HEYL: Albuminoid(?) Deposit in Optic Disc and Retina. 3 55 such an extent that horizontal rupture occurred. It is interesting to note that the traction seems primarily to have been at the macula lutea and in the vertical meridian. If, instead of one sudden snap-like contraction of the externus, we conceive of a large number of minute spasms constantly recurring for months and years, we might have, instead of a rupture, simply pigmentary changes occurring in the macular region, and, perhaps, such macular changes as are seen in certain cases of myopia, are to be referred to the abnormal action of the externus. 3. The more accurate designation of choroidal rupture is retino-choroidal rupture. How far, indeed, the choroid coat is involved seems to be unknown, but, undoubtedly, it is the splitting the pigmentary layer of the retina, which is the conspicuous feature of the lesion.
ALBUMINOID (?) DEPOSIT IN THE OPTIC DISC AND RETINA. BY ALBERT G. HEYL, M.D., OF PHILADELPHIA.
The term albuminoid, as descriptive of the rare lesion described in this paper, is used with hesitation, owing to the absence of chemical proof of its existence. The general appearance of the deposits, as observed with the ophthalmoscope, seemed to indicate them to be a derivative of the albumen of the blood. The history of the case is as follows: Mrs. , Oe. 49, applied at the eye dispensary of the Episcopal Hospital Feb. 25, I895, for glasses for near work, 0. D. 20/15, 0. S. 20/20. A glance in the 0. D. revealed the condition shown in the drawing. The optic disc was covered with a series of round bodies, many of them of a diameter slig,htly greater than that of a retinal vein. They were apparently flattened and piled one upon the other; those immediately adjacent to the periphery being overlapped by those more centrally located. The ring-like limit
356 HEYL:
Albuminoid(.?) Deposit in Optic Disc and Retina.
of the disc was concealed by them, although the general outline was preserved. The larger bodies had a narrow white ring on the periphery, inside of which they were of smoky hue, deepening toward the center into a dark point, whether depended on the form of the body, or its nature, or to a turbid fluid en-
ALBUMINOID (?) DEPOSIT IN OPTIC NERVE AND RETINA.
closing them could not be determined. Certain portions of the disc seemed to have this smoky hue, and it seemed to be those in which the bodies were smaller in size, and, during the months the case has been under observation, this smoky hue seems to have extended until the whole disc is turbid, and the size of the large bodies has decreased, so that it may be that a turbid fluid has been forming in the process of retrogression. Situated upon the pigmentary layer of the retina and adjoining the disc margin were four patches of white waxy-looking material. They were underneath the nerve fibre layer. Two
HEYL: Albuminoid (?) Deposit in Optic Disc and Retina. 357 were above and two below, and each two were separated by a black interval of retinal pigment. This streak of pigment was peculiar in appearance; beginning at the disc with an apparent breadth of 1.5 millimeters, it gradually tapered to a point; it may have been produced by the absorption of the white material, or it may have been that originally the four masses were deposited as shown in the drawing. It seems to me that the latter is the better hypothesis. Close examination showed that the retina was extensively infiltrated with a faint white deposit, closely connected with the pigmentary layer, and lying underneath the nerve-fibre layer. This deposit, which I think was to great extent at first evenly diffused over the fundus, was essentially of the same nature as the white masses adjacent to the optic disc, but not nearly so thick and white. Four months later the white masses were becoming absorbed perceptibly. At this time, over the fundus punctate black spots began to show themselves, and then the existence of a milky deposit over the fundus was more clearly shown. A patch of these punctate spots are shown in the drawing below the disc. In the temporal region above the macula a group of white round bodies were observed, appearing to have been originally deposited in this form. They were somewhat smaller than the largest rounded bodies upon the optic disc, but whether identical with them could not be ascertained. In the nasal half of the fundus there was a solitary rounded body very similar in appearance to the disc bodies, and I judge of the same nature. It is represented in the drawing. The surface of the disc was convex, owing to the deposits. The blood-vessels on the disc were not overlapped by them. This is in accord with the results of microscopic investigation, which show that the masses of deposit are anterior to the lamina cribosa, and push the nerve fibre layer before them. In the L. E. the same condition existed, but in much less marked degree. Urine examined when the patient first seen and also four months later showed no albumen or tube casts. There was cedema of both lower extremities below the knees. Examination of the heart, by Dr. Harvey Shoemaker, showed the existence of a mitral systolic murmur. The patient was
358 HEYL: Albuminoid (?) Deposit in Optic Disc and Retina. given Fowler's solution of arsenic, and, under its use, the oedema of the lower limbs subsided and the white patches on the fundus began to disappear. Examination of the visual field showed a marked limitation of the nasal half of each eye, recalling the form of limitation seen in incipient glaucoma. Remarks. The pathological change observed in the right optic nerve and figured in the drawing is a conspicuous example of the "drusen " of the optic nerve. Under this name German writers have described small, round bodies situated in the optic nerve head; they occur in clusters and also singly, occupy the plane just anterior to the lamina cribosa, but posterior to the nerve fibre layer of the optic (lisc; cross sections show them to be laminated in structure, destitute of cells or nuclei; also they have within them a lime salt. A complete account of this lesion may be found in the paper by Hirschberg and Cirincione,* and also in that by G. De Schweinitz.t The significance of this pathological product is a mystery; why it should be located just anterior to the lamina cribosa, why it pushes the disc nerve fibres before it and does not infiltrate into retinal structure, and what is the real source of the hyaline material of which these bodies are composed may be looked upon as unanswered questions. The association in my case with a peculiar retinal deposit is worthy of note. The latter gave the impression as if from the inner surface of the pigmentary layer a white material had exuded, and in a special degree immediately about the periphery of the disc. There is a glistening white exudate lying upon and closely connected with the pigmentary layer of the retina, which I have several times seen, and which seems ophthalmoscopically to be different from the white masses seen in the retinitis of Bright's disease. It may be likened in appearance to the milkcurd produced by the action of rennet upon milk. It is unaccompanied by signs of acute inflammation, but seems to have the power, probably through pressure, to erode the pigmentary layer and to cause absorption of its cells. It is to this form of exudation that the white deposit seen in the drawing belonged, CCentralblattf. Prakt. Augenheilk., June and July, t Trans. Amer. Ophthalmolog. Soc., p. 349.
189I.
HEYL: Albuminoid(?) Deposit in Optic Disc and Retina. 359and it seems to me, therefore, this latter was not an inflammatory exudation, but one depending upon an abnormal state of the blood and analogous to what is commonly known as amyloid deposit, such as is seen in the liver or kidneys. Taking, then, this view, what remains to be said may be put in the form of question and answer. i. From what vessels was this white material transuded ? Evidently it was from the chorio-capillaris system. This system is adjacent to the pigmentary layer of the retina and no doubt is concerned in its tissue changes. Included in this system is what is usually described as the circle of Zinn, which furnishes a capillary net-work immediately about the optic disc, and also to the lamina cribrosa. Supposing, now, a transudation to occur from all parts of this system, we should expect to find it over the whole extent of the pigmentary layer and also anterior to the lamina cribrosa; accordingly, I think the white transudation seen in the fundus and the rounded bodies on the disc are of the same nature. 2. Why, then, does 'the transudation assume the form of rounded bodies in the disc and a diffuse layer-like deposit in other parts of the fundus? I think this depends upon the size of the capillary net-work. Any one who will glance at Leber's drawings of the net-work of the chorio-capillaris will find that the meshes over the lamina cribrosa are very large; immediately around the optic disc they are very fine, further away from the disc they become coarser. The result of this is that over the lamina cribrosa the transudation occurs in minute isolated points which again become coated with successive layers of transuded material until the body has become quite large; thus is explained the lamellated structure of these bodies. The latter is well shown in the microphotograph in deSchweinitz's paper. It would follow that if in the other parts of the fundus the capillary meshes were sufficiently large, we should find there the same rounded bodies. It may be that the rounded white bodies observed above the macula were of this nature as well as the single one to the nasal side of the disc. If, again, the meshes were very fine we might expect a confluent transudation, OPH.-8
360 HEYL: Albuminoid(.P) Deposit in Optic Disc and Retina. and this, I think, was the case over a large part of the fundus, especially immediately around the disc. 3. If this transudation depended uipon an altered condition of the blood how does it happen that the chorio-capillaris system was involved, while the retinal capillary system was unaffected? The answer is found in the different characters of the two systems. The chorio-capillaris system is supplied by from one to two dozen arteries, and the retinal system by one. The result of this, other things being equal, would be at least to increase the velocity of the capillary current as well as the escape of constituents of the blood into the surrounding tissues. The more one ponders this fact the more distinct will appear the physiological distinction which, no doubt, exists between these two systems. Thus, it might happen that in a certain abnormal state of the blood, transudation of the white material observed in this case might take place from one capillary system and not from the other. 4. What is the nature of this transudation ? (a) It can hardly exist within the capillaries in the form in which it was observed in the fundus, otherwise it would have formed capillary emboli. (b) It is capable of being absorbed. (c) Examination of the " drusen " of the nerve-head have 4emonstrated the existence of a lime-salt in them. When one remembers that the clotting of blood is due to interaction of two proteids, the fibrogen and another derived from the white corpuscles (wooldridge) in the presence of a lime-salt, or that the clotting of milk from the action of rennet requires also the presence of a lime-salt, we might readily suppose that the white deposit seen in this case was clotted material caused by the interaction of two proteids in the presence of a lime-salt. The albumen of of the blood may be one of these proteids. References: (I) Centralblat. f. Pract. Augenheilk, June and July, I89I; (2) Trans. Amer. Ophth. Soc., I894, p. 349.
ALBUMINOID (?) DEPOSIT IN THE OPTIC DISC AND RETINA. BY ALBERT G. HEYL, M.D., OF PHILADELPHIA.
The term albuminoid, as descriptive of the rare lesion described in this paper, is used with hesitation, owing to the absence of chemical proof of its existence. The general appearance of the deposits, as observed with the ophthalmoscope, seemed to indicate them to be a derivative of the albumen of the blood. The history of the case is as follows: Mrs. , Oe. 49, applied at the eye dispensary of the Episcopal Hospital Feb. 25, I895, for glasses for near work, 0. D. 20/15, 0. S. 20/20. A glance in the 0. D. revealed the condition shown in the drawing. The optic disc was covered with a series of round bodies, many of them of a diameter slig,htly greater than that of a retinal vein. They were apparently flattened and piled one upon the other; those immediately adjacent to the periphery being overlapped by those more centrally located. The ring-like limit
356 HEYL:
Albuminoid(.?) Deposit in Optic Disc and Retina.
of the disc was concealed by them, although the general outline was preserved. The larger bodies had a narrow white ring on the periphery, inside of which they were of smoky hue, deepening toward the center into a dark point, whether depended on the form of the body, or its nature, or to a turbid fluid en-
ALBUMINOID (?) DEPOSIT IN OPTIC NERVE AND RETINA.
closing them could not be determined. Certain portions of the disc seemed to have this smoky hue, and it seemed to be those in which the bodies were smaller in size, and, during the months the case has been under observation, this smoky hue seems to have extended until the whole disc is turbid, and the size of the large bodies has decreased, so that it may be that a turbid fluid has been forming in the process of retrogression. Situated upon the pigmentary layer of the retina and adjoining the disc margin were four patches of white waxy-looking material. They were underneath the nerve fibre layer. Two
HEYL: Albuminoid (?) Deposit in Optic Disc and Retina. 357 were above and two below, and each two were separated by a black interval of retinal pigment. This streak of pigment was peculiar in appearance; beginning at the disc with an apparent breadth of 1.5 millimeters, it gradually tapered to a point; it may have been produced by the absorption of the white material, or it may have been that originally the four masses were deposited as shown in the drawing. It seems to me that the latter is the better hypothesis. Close examination showed that the retina was extensively infiltrated with a faint white deposit, closely connected with the pigmentary layer, and lying underneath the nerve-fibre layer. This deposit, which I think was to great extent at first evenly diffused over the fundus, was essentially of the same nature as the white masses adjacent to the optic disc, but not nearly so thick and white. Four months later the white masses were becoming absorbed perceptibly. At this time, over the fundus punctate black spots began to show themselves, and then the existence of a milky deposit over the fundus was more clearly shown. A patch of these punctate spots are shown in the drawing below the disc. In the temporal region above the macula a group of white round bodies were observed, appearing to have been originally deposited in this form. They were somewhat smaller than the largest rounded bodies upon the optic disc, but whether identical with them could not be ascertained. In the nasal half of the fundus there was a solitary rounded body very similar in appearance to the disc bodies, and I judge of the same nature. It is represented in the drawing. The surface of the disc was convex, owing to the deposits. The blood-vessels on the disc were not overlapped by them. This is in accord with the results of microscopic investigation, which show that the masses of deposit are anterior to the lamina cribosa, and push the nerve fibre layer before them. In the L. E. the same condition existed, but in much less marked degree. Urine examined when the patient first seen and also four months later showed no albumen or tube casts. There was cedema of both lower extremities below the knees. Examination of the heart, by Dr. Harvey Shoemaker, showed the existence of a mitral systolic murmur. The patient was
358 HEYL: Albuminoid (?) Deposit in Optic Disc and Retina. given Fowler's solution of arsenic, and, under its use, the oedema of the lower limbs subsided and the white patches on the fundus began to disappear. Examination of the visual field showed a marked limitation of the nasal half of each eye, recalling the form of limitation seen in incipient glaucoma. Remarks. The pathological change observed in the right optic nerve and figured in the drawing is a conspicuous example of the "drusen " of the optic nerve. Under this name German writers have described small, round bodies situated in the optic nerve head; they occur in clusters and also singly, occupy the plane just anterior to the lamina cribosa, but posterior to the nerve fibre layer of the optic (lisc; cross sections show them to be laminated in structure, destitute of cells or nuclei; also they have within them a lime salt. A complete account of this lesion may be found in the paper by Hirschberg and Cirincione,* and also in that by G. De Schweinitz.t The significance of this pathological product is a mystery; why it should be located just anterior to the lamina cribosa, why it pushes the disc nerve fibres before it and does not infiltrate into retinal structure, and what is the real source of the hyaline material of which these bodies are composed may be looked upon as unanswered questions. The association in my case with a peculiar retinal deposit is worthy of note. The latter gave the impression as if from the inner surface of the pigmentary layer a white material had exuded, and in a special degree immediately about the periphery of the disc. There is a glistening white exudate lying upon and closely connected with the pigmentary layer of the retina, which I have several times seen, and which seems ophthalmoscopically to be different from the white masses seen in the retinitis of Bright's disease. It may be likened in appearance to the milkcurd produced by the action of rennet upon milk. It is unaccompanied by signs of acute inflammation, but seems to have the power, probably through pressure, to erode the pigmentary layer and to cause absorption of its cells. It is to this form of exudation that the white deposit seen in the drawing belonged, CCentralblattf. Prakt. Augenheilk., June and July, t Trans. Amer. Ophthalmolog. Soc., p. 349.
189I.
HEYL: Albuminoid(?) Deposit in Optic Disc and Retina. 359and it seems to me, therefore, this latter was not an inflammatory exudation, but one depending upon an abnormal state of the blood and analogous to what is commonly known as amyloid deposit, such as is seen in the liver or kidneys. Taking, then, this view, what remains to be said may be put in the form of question and answer. i. From what vessels was this white material transuded ? Evidently it was from the chorio-capillaris system. This system is adjacent to the pigmentary layer of the retina and no doubt is concerned in its tissue changes. Included in this system is what is usually described as the circle of Zinn, which furnishes a capillary net-work immediately about the optic disc, and also to the lamina cribrosa. Supposing, now, a transudation to occur from all parts of this system, we should expect to find it over the whole extent of the pigmentary layer and also anterior to the lamina cribrosa; accordingly, I think the white transudation seen in the fundus and the rounded bodies on the disc are of the same nature. 2. Why, then, does 'the transudation assume the form of rounded bodies in the disc and a diffuse layer-like deposit in other parts of the fundus? I think this depends upon the size of the capillary net-work. Any one who will glance at Leber's drawings of the net-work of the chorio-capillaris will find that the meshes over the lamina cribrosa are very large; immediately around the optic disc they are very fine, further away from the disc they become coarser. The result of this is that over the lamina cribrosa the transudation occurs in minute isolated points which again become coated with successive layers of transuded material until the body has become quite large; thus is explained the lamellated structure of these bodies. The latter is well shown in the microphotograph in deSchweinitz's paper. It would follow that if in the other parts of the fundus the capillary meshes were sufficiently large, we should find there the same rounded bodies. It may be that the rounded white bodies observed above the macula were of this nature as well as the single one to the nasal side of the disc. If, again, the meshes were very fine we might expect a confluent transudation, OPH.-8
360 HEYL: Albuminoid(.P) Deposit in Optic Disc and Retina. and this, I think, was the case over a large part of the fundus, especially immediately around the disc. 3. If this transudation depended uipon an altered condition of the blood how does it happen that the chorio-capillaris system was involved, while the retinal capillary system was unaffected? The answer is found in the different characters of the two systems. The chorio-capillaris system is supplied by from one to two dozen arteries, and the retinal system by one. The result of this, other things being equal, would be at least to increase the velocity of the capillary current as well as the escape of constituents of the blood into the surrounding tissues. The more one ponders this fact the more distinct will appear the physiological distinction which, no doubt, exists between these two systems. Thus, it might happen that in a certain abnormal state of the blood, transudation of the white material observed in this case might take place from one capillary system and not from the other. 4. What is the nature of this transudation ? (a) It can hardly exist within the capillaries in the form in which it was observed in the fundus, otherwise it would have formed capillary emboli. (b) It is capable of being absorbed. (c) Examination of the " drusen " of the nerve-head have 4emonstrated the existence of a lime-salt in them. When one remembers that the clotting of blood is due to interaction of two proteids, the fibrogen and another derived from the white corpuscles (wooldridge) in the presence of a lime-salt, or that the clotting of milk from the action of rennet requires also the presence of a lime-salt, we might readily suppose that the white deposit seen in this case was clotted material caused by the interaction of two proteids in the presence of a lime-salt. The albumen of of the blood may be one of these proteids. References: (I) Centralblat. f. Pract. Augenheilk, June and July, I89I; (2) Trans. Amer. Ophth. Soc., I894, p. 349.
