined. Fig. I., from a drawing made by Dr. P. N. R. Schwink, is an accurate picture of the fundus. Five or six weeks later the hamorrhages had all been absorbed, but there was no other change in the ophthalmoscopic picture. The spots on the vessels that had been concealed by hoemorrhages were left white like the rest. In the right eye there were several small h2emorrhages, and, on the temporal side of the disc, two indistinct, filmy, whitish patches. The macula was sound, and correction of a high degree of myopic astigmatism (-2.50) gave V. =a. The haemorrhages were afterwards absorbed, and the whitish films disappeared, leaving the fundus almost normal. The patient, who lives at some distance in the suburbs, promised to come on July I5th for another examination, but sent word that she had just had an attack of paralysis. My clinical assistant, Dr. Schwenk, visited her at her home and found a slight attack of partial hemiplegia of the left side. An examination of the urine now showed a small quantity of albumen and several hyaloid casts. Supposing the account given of the course of the disease to be correct, the rapidity of the onset and the occurrence of complete blindness in a few hours would seem to exclude opacity of the vessel-walls; and the history furnished by the patient appears to be quite positive and reliable on this point. She volunteered the statement that she had been reading-, without suspicion of her eyes, the evening before the trouble commenced, and was quite sure that she was still able to distinguish large print the next morning with the right eye closed. Embolism would not of itself account for the appearance of the vessels, particularly of the arteries; as its immediate effect upon a retinal artery is to make it invisible if the obstruction is complete, or reduce it to a slender red thread if the obstruction is partial. It is true that an artery partially emptied by embolus might refill and a clot be formed in the

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sluggish circulation, but even in this case the appearance of the vessel would still be due to thrombosis. I would venture to suggest that the partial blindness of the first few hours might be the result of haemorrhage, and the subsequent absolute blindness be due to thrombosis; though it must be admitted that a complete white thrombosis of a whole system of vessels, both arteries and veins, is contrary to our preconceived ideas, is opposed to experience in other regions, and has never been demonstrated in the eye. Gowers says, "thrombosis is occasionally observed in the smaller branches of the veins, which then lose their double contour, i. e. their central reflection disappears, and they appear dark and large, their branches being unusually conspicuous. The condition usually depends on local causes." Also, "'thrombosis has been observed in the retinal artery with ocular signs identical with those of embolism." Of course, a thrombosis with such signs could only be a plug of the main trunk, and not a clot extending the whole length of the vessel and involving its minutest branches. According to Ziegler, "coagulation may take place in blood which is at rest, or in blood which is in motion, and the appearance of the thrombus is different in the two cases. In blood at rest, coagulation takes place through the whole mass simultaneously. The thrombus is thus dark red or brown in color, and consists of granular and fibrous clots of fibrin containing multitudes of red corpuscles and a few white corpuscles. Red- thrombi of this kind are usually formed in occluded or hig,hly engorged vessels. * * * * "When coagulation begins in blood which is still flowing, the entire mass does not coagulate at once; minute flakes separate out, consisting chiefly of white blood cells with a varying number of red blood cells. In the case of pure white thrombi, only the colorless elements of the blood separate out. When the necessary local conditions for coagulation are set up at any spot, the clots adhere to the surface, and gradually increase in size by successive additions. * * * * "Thrombosis depends upon two factors; one is the morbid alteration of the vessel-wall, the other the retardation or stoppage of the blood current. In most cases the two

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factors are in action simultaneously." As to the second factor, as the heart was normal, there seems to have been no cause beyond the eye to retard the circulation; as to the first, the retinal hamorrhages, the albuminuria, the family history and the subsequent paralysis leave no doubt of a diseased condition of the vessels. I am not pathologist enough to know how long the condition I have supposed could continue without an organization of the clot, or just what changes in the appearance of the vessels are to be expected from that process. Ziegler believes that migrating white blood discs are chiefly concerned in the organization of thrombi, and that proliferation of the endothelium has little or nothing to do with it. Another possible explanation of this case occurs to me in the supposition that disease might have been progressing for some time in the outer walls or sheaths of the vessels, and, not interfering pinaterially with the circulation, might not have occasioned sufficient disturbance of vision to attract the patient's attention until the hmmorrhagre occurred; though this would scarcely account satisfactorily for the sudden occurrence of absolute blindness. The term "peri-vasculitis" occurs, I believe, only in ophthalmological literature, though a similar pathological condition has been observed, post mortem, in the vessels of the brain, where much the same anatomical structure of the peri-vascular sheath prevails. Wecker (Maladies des Yeux) refers to three observations of what he calls " peri-vascular retinitis." One is the description by Iwanoff of the examination of an injured eye that had been enucleated on account of sympathetic imitation of its fellow. All the retinal vessels were transformed into white cords. Some of the larger vessels had double the normal width; while others were less thickened, and, with a glass, globules of red blood could be seen within them. Under the microscope the interior of the vessels was found intact and filled with blood. The morbid change was in the external envelope or adventitious membrane, which was thickened and opaque. The microscopic appearances are given in detail. The second was a case uf a patient with albuminuria treated

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HARLAN: Thrombosis and Peri- Vasculitis

at Wecker's clinic from the commencement of the failure of vision. At first there was general opacity of the retina, particularly about the disc, the margins of which were obscured. Two or three weeks afterwards the retina commenced to clear and at the same time the larger vessels began to be surrounded by a white coat, which at certain points enveloped them entirely. This change advanced until all the arteries, which were particularly affected, were converted into white bands of a diameter a little larger than the normal calibre. In some places the red blood was entirely concealed, and in others could be seen through the thin coating. Wecker presented this case to his class as one of fatty degeneration of the adventitious membrane of the retinal vessels. The third case is quioted from Nagel, who however considers it to be one of hypertrophy of the cellular tissue of the vascular walls; all the arteries in both eyes, except a few slender arterioles, were converted into white cords. In some the blood could be seen as a fine red line along- the axes. Some peripheral branches of the veins were also affected. The patient's general health was good; blindness was not complete, and the progress of the morbid changes was very slow. Leber (Grefe-Samisch, v. 5, p. 530) speaks of fatty and chalky degenerationi of the vessels, and says that in marked cases of the latter the vessels may become stiff, white, opaque tubes. This occurs in eyes destroyed by intraocular inflammation. In nephritic retinitis, the walls of the vessels are sometimes changed into yellowish white glistening tubes, whose calibre is narrowed, and in some places occluded. "Pronounced fatty degeneration and calcification must naturally be associated with complete opacity of the vessel-walls, but this has as yet scarcely ever been seen ophthalmoscopically." Gowers says, " in very rare cases, there is such a thickening of the outer coat of the vessels, or an increase of its perivascular sheath, that the tissue is visible not merely on the sides of the vessel but in front of it, and broad white lines then indicate the position and course of the vessels. These may cease suddenly, and lengths of red blood may alternate with the white bands. * * * * In all inflammatory conditions,

IL.

SCMWENK. DEL.

GUTEKUNST, PHOTOTYPE.

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leucocytes may accumulate in the peri-vascular sheaths of vessels, and in the retina they may give rise to an appearance similar to that just described, and which has been termed "peri-vasculitis." It has been said by Liebreich, that by a careful examination of the relative width of the column of blood and of the white band, an opinion may be formed of the position of the new tissue, whether in or outside of the wall of the vessel. In the case just described, the white bands correspond so nearly in width to the normal calibre of the vessels, and their margins are so uniform and so clearly defined, that it is difficult to resist the conclusion that the appearance is due to changes within the vessels. Certainly the ophthalmoscopic picture does not closely correspond to the appearances noted in any of the cases that I have seen accurately detailed as peri-vasculitis. By way of comparison I present an ophthalmoscopic picture of another case that was under observation at the same time, and which seems to me to be one of opacity from peri-vasculitis. It is that of a negro woman, beyond middle age, partially blind for a long time, with no definite history, but with evident marks of extensive intraocular inflammation. The right eye was quite blind, with wide and firm posterior synechiae of the iris and dense inflammatory cataract. In the left eye V. _ -?f. There were several slig-ht synechiae, the lens was hazy, obscuring but not concealing- the fundus, the dicc was pale and there was a pigmented choroidal atrophy at the macula. A large artery running almost directly upward was completely converted into a white chalky-looking band with somewhat ragged margins and of varying width. There was no red blood seen in it from its entrance on the disc to the farthest poinlt to which it could be followed. In two smaller arteries passing downward the red color was replaced in parts by irregular segments of the same white appearance; and there were some smaller patches on one of the large veins. On this dead white ground were scattered numerous scintillating specks, which looked like crystals of cholesterine. (Fig. II.) 27

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JEFFRIES: Some Medico-Legal Cases

DISCUSSION. DR. NORRIS.-I have had an opportunity of examining this remarkable case, and I can bear witness to the accuracy of the picture. The appearances were absolutely limited to the distribution of the retinal vessels.

SOME MEDICO-LEGAL CASES UNDER STATE AND NATIONAL LAWS. By B. JOY JEFFRIES, M.D., BOSTON.

IN the Massachusetts Statutes, Chap. 194, as amended, is an Act relative to the employment by Railroad Companies of persons affected with Defective Sight or Color-Blindness, which reads as follows: SECT. I. No railroad company shall employ or keep in its employment any person in a position which requires him to distinguish form or color signals, unless such person has been examined for color-blindness or other defective sight, by some competent person employed and paid by the railroad companv, and has received a certificate that he is not disqulalified for such position by color-blindness or other defective sight. SECT. 2. A railroad company shall be liable to a fine of one hundred dollars for each violation of the preceding section.

The Act went in force July I, i88i. Its wording was designedly made so as to defeat the purpose of a law protective to stockholder, employe and the community. It is practically as dead a letter as the liquor laws, as I recognized that it would be when passed. In my Infirmary and private practice, cases quite frequently occur illustrating the truth of the above statement. Many more must naturally occur in the practice of my colleagues. Attention should be called to them. The following cases are typical of those I have records of. Any member of the Society is liable to be summoned in court as witness or expert in connection with such cases, and under the gravest circumstances.

Thrombosis and Peri-Vasculitis of the Retinal Vessels.

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