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.