Peri-Papillary Detachment of the Retina Accompanying Papilledema*

PERI-PAPILLARY DETACHMENT OF THE RETINA ACCOMPANYING PAPILLEDEMA*

ALGERNON B. REESE, M.D. New York The differentiation clinically between an active edema or inflammation of the disc as seen in optic neuritis and a pas- sive edema as seen in papilledemat is, as a rule, easy when the cases are typical. The neuritis which is characterized by exudate throughout the interstices of the nerve-fiber bundles of the disc and along the vessels, fine vitreous opacities, and often a marked visual disturbance on the one hand, and, on the other hand, the papilledema which is typi- cally that of a mushroom-shaped swelling of the disc, hemorrhages, and white areas of degeneration with com- paratively good vision for a long time, are known to be the extremes of the two groups. However, a swelling of the papilla not infrequently occurs which does not fall into such extreme groups of either condition, and then the classifica- tion as an optic neuritis or a papilledema is not so sharply demarcated. For instance, the swelling of the disc seen in albumiiiuric neuroretinitis is called by some an optic neuritis and by others, a papilledema. Also, to the border line group of cases belongs the swelling of the disc which appears as a consequence of hypotony of the globe. In these cases the ophthalmoscopic picture usually resembles an optic neuritis, but the vision may be unaffected if the media are clear. This swelling of the disc as a consequence of hypotony of the globe may be seen in the following conditions: * Candidate's thesis for membership accepted by the Committee on Thes-s. t For the sake of clarity the author has employed the term "optic neuritis" to designate active inflammatory edema of the disc and "papilledema" to designate passive non-inflammatory edema of the disc. 341 342 REESE: Peri-Papillary Detachment of the Retina I. Those that give rise to a filtration or fistulization of aqueous out of the anterior chamber through the cornea or limbus. A. Following cataract extractions when lens matter, lens capsule, or iris is allowed to remain between the wound lips, as a consequence of which delayed healing may occur, and thus fistulization take place. B. Following glaucoma operations, hypotony or relative hypotony may occur: a. In acute glaucoma. There sometimes appears a swelling of the disc and a congestion of its blood- vessels which resembles an optic neuritis but is usually regarded as a papilledema. b. In chronic glaucoma. The cupping of the disc sometimes disappears, which is due to the fact that the disc has become edematous, and in a month or so, when the edema disappears, the cupping reappears. C. Following perforating injuries of the globe even when the wound heals by primary intention and there is no infection in the interior of the eye. In these cases there is perhaps delayed closure of the wound because of the presence of lens matter, lens capsule, or the iris between the wound edges, or because of poor approximation of the wound lips. D. Following the perforation of an ulcer, the cicatrix of which afterward fistulates. II. Chronic diseases in and around the ciliary body which produce: A. Functional hypotony. This is due supposedly to the inhibition in the production of aqueous by the ciliary body. The more frequent diseases which give rise to this form of hypotony are spontaneous iridocyclitis, metastatic endophthalmitis in children, tuberculosis, syphilis, and tumors. If these diseases subside, the REESE: Peri-Papillary Detachment of the Retina 343 ciliary body may resume its function and the normal tension appear. However, very often in the course of these diseases and others the exudate which is thrown out around the ciliary body is organized into connec- tive-tissue membranes, the contracture of which leads to atrophy of the globe, and thus- B. Organic hypotony ensues, which is permanent. III. In cases of glaucoma with high tension which has been reduced suddenly to normal by intensive non-operative treat- ment. In such cases a papilledema is sometimes observed although no real hypotony exists, but only a relative one. The swelling of the disc from this relative hypotony dis- appears in a few days. IV. Contusions of the globe maygive rise to a papilledema which in part is probably due to the ensuing hypotony and in part to contrecoup. Hirschberg as early as 1888 called attention to the frequent association of a swollen disc with perforating wounds of the globe and with chronic diseases of the anterior part of the globe in which there is hypotony. He stated, however, that the condition is not often seen clinically because of the accom- panying cloudy media. Since that time many other cases of hypotonic conditions of the eye associated with swelling of the disc have been reported. In those cases in which the hypotony was secondary to either an inflammation in the anterior part of the eye (Uhthoff, Elschnig, Borgh) or to a perforating wound with infection (Stock, Borgh), there is a question as to whether or not the disc swelling was caused by the hypotony or by the spread of toxins from the anterior part of the eye posteriorly to the disc, thereby causing an optic neuritis. However, in those cases in which the hypotony was secondary to a perforation of the globe which healed by primary intention with absolutely no infection (Behr, Borgh) or to a filtrating congenital scleral cyst (Friede), or to a contusion of the globe (Horvath), the question of a toxic origin of the disc swelling does not come into consideration. 344 REESE: Peri-Papillary Detachment of the Retina Those cases which were examined microscopically showed a passive edema of the papilla which extendedfor a shortdis- tance in the fiber layer of the retina, and posteriorly as far as the lamina cribrosa. This edematous swelling of the papilla was accompanied by no inflammatory signs other than a few lymphocytes and occasionally a slight peri- vascular infiltration of lymphocytes. According to Kam- pherstein, the swelling was confined mainly to the central part of the papilla, thereby obliterating the physiologic cup, whereas in papilledema accompanying increased intra- cranial pressure the peripheral part of the papilla is more markedly edematous. The writer has observed this and attributes it to the fact that in cases of hypotony the swelling is permitted to confine itself more to the central part of the papilla and even extend inward toward the vitreous because of the subnormal intra-ocular tension, whereas in papilledema following increased intracranial pressure the swelling is forced over a broader area of the papilla because of the normal intra-ocular tension. According to Elschnig, in cases of papilledema due to hypotony the lamina cribrosa remains in its normal position in contrast to cases of papill- edema following increased intracranial pressure, in which the lamina cribrosa, instead of being normally convex posteriorly, is apparently pushed inward and is convex anteriorly. This edema of the papilla following hypotonic conditions of the globe is properly classified as a papilledema because microscopically it is a non-inflammatory passive edema of the nerve head in contrast to the inflammatory active edema seen in optic neuritis. Its occurrence is not dependent on any infection or toxins present in the eye, for it is observed following: 1. Clean operations (Elliot trephine, cataract extractions, etc.). 2. Non-infected perforating wounds of the globe (Hirsch- berg, Fehr, Borgh, Hoppe). REESE: Peri-Papillary Detachment of the Retina 345 3. Congenital filtrating cyst of the sclera (Friede). 4. Simple contusions of the globe (Horvath). Several explanations for papilledema have been advanced, but they have usually concerned that type which follows increased intracranial pressure, and have disregarded the type following hypotony and that accompanying general diseases and blood affections. The explanation for papill- edema which is probably more generally accepted today than any other, and which best covers every type of papilledema from whatever cause and which is perhaps best supported by experimental and clinical evidence, is the so-called lymph- stasis theory. This has been championed by Kines, von Michel, and, more recently, fully and convincingly by Behr. It claims that the fluids given off by the tissues of the retina and papilla normally pass out through the optic nerve by way of the perivascular lymph channels in the central sup- portingtissue strand. For the outflow of this lymph it is nec- essary that the intra-ocular tension be greater than the intra- cranial. In the case of papilledema from increased intra- cranial pressure this relation is reversed by the intracranial pressure being abnormally elevated above that of the normal intra-ocular pressure, whereas in the case of hypotony of the globe the normal intracranial pressure is relatively elevated above that of the abnormally reduced intra-ocular pressure. This reversal of the relation between the two pres- sures causes a lymph stasis in the papilla, or papilledema, because the necessary vis-a-tergo is wanting, and the tissue fluids therefore accumulate in the papilla, where the intra- ocular pressure is least exerted. The edema of the papilla is not seen posterior to the lamina cribrosa because the intra-ocular pressure at this level has no influence. Also, the edema increases gradually more and more from the lamina cribrosa toward the apex of the papilla. All forms of papilledema from whatever cause have the same mechanical explanation, i. e., a lymph stasis in the nerve itself. The 346 REESE: Peri-Papillary Detachment of the Retina explanation of the cause varies according to the condition provoking the stasis. Behr's classification of all forms of papilledema is as follows: A. Following passive lymph stasis of the papilla: I. Through compression of the optic nerve and through it the central path of lymph conduction: (a) 1. Intracranial conditions with increased intracranial pressure such as tumors, abscesses, internal hydro- cephalus, etc. 2. Intracranial conditions without increased intra- cranial pressure, the lesions of which are around the optic foramen, such as aneurysms of the internal carotid artery, oxycephalus, etc. (b) Intra-orbital conditions such as tumors, inflammation, hematoma, hemorrhage in the optic nerve sheaths, etc. II. Through retention of the tissue fluids of the papilla as a consequence of marked hypotony of the bulb following perforating injuries, diseases of the anterior half of the globe, etc. 0 B. Following active lymph stasis of the papilla caused by increased outflow of lymph because of functional injury or damage to the blood-vessel walls. I. In general diseases and blood affections, such as ne- phritis, chlorosis, leukemia, polycythemia, etc. II. In contusions of the globe as a result of contrecoup. Papilledema is not seen in all cases of hypotony, such as every slowly closing wound following a cataract extraction and every perforating wound, because apparently the hy- potony must exist at least three weeks and be of a marked degree to give rise to a papilledema. However, in the case reported by Horvath of a choked disc accompanying hypotony as a consequence of a contusion of the globe, the choked disc made its appearance in three days. Also, the papilledema seen anatomically in cadaver eyes consequent REESE: Peri-Papillary Detachment of the Retina 347 to the postmortem hypotony of the globe appears in a few days instead of the usual period of about three weeks or more. It may be possible for the edema to appear in the course of days when the hypotony is of an extreme degree. If hypotony can cause a papilledema, then one should find it in cases of atrophy of the globe. In a microscopic study of 100 atrophic globes due to numerous pathologic condi- tions, the writer has found papilledema present in 45 per cent. of the cases. The edema was confined to the papilla, and although definitely present, it was in many cases not particularly striking, and did not give rise to a great eleva- tion of the papilla. It was important and sometimes diffi- cult in these cases to decide whether a passive edema of the papilla was present or an optic neuritis secondary to the factor causing the atrophy of the globe, but only those cases which showed an unmistakable passive, non-inflammatory edema of the papilla were listed as papilledema. Of course, this is not seen clinically, for almost without exception the condition of the media or a detached retina prevents an ophthalmoscopic examination of the disc. Among the forty- five cases which showed a papilledema there were two which had developed glaucoma with cupping of the disc secondary to an iridocyclitis, and later the globe became atrophic and showed microscopically an edema of the edges of the disc; in other words, a cupping of the disc and a papilledema were present in the same disc. Those cases of atrophy of the globe which did not show a papilledema were usually mark- edly degenerated eyes in which perhaps all lymph passages were disarranged, or they were cases in which the detached retina lay in the mid-line in apposition, in which case usually the entire retina was edematous and cysts of the retina were common. The detachment of the retina commonly present in atrophic globes is, of course, usually due to traction result- ing from contracture of connective-tissue membranes. But 348 REESE: Peri-Papillary Detachment of the Retina there occurs sometimes in hypotonic globes, other than atrophic ones, a peri-papillary or perhaps a circumpapillary detachment of the retina caused by the papilledema. The explanation of this fact is evident, i. e., that the protrusion of the papilla toward the interior of the eye pulls the ad- jacent retina with it, because the intra-ocular pressure is not sufficient to hold the retina in apposition to the choroid over this circumpapillary area. An example of such a peri-papillary detachment of the retina is the following: A boy, aged thirteen years, had an endogenous abscess of the vitreous confined to the anterior portion of the globe in the region of the ciliary body. Hypotony ensued. No evidence of injury. Microscopic Examination. Posterior to the lens in the lower half of the globe was an abscess of the vitreous. It was well encapsu- lated with scar tissue, which by its contracture had pulled the ciliary body inward, causing a detachment of it and the choroid as far as the insertion of the recti muscles. The retina and posterior capsule of the lens were pulled into the contracting fibrous capsule to a slight degree. The former, therefore, showed a narrow slit detachment along the ora serrata over the lower half of the globe. No membranes were noted along the surface of the retina or in the vitreous. The optic nerve head (fig. 1) protruded into the vitreous almost 2 mm., carrying with it the adjacent retina and thereby detaching it over an area of 0.75 mm. around the disc. Under the detached retina was the usual eosin-staining albuminous fluid. The papilla and the surrounding retina for about 3 mm. were markedly edematous. The retinal edema was confined mostly to the fiber layer, because of which the external retinal layers sur- rounding the disc were thrown into folds. There were several dilated blood-vessels in the fiber layer around the papilla. The edema obliterated the physiologic cup. There was no infiltration of leukocytes and no exudate indicative of an inflammatory reaction. The lamina cribrosa was in normal position, and marked the posterior limit of the optic nerve edema. The intervaginal space was normal. This patient had a metastatic infection in the anterior part of the eye, in the region of the ciliary body. Hypotony Fig. 1.

Ii

Fig. 3.-This microphotograph is of a section tangential to the disc which serves best to show the juxta-papillarv detachment of the retina. A. the eosin staining albuminous fluid under the detached retina. B, the edematous nerve head at its margin. C, a tangential section of the optic nerve. D, an artefact (piece of choroid detached in mounting). E, artefactous separation of choroid from sclera. REESE: Peri-Papillary Detachment of the Retina 349 ensued, which gave rise to a papilledema and circum- papillary detachment of the retina. Although the globe was atrophic, this circumpapillary detachment of the retina was not due to contracture of connective-tissue membranes, which is the usual cause of retinal detachment in atrophic eyes, but was due to the fact that the protrusion of the edematous nerve head detached the retina around the disc, because the subnormal intra-ocular tension was not suffi- cient to hold the retina over this area in apposition to the choroid. The edema of the nerve head was non-inflam- matory and is regarded as a papilledema secondary to the hypotony and not an optic neuritis secondary to the in- flammation in the anterior part of the eye. These anatomic findings have a definite clinical bearing for they serve to explain and substantiate the following case :* A woman, aged fifty-eight years, had a cataract extraction per- formed on her right eye two years ago. Examination. -V. 0. D. with +12 sph. = 3/60. With +3 sph. added counts J 7. T. = -3. Along the upper part of the sclerocorneal junction the incision for the cataract operation was visible and around it, particularly in its central portion, the aqueous was filtering out under the conjunctiva causing slight elevation of it. The anterior chamber was somewhat shallow. The iris did not seen to be incarcerated in the wound, nor was any lens capsule visible in the wound. The iris did not appear to be congested. No deposits were visible on the posterior surface of the cornea. The pupil was clear and the fundus plainly visible. Ophthalmoscopic Examination (fig. 2).-The papilla protrudes forward 4 diopters and is somewhat grayish in color, with indistinct borders. The blood-vessels on its surface are slightly distended and tortuous, and partly obscured in places, especially toward the center of the disc. The blood-vessels can be seen descending from the level of the papilla to the lower level of the retina, and inferiorly the border of the papilla seems to be overhanging so that the blood- vessels disappear and reappear in descending from the papilla to the retina. On the nasal side of the swollen disc is an indistinctly outlined grayish area of the retina raised above that of the sur- * This case was studied at the Meller Clinic in the Allgemeine Krankenhaus, Vienna, Austria, through the courtesy of Prof. Adalbert Fuchs. 350 REESE: Peri-Papillary Detachment of the Retina rounding retina. This area extends out from the disc for about 1.5 disc-diameters. Over this area the blood-vessels are somewhat tortuous and darker than elsewhere. On the temporal side is a similar area extending toward the macular region and in this area is a round bright hemorrhage. The measurement of the blind-spot shows an enlargement, to the temporal side, of 30 degrees, including the macula, and to the nasal side of 20 degrees. There is also an enlargement above and below of 15 degrees. 0. S. Negative except for incipient cataractous changes. This patient had a cataract extracted, at which time the technique of the operation was apparently satisfactory and there were no mishaps. Following the operation the globe remained hypotonic because of the filtration of aqueous from the anterior chamber through the cataract incision. This perhaps was due to the fact that a piece of the lens capsule remained between the wound lips. A papilledema appeared secondary to the hypotony. The protrusion of the disc in the presence of the lowered intra-ocular tension caused a peri-papillary detachment of the retina temporally and nasally. The retinal detachment temporally included the macular region and thus the visual acuity was reduced. In this presentation so far it has been the purpose of the writer to show that a papilledema secondary to hypotony of the globe can cause a detachment of the retina adjacent to the papilla, because the lowered intra-ocular pressure is not sufficient to hold the retina in apposition to the choroid in the presence of the protruding disc. In regard to papilledema in the presence of normal intra-ocular pressure such as that commonly seen secondary to increased intracranial pressure (brain tumors, hydrocephalus, etc.), the writer believes that a similar peri-papillary detachment of the retina can and does exist, but, to be sure, to a lesser degreethan seen accompanying papilledema in hypotonic globes. These peri-papillary de- tachments of the retina accompanying papilledema in globes with normal intra-ocular pressure are so slightly elevated and of such small extent that they are not detected oph- thalmoscopically. Fig. 2 REESE: Peri-Papillary Detachment of the Retina 351 The following case illustrates this: A child, aged eight years, had an intracranial tumor (glioma of the cerebellum). The discs showed marked choking. No detach- ment of the retina was noted around the papilla. Microscopic examination showed a detachment of the retina for 2 disc-diameters around the edematous nerve head (fig. 3). That this detachment was not artefactous was evidenced by the usual eosin-staining albuminous fluid in the subretinal space under the detached portion of the retina. This detachment was not detected ophthalmoscopic- ally, perhaps because of its slight degree and because the retina over this area was somewhat opaque from the papilledema. This occurrence of a peri-papillary detachment of the retina consequent to papilledema can serve to explain the excessively enlarged blind spot often associated with a papilledema from intracranial lesions. A retina detached from the choroid, no matter how slight the detachment, does not function, for the rods and cones must be in apposition to the pigment epithelium for them to initiate the visual impulse. CONCLUSIONS 1. A peri-papillary detachment of the retina can accom- pany a papilledema from hypotony of the globe. 2. A peri-papillary detachment of the retina can accom- pany a papilledema from increased intracranial pressure. 3. A peri-papillary detachment of the retina is no doubt often a factor in causing enlargement of the blind spot from papilledema. REFERENCES Hirschberg: Ophthalmoskopie in Eulenburg's Real. Enzyklopadie, 1888. Elschnig: Wien. klin. Rundschau, 1902, xix. Stock: Klin. Monatsbl. f. Augenh., 1903, xli, p. 100. Hirschberg: Klin. Monatsbl. f. Augenh., 1904, p. 521. Fehr: Centralbl. f. prakt. Augenh., 1904, p. 46. Hirschberg: Klin. Monatsbl. f. Augenh., 1904, xli, p. 111. Uhthoff: Klin. Monatsbl. f. Augenh., 1908, p. 359. Kampherstein: Kln. Monatsbl. f. Augenh., 1904, p. 518. Borgh: Kln. Monatsbl. f. Augenh., 1908, p. 359. Hoppe: Klin. Monatsbl. f. Augenh., 1908, p. 468. Horvath: Klin. Monatsbl. f. Augenh., lxxi, p. 698. Behr: Klin. Monatsbl. f. Augenh., 1912, p. 56. Friede: Khln. Monatsbl. f. Augenh., 1920, p. 783. Behr: Graefe's Arch. f. Ophth., 1920, ci, p. 206. Behr: Kln. Monatsbl. f. Augenh., lxxi, p. 698.