T H E CLINICAL COURSE O F SERPIGINOUS CHOROIDOPATHY HAROLD WEISS, Detroit,
M.D.
Michigan
W I L L I A M H. A N N E S L E Y , J R . , M.D., J E R R Y A. S H I E L D S , AND T E R R A N C E T O M E R , B.A. Philadelphia,
M.D.,
Pennsylvania
AND KENNETH CHRISTOPHERSON, Detroit,
Serpiginous choroidopathy is a pro gressive condition that primarily affects the choriocapillaris and retinal pigment epithelium and often leads to profound visual loss if the foveal or parafoveal areas are involved. It is almost always bilateral and thus far has been described only in whites, although we have seen two cases in blacks. The cause remains obscure. The clinical appearance and fluorescein angiographic findings in serpig inous choroidopathy have been well de scribed. 1 - 3 The long-term follow-up and clinical course of this progressive disease, however, have not been well docu mented, particularly with regard to the prognosis for central vision. Chisholm, Gass, and Hutton 4 provided the only available information in their series of 40 eyes observed from single visits to nine years. A significant reduction in visual acuity was seen in only three eyes of their series, although many started out with poor vision. We report herein the clinical course of this disease, particu larly with regard to visual acuity. From the Kresge Eye Institute, Wayne State University, Detroit, Michigan (Dr. Weiss and Mr. Christopherson); and the Retina Service, Wills Eye Hospital, Philadelphia, Pennsylvania (Drs. Annesley and Shields, and Mr. Tomer). This study was supported in part by the Retina Research and Development Foundation, Philadelphia, and the Lions Club of Pennsylvania. Dr. Weiss was a Heed Foundation Fellow (1975-1976). Reprint requests to Harold Weiss, M.D., Kresge Eye Institute, 3994 John R St., Detroit, Ml 48201.
B.S.
Michigan S U B J E C T S AND M E T H O D S
Of the nine patients in our study, seven were men and two were women. Their average age was 46 years with a range of 22 to 58 years. All the patients were bilaterally affected except for one patient who lost an eye in childhood because of trauma, so that there was a total of 17 eyes in our study. The period of follow-up ranged from two to ten years. We reviewed all patients with serpigi nous choroidopathy seen here who had a minimum of two years follow-up. Nine patients met this criteria. All patients had multiple fundus photographs and fluorescein angiograms, and had been seen by us on multiple occasions for clinical ex amination. Eight out of nine patients were recalled, and detailed ocular, sys temic, and social histories were taken together with complete ocular exami nation, indirect ophthalmoscopy, and slit-lamp biomicroscopy with a fundus contact lens. Fundus photographs and fluorescein angiograms were taken. Electroretinography and electro-oculography as well as visual fields were performed. CASE REPORTS Case 1—A healthy 48-year-old white man noticed painless decreased vision in his right eye in Septem ber 1966. His past medical and family history were unremarkable. The patient had worked in a plastic plant since 1959, and had been exposed constantly to asbestos, phenol, phenole resins, furfuryl, furene resins, paraformaldehyde, hydrochloric acid, for maldehyde, epoxy resins, silica fibers, polypropyl ene, polyethylene, and polycarbonate.
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On examination Sept. 9, 1966, best corrected visu al acuity was R.E.: 6/24 (20/80) and L.E.: 6/6 (20/20). External and slit-lamp exam inations were normal. The right fundus showed a gray discoloration of the pigment epitheli um on the nasal edge of the fovea, and the left fundus was normal. Visual acuity in the right eye gradually improved over the next six months to 6/6 (20/20). At the onset of his visual loss, the patient was given oral prednisone, 40 mg/day for three months, after which the dosage was gradually ta pered and discontinued. Four years later visual acuity in his left eye decreased to 6/60 (20/200). Examination revealed a typical active area of a gray discoloration of the pigment epithelium. In contrast to the right eye, the
F E B R U A R Y , 1979
active area involved the whole fovea, rather than just its margin. Visual acuity in the left eye remained at 6/60 (20/200) for seven years. Since May 1970, the patient had experienced no change in vision and although his visual acuity remained stable at 6/6 (20/20) in the right eye and 6/60 (20/200) in the left eye, his disease continued to progress (Fig. 1). The asymptomatic progression of the disease was not recognized until the serial fun dus photographs were reviewed, which showed cen tripetal progression from the fovea toward the disk. The disease was characterized by periods of activ ity alternating with periods of quiescence. In the active stage, a localized area of gray-green or creamcolored opacification developed beneath the retina at the level of the retinal pigment epithelium. The
Fig. 1 (Weiss and associates). Case 1. Top left, Appearance of the posterior pole of the right eye in May 1970. The fovea has been spared and visual acuity was 6/6 (20/20). Top right, Although asymp tomatic, the patient was seen again in December 1971. Visual acuity remains 6/6 (20/20). Note the extension of the scar toward the disk (arrows). Bot tom left, Five years later, the patient stated his right eye had not changed. However, the scars had en larged by careful comparison with the photographs taken five years earlier (arrows).
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area of activity was often, but not always, adjacent to an old choroidal scar. Over a period of weeks, the area of new activity showed mottled pigmentation and eventually atrophy of the retinal pigment epi thelium and choriocapillaris resulted. There were no instances of serious elevation of the retina over these active areas and no evidence of subretinal neovascular membranes was found. Case 2—A healthy 22-year-old white man was first seen in April 1974, with a three-week history of blurred vision in his right eye and a one-week history of blurred vision in his left eye. His occupa tional history revealed exposure to various types of paint, diesel fuel, and domestic animals. On examination April 9, 1974, best corrected visual acuity was R.E.: 6/12 (20/40) and L.E.: 6/6 (20/20). External and slit-lamp examination were normal. The fundi of both eyes showed a large area of pigment alteration below which was a broad active front of gray discoloration of the pigment epthelium (Fig. 2, top left). Similar areas were present around the disk (Fig. 2, top right). In the equatorial area of the right eye at the 12 o'clock position, there was perivenous sheathing with some nearby intraretinal hemorrhages (Fig. 2, middle
left).
Three days later, the visual acuity in the right eye had deteriorated to 6/60 (20/200) while the visual acuity in the left eye had remained 6/6 (20/20). Fundus examination showed that the active front had extended down to involve the fovea of the right eye and was moving closer to the fovea of the left eye. The medical and neurologic examinations were normal as were an extensive battery of x-ray and laboratory studies including skin test for coccidiomycosis, histoplasmosis, tuberculosis; sedimenta tion rate, FTA-abs, Anti-nuclear antibody, and lupus erythematosis preparation. Electroretinography and electro-oculography were normal. Eleven days later, visual acuity in the right eye remained 6/60 (20/200) and in the left eye had dropped to 6/15 (20/50). Fundus examination showed that the active fronts had continued to progress inferiorly in both eyes across the fovea (Fig. 2, middle right). Pluorescein angiography was performed (Fig. 2, bottom). Visual acuity in the left eye dropped to 6/30 (20/100), but one month later visual acuity had improved to 6/24 (20/80) in the right eye and 6/21 (20/70) in the left eye. The lesions had extended to the inferior arcades leaving extensive pigment alter ation across the entire posterior pole. Two months after initial examination, visual acu ity had improved to R.E.: 6/6 (20/20) and L.E.: 6/21 (20/70). Four months later the visual acuity in the left eye had improved to 6/18 (20/60) and by six months had returned to 6/6 (20/20). When re-examined 2V2 years later the- patient stated that there had been no change in vision since the initial course of his disease. Comparison of fundus photographs, however, showed extension of scarring along the superior arcades and around the
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disk in the left eye, marked pigment clumping, and several areas of subretinal fibrous proliferation. This patient is remarkable in the symmetry of his disease, his visual recovery, and the perivascular sheathing. The visual recovery possibly can be at tributed to a failure of the disease to destroy the choriocapillaries in the fovea. Case 3—A 44-year-old white man developed a spot on his right eye in July 1970. At the time, the patient was working in a sheet metal shop and was exposed to various fumes of unknown type. Since 1958, he had performed greenhouse work and was exposed to vapone, various kinds of fertilizer, malathrone, Triban, and nicotine bombs. When first seen on Sept. 16,1970, his visual acuity was 6/6 (20/20) in both eyes. External and slit-lamp examinations were normal. Fundus examination re vealed multiple serpiginous and isolated choroidal scars in both eyes. A large scar extending from the disk to just above the fovea in the right eye appeared with modest pigment mottling. In October 1970, the patient had a systemic exam ination. Significant findings were a positive tubercu lin skin test (second strength), a dental abscess, and mild chronic maxillary sinusitis. He was given isoniazid, 300 mg daily, and pyridoxine HC1, 50 mg daily. The patient was examined regularly; in July 1974 an active lesion developed in the left macular area, nasal to an older choroidal scar (Fig. 3, top left). Visual acuity in the left eye was reduced to counting fingers. No new activity in the right eye was noted, and the patient stated that there had been no change in his vision. However, on careful review of the fundus photographs, extension of the round scar below the fovea had occurred in a centripetal man ner toward the disk. The patient's left eye was treated with a retrobulbar injection of 40 mg of methylprednisolone acetate (Depomedrol). Six weeks later, visual acuity had improved to 6/60 (20/200) in the left eye and the area of previous activity showed pigment mottling (Fig. 3, top right). Additionally, several isolated round areas of fresh activity were present along the superi or arcades. In March 1975, visual acuity in the left eye had decreased to counting fingers. Fundus examination revealed a resolving lesion with pigment mottling involving the foveal area. The right eye was un changed. In June 1976, visual acuity was counting fingers in the left eye and 6/15 (20/50) in the right eye. In the left eye, there was further extension of the scarring, which involved a broad area of the posterior pole bounded by the vascular arcades (Fig. 3, bottom left). No new activity was present at this time. The electroretinogram and electrooculogram were both normal. Goldmann perimetry of the right eye showed scotomas of variable depth corresponding to the fundus lesions (Fig. 3, bottom right). The patient showed asymptomatic progres sion, centripetal progression, and temporary visual recovery. Case 4—A healthy 46-year-old white man devel-
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-< Fig. 2 (Weiss and associates). Case 2. Top left, Posterior pole of the right eye on April 9, 1974, showing a broad active front (arrows) extending down across the posterior pole, leaving extensive pigmentary changes in its wake. Top right, Simultaneous activity was also present around both disks. Middle left, Equatorial area of the right fundus showing focal and diffuse areas of perivenous sheathing. Middle right, Eleven days later, the active front extended inferiorly (arrows). Bottom, Fluorescein angiography of the right eye in middle right. The active front is densely hypofluorescent in the early phase (arrows) (bottom left); and diffusely hyperfiuorescent in the late phase (arrows) (bottom right). The older areas of involvement near the superior vascular arcades show extensive pigment changes with mottled hyperfluorescence.
oped a sudden onset of reduced visual acuity in the left eye in 1970, which continued to deteriorate until 1974. Visual acuity remained good in his right eye until January 1974, when he suddenly noted blurring of vision. The patient was a race horse trainer
and was exposed to a variety of liniments as well as dimethyl-sulfoxide. On examination Jan. 14, 1974, best-corrected visual acuity was 6/15 (20/50) in the right eye and counting fingers in the left eye. External and slit-
Fig. 3 (Weiss and associates). Case 3. Top left, Fundus photograph of the left eye in July 1974, showing the area of fresh activity (arrows) involving the fovea and demonstrating a grayish opacification at the level of the pigment epithelium. Top right, Six weeks later, there was pigment mottling where the active lesion had been (arrows). Bottom left, Fifteen months later, there was extensive scarring now involving most of the posterior pole.
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lamp examinations were normal. In the posterior pole of the left eye was a large serpiginous area of loss of the pigment epithelium, choriocapillaris, and inner choroid (Fig. 4, top left) without any new activity. In the right eye, an active area of gray opacification at the level of the pigment epithelium was present at the inferior margin of the fovea. Several small choroidal scars were present nasal to the fovea (Fig. 4, top right). The patient was treated with oral prednisone, 40 mg daily, for six months and over this period of time visual acuity in the right eye gradually improved from 6/15 (20/50) to 6/6 (20/20). In June 1976, the patient stated that his vision had not changed over the past two years. However, the appearance of the fundus in both eyes had changed.
FEBRUARY, 1979
In the left eye, the large serpiginous scar had extend ed in a centrifugal manner temporally and along the superior arcades. In the right eye, additional scars were present superior to the macula and the area of new activity in 1974 showed pigment dispersion and atrophy of the inner choroid (Fig. 4, bottom). The patient showed both visual recovery following parafoveal involvement and asymptomatic progres sion in the right eye. RESULTS
The clinical course of the disease in all of the patients was characterized by mul tiple recurrences with the development of
Fig. 4 (Weiss and associates). Case 4. Top, Fundus appearance of the right and left eyes in January 1974. The active area in the right eye is indicated by arrows. Bottom left, Two years later, additional scarring was present superioral to the fovea and the area of activity seen two years previously be neath the fovea demonstrated pigment changes and loss of choriocapillaris.
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new scars and extension of old scars. There was often a discrepancy between the patients' histories regarding progres sion of their disease and the findings on fundus examination. Five of the patients were unaware that their disease had con tinued to progress because their recur rences were not near the macula and were, therefore, not recognized. Progres sion might not have been detected by the examiners if serial fundus photographs had not been available. Most of the pa tients had multiple and complex chorioretinal scars and new areas of activity or scarring were sometimes subtle and de tectable only by close scrutiny of serial photographs. Serial fundus photography provided us with the information that the disease may progress in the absence of symptoms and that progression can con tinue for up to ten years, the maximum duration of our follow-up. Most of the eyes in our series showed progression of the disease in a centrifugal manner, beginning near the disk and ex tending outward toward the periphery. However, in three eyes in our series, there was either centripetal progression or new activity in an area that had been previous ly bypassed. In one of these cases, visual acuity was permanently affected. Visual acuity was affected only when the foveal or parafoveal areas were direct
ly involved by the disease. In nine of the 17 eyes, visual acuity dropped to a varia ble degree during foveal or parafoveal involvement, but subsequently improved as the acute lesion resolved (Table 1). In some cases, visual recovery was re markable despite significant initial reduc tion in vision. In another six of the 17 eyes in our series, the fovea or parafoveal areas were affected and visual acuity dropped but did not recover. Final visual acuities were 6/12 (20/40) (one eye); 6/15 (20/50) (one eye); 6/30 (20/100) (one eye); 6/60 (20/ 200) (one eye); hand movements (two eyes). In the remaining two of the 17 eyes, the foveal and parafoveal areas were not involved at all and visual acuity was unaffected. At the conclusion of our study, no pa tient was legally blind and only three of the 17 eyes had visual acuity of less than 6/60 (20/200) (two had hand movements as reported above, and one eye that recov ered visual acuity to 6/30 (20/100) subse quently developed foveal involvement again and became counting fingers). Final visual acuity in these 17 eyes is shown in Table 2. Foveal or parafoveal involvement with its attendant reduction in visual acuity is common in serpiginous choroidopathy and occurred in 15 of the 17 eyes. The
TABLE 1 PATIENTS WHO DEMONSTRATED RECOVERY OF VISUAL ACUITY AFTER FOVEAL OR PARAFOVEAL INVOLVEMENT
Patient No.
Worst Visual Acuity
1 2 3 4 5 6 7 8 9
6/24 (20/80) 6/60 (20/200) 6/30 (20/100) 6/60 (20/200) 6/21 (20/70) 6/15 (20/50) Counting fingers Counting fingers Counting fingers
Best Recovered Visual Acuity 6/6 (20/20) 6/6 (20/20) 6/6 (20/20) 6/9 (20/30) 6/9 (20/30) 6/6 (20/20) 6/15 (20/50) 6/30 (20/100) 6/30 (20/100)
Time To Maximum Recovery (mos.) 4-9 2 4-6 12 3 6 22 3
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AMERICAN JOURNAL OF OPHTHALMOLOGY TABLE 2 FINAL VISUAL ACUITIES IN ALL 17 EYES OF THE STUDY
Visual Acuity
No. of Eyes
6/6 (20/20) 6/9 (20/30) 6/12 (20/40) 6/15 (20/50) 6/21 (20/70) 6/30 (20/100) 6/60 (20/200) Counting fingers Hand movements
6 1 1 2 1 2 1 1 2
reduction of visual acuity was not neces sarily permanent, and in our series it was common to recover some or all central vision. Involvement of a given area by this disease does not necessarily result in a permanent absolute scotoma, which was confirmed by our findings on quantitative perimetry and sequential Amsler grids. Goldmann perimetry was performed on six of the nine patients. Scotomas corre sponding to the fundus lesions were pre sent in all the patients tested; however, the scotomas were not absolute and usu ally showed a dense center with a lighter surrounding area. Two patients adminis tered Amsler grid fields to themselves on a daily basis on eyes with recurrent activi ty near the fovea. Both reported on several occasions that early in the course of an acute lesion, there was an absolute scoto ma, but over a period of weeks, the scoto mas became less dense and vision im proved in those areas. Seven of the nine patients received corticosteroids in variable amounts and by various routes, but the course of the disease was not noticeably altered by this treatment. Electroretinography and electro-oculography were performed on eight of the nine patients, and both tests were recorded within normal limits for all eyes. Although the characteristic appearance of serpiginous choroidopathy in its acute
FEBRUARY, 1979
and chronic stages was noted in all of our patients, there were several interesting and unusual variations. Inflammatory signs, such as cells in the vitreous or aqueous, are known to be uncommon or at most, of minimal proportions in this disease. Most of our patients had a sud den onset of a "blind spot" in their vision in the absence of any pain, redness, or photophobia. One patient developed a fairly extensive vitritis with 2 to 3+ cells, and another developed a mild vitritis along with perivascular sheathing and intraretinal hemorrhages in the far pe riphery of one eye, which had extensive activity in the posterior pole. The disease usually begins in the pos terior pole, often adjacent to the disk, and extends centrifugally. However, in one of our patients with a classical serpiginous scar in the posterior pole of one eye, the second eye had a normal posterior pole by ophthalmoscopy and fluorescein angiography. In the nasal periphery, between the equator and pars plana, there was a small circumferential scar at one end of which a typical acute lesion was found with gray-green discoloration of the pig ment epithelium, which was densely hypofluorescent early in the fluorescein angiogram and diffusely hyperfluorescent in the late phase. One patient, again with the characteris tic fundus appearance, who had experi enced multiple typical recurrences over many years, developed a recurrence in which the new lesions were multiple, small, isolated round white plaques typi cal of those seen in acute posterior multifocal placoid pigment epitheliopathy. 5,6 These isolated lesions coalesced and left a typical serpiginous scar with loss of the retinal pigment epithelium and choroid. Detailed medical and social histories were obtained from all patients in our series. No patient reported any systemic illness or symptoms before or during the onset of disease or during any of the acute recurrences. Two patients had known tu-
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berculin sensitivity; two had mild hyper tension; one was diabetic; one had glau coma; and one had maxillary sinusitis. DISCUSSION
The earliest description of serpiginous choroidopathy was mady by Junius 7 who called it parapapillary and central retinochoroiditis. Sorsby 8 described and il lustrated what appeared to be several typ ical cases of serpiginous choroidopathy but classified them as a peripapillary type of choroidal sclerosis. Franceschetti 9 pointed out there were two groups of diseases that had similar appearance but which had different clinical courses. One group, pigmented paravenous chorioretinal degeneration, was relatively stationary and probably degenerative. Peripapillary choroidal sclerosis would presumably also fit into this group. Franceschetti's second category was called helicoid peripapillary chorioretinal degeneration, and was described as a progressive disease that represents what we now call serpiginous choroidopathy. The clinical features and fluorescein angiographic findings in serpiginous choroidopathy have been well described by Schatz, Maumenee, and Patz, 3 Hamil ton and Bird, 2 and Laatikainen and Erkkila. 1 In the inactive stage, serpigi nous irregular scars are seen, which rep resent loss of the retinal pigment epitheli um and choriocapillaris associated with varying degrees of hyperpigmentation and fibrous tissue proliferation. Since the disease often begins in the peripapillary area, the scars often interface with the disk and pursue an irregular course. Large choroidal vessels in the base of the scars frequently are the one visible remnant of the choroid. In the early fluorescein angiogram, these inactive scars are hypofluorescent because most of the chorio capillaris is absent. As the angiogram proceeds, an increasing hyperfluorescence is seen at the margins of the scar as fluorescein diffuses into the scarred area
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from the bordering normal choriocapil laris. In the late angiogram, the scars themselves are diffusely hyperfluorescent as the dye stains the sclera and fibrous tissue. In the active stage, a gray or creamcolored opacification develops beneath the retina at the level of the retinal pig ment epithelium. Often, the active area is adjacent to an old scar. There is no serous elevation of the overlying sensory retina and other inflammatory signs are rare. The acute lesion usually lasts several weeks, eventually becomes lighter in col or, and over the subsequent months, shows pigment dispersion and takes on the appearance of the other scarred areas with atrophy of the retinal pigment epi thelium and choriocapillaris, and variable fibrous tissue and pigment proliferation. The fluorescein angiographic appear ance of the acute lesion is characteristic. The active lesion is initially densely hypofluorescent and remains so until late in the angiogram when it becomes variably hyperfluorescent. This angiographic ap pearance could represent either blockage of underlying fluorescence because of opacification of the pigment epithelium or primary closure or nonperfusion of the choriocapillaries. We believe the result ing scar, which indicates loss of both the pigment epithelium and choriocapillaris, points to the choriocapillaris as the loca tion of the initial pathologic process. Chisholm, Gass, and Hutton 4 stated that the progress of serpiginous choroido pathy is usually centrifugal, beginning at the disk and extending outward. How ever, three eyes in our series showed centripetal progression, beginning in an extrapapillary location and progressing toward the optic disk. Patients whose fovea has been bypassed are not safe from future foveal involvement. One eye in our series lost central vision through such centripetal progression. The possibility of visual recovery after foveal or parafoveal involvement has not
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been emphasized previously. Laatikainen and Erkkila 1 recorded visual recovery in six of the 18 eyes in their series. Their average duration to recovered vision was five months. Hamilton and Bird 2 reported a visual improvement from 6/60 (20/200) to 6/9 (20/30) in one eye of the five pa tients they described, but did not other wise provide follow-up data on vision. Schatz, Maumenee, and Patz 3 did not find visual recovery in any of the nine patients in their series, but their follow-up was limited. The patients in our series were exam ined closely and regularly, and we found that visual recovery was not uncommon. In nine eyes with foveal or parafoveal involvement in which visual acuity dropped from 6/15 (20/50) to counting fingers, variable visual recovery occurred. The most dramatic example was in our one-eyed patient in whom vision dropped to counting fingers and recovered to 6/15 (20/50). Recovery of vision generally oc curred slowly over a period of months (Table 1) and was more likely to occur in eyes in which the edge of the active lesion involved the fovea. Our data support this observation. Laatikainen and Erkkila 1 found a high incidence of active or presumed tubercu losis in their patients and treated most of them with aggressive antituberculous therapy with poor results. The patients in Hamilton and Bird's 2 series all had normal general medical evaluations. Schatz, Maumenee, and Patz 3 reported that one of their patients had influenza and a second had sinusitis, but otherwise their medical histories were unremark able. In our series, two patients had tu berculin sensitivity without evidence of active tuberculosis; one was diabetic and one had glaucoma. SUMMARY
We closely observed nine patients (17 eyes) with serpiginous choroidopathy for time periods ranging from two to ten
FEBRUARY, 1979
years. There were seven men and two women with ages ranging from 22 to 58 years, average age was 46 years. Eight patients were bilaterally affected; one pa tient had only one eye. The clinical course of the disease was characterized by multiple recurrences. The recurrences and progression of the disease often were not noticed by patients if the macular area was not involved. Serial fundus photo graphs proved to be invaluable in estab lishing progression. Although the disease usually progresses in a centrifugal man ner from the disk toward the periphery, three patients demonstrated centripetal progression and one of these three perma nently lost central acuity. Visual acuity was affected only when the foveal or parafoveal areas became involved, but in nine of the 17 eyes, visual acuity returned to a variable degree. None of the patients had any general systemic illness. Howev er, five of the patients reported chronic exposure to an unusual variety of chemi cals. REFERENCES 1. Laatikainen, L., Erkkila, H.: Serpiginous choroiditis. Br. J. Ophthalmol. 58:777, 1974. 2. Hamilton, A. M., and Bird, A. C : Geographical choroidopathy. Br. J. Ophthalmol. 58:784, 1974. 3. Schatz, H., Maumenee, A. E., and Patz, A.: Geographic helicoid peripapillary choroidopathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 78:747, 1974. 4. Chisholm, I. H„ Gass, J. D. M., and Hutton, W. L.: The late state of serpiginous (geographic) choroiditis. Am. J. Ophthalmol. 82:343, 1976. 5. Gass, J. D. M.: Acute posterior multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 80: 177, 1968. 6. Annesley, W. H., Tomer, T. L., and Shields, J. A.: Multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 76:511, 1973. 7. Junius, P.: Seltene augenspiegelbilder zum klinischen phanomen der retinitis exsudativa coats und der retino-choroiditis "parapapillaris." Arch. Augenheilkd. 106:475, 1932. 8. Sorsby, A.: Choroidal angio-sclerosis with spe cial reference to its hereditary character. Br. J. Ophthalmol. 23:433, 1939. 9. Franceschetti, A.: A curious affection of the fundus oculi. Helicoid peripapillary chorioretinal degeneration. Its relation to pigmentary paravenous chorioretinal degeneration. Doc. Ophthalmol. 16:81, 1962.
M.D.
Michigan
W I L L I A M H. A N N E S L E Y , J R . , M.D., J E R R Y A. S H I E L D S , AND T E R R A N C E T O M E R , B.A. Philadelphia,
M.D.,
Pennsylvania
AND KENNETH CHRISTOPHERSON, Detroit,
Serpiginous choroidopathy is a pro gressive condition that primarily affects the choriocapillaris and retinal pigment epithelium and often leads to profound visual loss if the foveal or parafoveal areas are involved. It is almost always bilateral and thus far has been described only in whites, although we have seen two cases in blacks. The cause remains obscure. The clinical appearance and fluorescein angiographic findings in serpig inous choroidopathy have been well de scribed. 1 - 3 The long-term follow-up and clinical course of this progressive disease, however, have not been well docu mented, particularly with regard to the prognosis for central vision. Chisholm, Gass, and Hutton 4 provided the only available information in their series of 40 eyes observed from single visits to nine years. A significant reduction in visual acuity was seen in only three eyes of their series, although many started out with poor vision. We report herein the clinical course of this disease, particu larly with regard to visual acuity. From the Kresge Eye Institute, Wayne State University, Detroit, Michigan (Dr. Weiss and Mr. Christopherson); and the Retina Service, Wills Eye Hospital, Philadelphia, Pennsylvania (Drs. Annesley and Shields, and Mr. Tomer). This study was supported in part by the Retina Research and Development Foundation, Philadelphia, and the Lions Club of Pennsylvania. Dr. Weiss was a Heed Foundation Fellow (1975-1976). Reprint requests to Harold Weiss, M.D., Kresge Eye Institute, 3994 John R St., Detroit, Ml 48201.
B.S.
Michigan S U B J E C T S AND M E T H O D S
Of the nine patients in our study, seven were men and two were women. Their average age was 46 years with a range of 22 to 58 years. All the patients were bilaterally affected except for one patient who lost an eye in childhood because of trauma, so that there was a total of 17 eyes in our study. The period of follow-up ranged from two to ten years. We reviewed all patients with serpigi nous choroidopathy seen here who had a minimum of two years follow-up. Nine patients met this criteria. All patients had multiple fundus photographs and fluorescein angiograms, and had been seen by us on multiple occasions for clinical ex amination. Eight out of nine patients were recalled, and detailed ocular, sys temic, and social histories were taken together with complete ocular exami nation, indirect ophthalmoscopy, and slit-lamp biomicroscopy with a fundus contact lens. Fundus photographs and fluorescein angiograms were taken. Electroretinography and electro-oculography as well as visual fields were performed. CASE REPORTS Case 1—A healthy 48-year-old white man noticed painless decreased vision in his right eye in Septem ber 1966. His past medical and family history were unremarkable. The patient had worked in a plastic plant since 1959, and had been exposed constantly to asbestos, phenol, phenole resins, furfuryl, furene resins, paraformaldehyde, hydrochloric acid, for maldehyde, epoxy resins, silica fibers, polypropyl ene, polyethylene, and polycarbonate.
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On examination Sept. 9, 1966, best corrected visu al acuity was R.E.: 6/24 (20/80) and L.E.: 6/6 (20/20). External and slit-lamp exam inations were normal. The right fundus showed a gray discoloration of the pigment epitheli um on the nasal edge of the fovea, and the left fundus was normal. Visual acuity in the right eye gradually improved over the next six months to 6/6 (20/20). At the onset of his visual loss, the patient was given oral prednisone, 40 mg/day for three months, after which the dosage was gradually ta pered and discontinued. Four years later visual acuity in his left eye decreased to 6/60 (20/200). Examination revealed a typical active area of a gray discoloration of the pigment epithelium. In contrast to the right eye, the
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active area involved the whole fovea, rather than just its margin. Visual acuity in the left eye remained at 6/60 (20/200) for seven years. Since May 1970, the patient had experienced no change in vision and although his visual acuity remained stable at 6/6 (20/20) in the right eye and 6/60 (20/200) in the left eye, his disease continued to progress (Fig. 1). The asymptomatic progression of the disease was not recognized until the serial fun dus photographs were reviewed, which showed cen tripetal progression from the fovea toward the disk. The disease was characterized by periods of activ ity alternating with periods of quiescence. In the active stage, a localized area of gray-green or creamcolored opacification developed beneath the retina at the level of the retinal pigment epithelium. The
Fig. 1 (Weiss and associates). Case 1. Top left, Appearance of the posterior pole of the right eye in May 1970. The fovea has been spared and visual acuity was 6/6 (20/20). Top right, Although asymp tomatic, the patient was seen again in December 1971. Visual acuity remains 6/6 (20/20). Note the extension of the scar toward the disk (arrows). Bot tom left, Five years later, the patient stated his right eye had not changed. However, the scars had en larged by careful comparison with the photographs taken five years earlier (arrows).
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area of activity was often, but not always, adjacent to an old choroidal scar. Over a period of weeks, the area of new activity showed mottled pigmentation and eventually atrophy of the retinal pigment epi thelium and choriocapillaris resulted. There were no instances of serious elevation of the retina over these active areas and no evidence of subretinal neovascular membranes was found. Case 2—A healthy 22-year-old white man was first seen in April 1974, with a three-week history of blurred vision in his right eye and a one-week history of blurred vision in his left eye. His occupa tional history revealed exposure to various types of paint, diesel fuel, and domestic animals. On examination April 9, 1974, best corrected visual acuity was R.E.: 6/12 (20/40) and L.E.: 6/6 (20/20). External and slit-lamp examination were normal. The fundi of both eyes showed a large area of pigment alteration below which was a broad active front of gray discoloration of the pigment epthelium (Fig. 2, top left). Similar areas were present around the disk (Fig. 2, top right). In the equatorial area of the right eye at the 12 o'clock position, there was perivenous sheathing with some nearby intraretinal hemorrhages (Fig. 2, middle
left).
Three days later, the visual acuity in the right eye had deteriorated to 6/60 (20/200) while the visual acuity in the left eye had remained 6/6 (20/20). Fundus examination showed that the active front had extended down to involve the fovea of the right eye and was moving closer to the fovea of the left eye. The medical and neurologic examinations were normal as were an extensive battery of x-ray and laboratory studies including skin test for coccidiomycosis, histoplasmosis, tuberculosis; sedimenta tion rate, FTA-abs, Anti-nuclear antibody, and lupus erythematosis preparation. Electroretinography and electro-oculography were normal. Eleven days later, visual acuity in the right eye remained 6/60 (20/200) and in the left eye had dropped to 6/15 (20/50). Fundus examination showed that the active fronts had continued to progress inferiorly in both eyes across the fovea (Fig. 2, middle right). Pluorescein angiography was performed (Fig. 2, bottom). Visual acuity in the left eye dropped to 6/30 (20/100), but one month later visual acuity had improved to 6/24 (20/80) in the right eye and 6/21 (20/70) in the left eye. The lesions had extended to the inferior arcades leaving extensive pigment alter ation across the entire posterior pole. Two months after initial examination, visual acu ity had improved to R.E.: 6/6 (20/20) and L.E.: 6/21 (20/70). Four months later the visual acuity in the left eye had improved to 6/18 (20/60) and by six months had returned to 6/6 (20/20). When re-examined 2V2 years later the- patient stated that there had been no change in vision since the initial course of his disease. Comparison of fundus photographs, however, showed extension of scarring along the superior arcades and around the
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disk in the left eye, marked pigment clumping, and several areas of subretinal fibrous proliferation. This patient is remarkable in the symmetry of his disease, his visual recovery, and the perivascular sheathing. The visual recovery possibly can be at tributed to a failure of the disease to destroy the choriocapillaries in the fovea. Case 3—A 44-year-old white man developed a spot on his right eye in July 1970. At the time, the patient was working in a sheet metal shop and was exposed to various fumes of unknown type. Since 1958, he had performed greenhouse work and was exposed to vapone, various kinds of fertilizer, malathrone, Triban, and nicotine bombs. When first seen on Sept. 16,1970, his visual acuity was 6/6 (20/20) in both eyes. External and slit-lamp examinations were normal. Fundus examination re vealed multiple serpiginous and isolated choroidal scars in both eyes. A large scar extending from the disk to just above the fovea in the right eye appeared with modest pigment mottling. In October 1970, the patient had a systemic exam ination. Significant findings were a positive tubercu lin skin test (second strength), a dental abscess, and mild chronic maxillary sinusitis. He was given isoniazid, 300 mg daily, and pyridoxine HC1, 50 mg daily. The patient was examined regularly; in July 1974 an active lesion developed in the left macular area, nasal to an older choroidal scar (Fig. 3, top left). Visual acuity in the left eye was reduced to counting fingers. No new activity in the right eye was noted, and the patient stated that there had been no change in his vision. However, on careful review of the fundus photographs, extension of the round scar below the fovea had occurred in a centripetal man ner toward the disk. The patient's left eye was treated with a retrobulbar injection of 40 mg of methylprednisolone acetate (Depomedrol). Six weeks later, visual acuity had improved to 6/60 (20/200) in the left eye and the area of previous activity showed pigment mottling (Fig. 3, top right). Additionally, several isolated round areas of fresh activity were present along the superi or arcades. In March 1975, visual acuity in the left eye had decreased to counting fingers. Fundus examination revealed a resolving lesion with pigment mottling involving the foveal area. The right eye was un changed. In June 1976, visual acuity was counting fingers in the left eye and 6/15 (20/50) in the right eye. In the left eye, there was further extension of the scarring, which involved a broad area of the posterior pole bounded by the vascular arcades (Fig. 3, bottom left). No new activity was present at this time. The electroretinogram and electrooculogram were both normal. Goldmann perimetry of the right eye showed scotomas of variable depth corresponding to the fundus lesions (Fig. 3, bottom right). The patient showed asymptomatic progres sion, centripetal progression, and temporary visual recovery. Case 4—A healthy 46-year-old white man devel-
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-< Fig. 2 (Weiss and associates). Case 2. Top left, Posterior pole of the right eye on April 9, 1974, showing a broad active front (arrows) extending down across the posterior pole, leaving extensive pigmentary changes in its wake. Top right, Simultaneous activity was also present around both disks. Middle left, Equatorial area of the right fundus showing focal and diffuse areas of perivenous sheathing. Middle right, Eleven days later, the active front extended inferiorly (arrows). Bottom, Fluorescein angiography of the right eye in middle right. The active front is densely hypofluorescent in the early phase (arrows) (bottom left); and diffusely hyperfiuorescent in the late phase (arrows) (bottom right). The older areas of involvement near the superior vascular arcades show extensive pigment changes with mottled hyperfluorescence.
oped a sudden onset of reduced visual acuity in the left eye in 1970, which continued to deteriorate until 1974. Visual acuity remained good in his right eye until January 1974, when he suddenly noted blurring of vision. The patient was a race horse trainer
and was exposed to a variety of liniments as well as dimethyl-sulfoxide. On examination Jan. 14, 1974, best-corrected visual acuity was 6/15 (20/50) in the right eye and counting fingers in the left eye. External and slit-
Fig. 3 (Weiss and associates). Case 3. Top left, Fundus photograph of the left eye in July 1974, showing the area of fresh activity (arrows) involving the fovea and demonstrating a grayish opacification at the level of the pigment epithelium. Top right, Six weeks later, there was pigment mottling where the active lesion had been (arrows). Bottom left, Fifteen months later, there was extensive scarring now involving most of the posterior pole.
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lamp examinations were normal. In the posterior pole of the left eye was a large serpiginous area of loss of the pigment epithelium, choriocapillaris, and inner choroid (Fig. 4, top left) without any new activity. In the right eye, an active area of gray opacification at the level of the pigment epithelium was present at the inferior margin of the fovea. Several small choroidal scars were present nasal to the fovea (Fig. 4, top right). The patient was treated with oral prednisone, 40 mg daily, for six months and over this period of time visual acuity in the right eye gradually improved from 6/15 (20/50) to 6/6 (20/20). In June 1976, the patient stated that his vision had not changed over the past two years. However, the appearance of the fundus in both eyes had changed.
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In the left eye, the large serpiginous scar had extend ed in a centrifugal manner temporally and along the superior arcades. In the right eye, additional scars were present superior to the macula and the area of new activity in 1974 showed pigment dispersion and atrophy of the inner choroid (Fig. 4, bottom). The patient showed both visual recovery following parafoveal involvement and asymptomatic progres sion in the right eye. RESULTS
The clinical course of the disease in all of the patients was characterized by mul tiple recurrences with the development of
Fig. 4 (Weiss and associates). Case 4. Top, Fundus appearance of the right and left eyes in January 1974. The active area in the right eye is indicated by arrows. Bottom left, Two years later, additional scarring was present superioral to the fovea and the area of activity seen two years previously be neath the fovea demonstrated pigment changes and loss of choriocapillaris.
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new scars and extension of old scars. There was often a discrepancy between the patients' histories regarding progres sion of their disease and the findings on fundus examination. Five of the patients were unaware that their disease had con tinued to progress because their recur rences were not near the macula and were, therefore, not recognized. Progres sion might not have been detected by the examiners if serial fundus photographs had not been available. Most of the pa tients had multiple and complex chorioretinal scars and new areas of activity or scarring were sometimes subtle and de tectable only by close scrutiny of serial photographs. Serial fundus photography provided us with the information that the disease may progress in the absence of symptoms and that progression can con tinue for up to ten years, the maximum duration of our follow-up. Most of the eyes in our series showed progression of the disease in a centrifugal manner, beginning near the disk and ex tending outward toward the periphery. However, in three eyes in our series, there was either centripetal progression or new activity in an area that had been previous ly bypassed. In one of these cases, visual acuity was permanently affected. Visual acuity was affected only when the foveal or parafoveal areas were direct
ly involved by the disease. In nine of the 17 eyes, visual acuity dropped to a varia ble degree during foveal or parafoveal involvement, but subsequently improved as the acute lesion resolved (Table 1). In some cases, visual recovery was re markable despite significant initial reduc tion in vision. In another six of the 17 eyes in our series, the fovea or parafoveal areas were affected and visual acuity dropped but did not recover. Final visual acuities were 6/12 (20/40) (one eye); 6/15 (20/50) (one eye); 6/30 (20/100) (one eye); 6/60 (20/ 200) (one eye); hand movements (two eyes). In the remaining two of the 17 eyes, the foveal and parafoveal areas were not involved at all and visual acuity was unaffected. At the conclusion of our study, no pa tient was legally blind and only three of the 17 eyes had visual acuity of less than 6/60 (20/200) (two had hand movements as reported above, and one eye that recov ered visual acuity to 6/30 (20/100) subse quently developed foveal involvement again and became counting fingers). Final visual acuity in these 17 eyes is shown in Table 2. Foveal or parafoveal involvement with its attendant reduction in visual acuity is common in serpiginous choroidopathy and occurred in 15 of the 17 eyes. The
TABLE 1 PATIENTS WHO DEMONSTRATED RECOVERY OF VISUAL ACUITY AFTER FOVEAL OR PARAFOVEAL INVOLVEMENT
Patient No.
Worst Visual Acuity
1 2 3 4 5 6 7 8 9
6/24 (20/80) 6/60 (20/200) 6/30 (20/100) 6/60 (20/200) 6/21 (20/70) 6/15 (20/50) Counting fingers Counting fingers Counting fingers
Best Recovered Visual Acuity 6/6 (20/20) 6/6 (20/20) 6/6 (20/20) 6/9 (20/30) 6/9 (20/30) 6/6 (20/20) 6/15 (20/50) 6/30 (20/100) 6/30 (20/100)
Time To Maximum Recovery (mos.) 4-9 2 4-6 12 3 6 22 3
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Visual Acuity
No. of Eyes
6/6 (20/20) 6/9 (20/30) 6/12 (20/40) 6/15 (20/50) 6/21 (20/70) 6/30 (20/100) 6/60 (20/200) Counting fingers Hand movements
6 1 1 2 1 2 1 1 2
reduction of visual acuity was not neces sarily permanent, and in our series it was common to recover some or all central vision. Involvement of a given area by this disease does not necessarily result in a permanent absolute scotoma, which was confirmed by our findings on quantitative perimetry and sequential Amsler grids. Goldmann perimetry was performed on six of the nine patients. Scotomas corre sponding to the fundus lesions were pre sent in all the patients tested; however, the scotomas were not absolute and usu ally showed a dense center with a lighter surrounding area. Two patients adminis tered Amsler grid fields to themselves on a daily basis on eyes with recurrent activi ty near the fovea. Both reported on several occasions that early in the course of an acute lesion, there was an absolute scoto ma, but over a period of weeks, the scoto mas became less dense and vision im proved in those areas. Seven of the nine patients received corticosteroids in variable amounts and by various routes, but the course of the disease was not noticeably altered by this treatment. Electroretinography and electro-oculography were performed on eight of the nine patients, and both tests were recorded within normal limits for all eyes. Although the characteristic appearance of serpiginous choroidopathy in its acute
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and chronic stages was noted in all of our patients, there were several interesting and unusual variations. Inflammatory signs, such as cells in the vitreous or aqueous, are known to be uncommon or at most, of minimal proportions in this disease. Most of our patients had a sud den onset of a "blind spot" in their vision in the absence of any pain, redness, or photophobia. One patient developed a fairly extensive vitritis with 2 to 3+ cells, and another developed a mild vitritis along with perivascular sheathing and intraretinal hemorrhages in the far pe riphery of one eye, which had extensive activity in the posterior pole. The disease usually begins in the pos terior pole, often adjacent to the disk, and extends centrifugally. However, in one of our patients with a classical serpiginous scar in the posterior pole of one eye, the second eye had a normal posterior pole by ophthalmoscopy and fluorescein angiography. In the nasal periphery, between the equator and pars plana, there was a small circumferential scar at one end of which a typical acute lesion was found with gray-green discoloration of the pig ment epithelium, which was densely hypofluorescent early in the fluorescein angiogram and diffusely hyperfluorescent in the late phase. One patient, again with the characteris tic fundus appearance, who had experi enced multiple typical recurrences over many years, developed a recurrence in which the new lesions were multiple, small, isolated round white plaques typi cal of those seen in acute posterior multifocal placoid pigment epitheliopathy. 5,6 These isolated lesions coalesced and left a typical serpiginous scar with loss of the retinal pigment epithelium and choroid. Detailed medical and social histories were obtained from all patients in our series. No patient reported any systemic illness or symptoms before or during the onset of disease or during any of the acute recurrences. Two patients had known tu-
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berculin sensitivity; two had mild hyper tension; one was diabetic; one had glau coma; and one had maxillary sinusitis. DISCUSSION
The earliest description of serpiginous choroidopathy was mady by Junius 7 who called it parapapillary and central retinochoroiditis. Sorsby 8 described and il lustrated what appeared to be several typ ical cases of serpiginous choroidopathy but classified them as a peripapillary type of choroidal sclerosis. Franceschetti 9 pointed out there were two groups of diseases that had similar appearance but which had different clinical courses. One group, pigmented paravenous chorioretinal degeneration, was relatively stationary and probably degenerative. Peripapillary choroidal sclerosis would presumably also fit into this group. Franceschetti's second category was called helicoid peripapillary chorioretinal degeneration, and was described as a progressive disease that represents what we now call serpiginous choroidopathy. The clinical features and fluorescein angiographic findings in serpiginous choroidopathy have been well described by Schatz, Maumenee, and Patz, 3 Hamil ton and Bird, 2 and Laatikainen and Erkkila. 1 In the inactive stage, serpigi nous irregular scars are seen, which rep resent loss of the retinal pigment epitheli um and choriocapillaris associated with varying degrees of hyperpigmentation and fibrous tissue proliferation. Since the disease often begins in the peripapillary area, the scars often interface with the disk and pursue an irregular course. Large choroidal vessels in the base of the scars frequently are the one visible remnant of the choroid. In the early fluorescein angiogram, these inactive scars are hypofluorescent because most of the chorio capillaris is absent. As the angiogram proceeds, an increasing hyperfluorescence is seen at the margins of the scar as fluorescein diffuses into the scarred area
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from the bordering normal choriocapil laris. In the late angiogram, the scars themselves are diffusely hyperfluorescent as the dye stains the sclera and fibrous tissue. In the active stage, a gray or creamcolored opacification develops beneath the retina at the level of the retinal pig ment epithelium. Often, the active area is adjacent to an old scar. There is no serous elevation of the overlying sensory retina and other inflammatory signs are rare. The acute lesion usually lasts several weeks, eventually becomes lighter in col or, and over the subsequent months, shows pigment dispersion and takes on the appearance of the other scarred areas with atrophy of the retinal pigment epi thelium and choriocapillaris, and variable fibrous tissue and pigment proliferation. The fluorescein angiographic appear ance of the acute lesion is characteristic. The active lesion is initially densely hypofluorescent and remains so until late in the angiogram when it becomes variably hyperfluorescent. This angiographic ap pearance could represent either blockage of underlying fluorescence because of opacification of the pigment epithelium or primary closure or nonperfusion of the choriocapillaries. We believe the result ing scar, which indicates loss of both the pigment epithelium and choriocapillaris, points to the choriocapillaris as the loca tion of the initial pathologic process. Chisholm, Gass, and Hutton 4 stated that the progress of serpiginous choroido pathy is usually centrifugal, beginning at the disk and extending outward. How ever, three eyes in our series showed centripetal progression, beginning in an extrapapillary location and progressing toward the optic disk. Patients whose fovea has been bypassed are not safe from future foveal involvement. One eye in our series lost central vision through such centripetal progression. The possibility of visual recovery after foveal or parafoveal involvement has not
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been emphasized previously. Laatikainen and Erkkila 1 recorded visual recovery in six of the 18 eyes in their series. Their average duration to recovered vision was five months. Hamilton and Bird 2 reported a visual improvement from 6/60 (20/200) to 6/9 (20/30) in one eye of the five pa tients they described, but did not other wise provide follow-up data on vision. Schatz, Maumenee, and Patz 3 did not find visual recovery in any of the nine patients in their series, but their follow-up was limited. The patients in our series were exam ined closely and regularly, and we found that visual recovery was not uncommon. In nine eyes with foveal or parafoveal involvement in which visual acuity dropped from 6/15 (20/50) to counting fingers, variable visual recovery occurred. The most dramatic example was in our one-eyed patient in whom vision dropped to counting fingers and recovered to 6/15 (20/50). Recovery of vision generally oc curred slowly over a period of months (Table 1) and was more likely to occur in eyes in which the edge of the active lesion involved the fovea. Our data support this observation. Laatikainen and Erkkila 1 found a high incidence of active or presumed tubercu losis in their patients and treated most of them with aggressive antituberculous therapy with poor results. The patients in Hamilton and Bird's 2 series all had normal general medical evaluations. Schatz, Maumenee, and Patz 3 reported that one of their patients had influenza and a second had sinusitis, but otherwise their medical histories were unremark able. In our series, two patients had tu berculin sensitivity without evidence of active tuberculosis; one was diabetic and one had glaucoma. SUMMARY
We closely observed nine patients (17 eyes) with serpiginous choroidopathy for time periods ranging from two to ten
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years. There were seven men and two women with ages ranging from 22 to 58 years, average age was 46 years. Eight patients were bilaterally affected; one pa tient had only one eye. The clinical course of the disease was characterized by multiple recurrences. The recurrences and progression of the disease often were not noticed by patients if the macular area was not involved. Serial fundus photo graphs proved to be invaluable in estab lishing progression. Although the disease usually progresses in a centrifugal man ner from the disk toward the periphery, three patients demonstrated centripetal progression and one of these three perma nently lost central acuity. Visual acuity was affected only when the foveal or parafoveal areas became involved, but in nine of the 17 eyes, visual acuity returned to a variable degree. None of the patients had any general systemic illness. Howev er, five of the patients reported chronic exposure to an unusual variety of chemi cals. REFERENCES 1. Laatikainen, L., Erkkila, H.: Serpiginous choroiditis. Br. J. Ophthalmol. 58:777, 1974. 2. Hamilton, A. M., and Bird, A. C : Geographical choroidopathy. Br. J. Ophthalmol. 58:784, 1974. 3. Schatz, H., Maumenee, A. E., and Patz, A.: Geographic helicoid peripapillary choroidopathy. Trans. Am. Acad. Ophthalmol. Otolaryngol. 78:747, 1974. 4. Chisholm, I. H„ Gass, J. D. M., and Hutton, W. L.: The late state of serpiginous (geographic) choroiditis. Am. J. Ophthalmol. 82:343, 1976. 5. Gass, J. D. M.: Acute posterior multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 80: 177, 1968. 6. Annesley, W. H., Tomer, T. L., and Shields, J. A.: Multifocal placoid pigment epitheliopathy. Arch. Ophthalmol. 76:511, 1973. 7. Junius, P.: Seltene augenspiegelbilder zum klinischen phanomen der retinitis exsudativa coats und der retino-choroiditis "parapapillaris." Arch. Augenheilkd. 106:475, 1932. 8. Sorsby, A.: Choroidal angio-sclerosis with spe cial reference to its hereditary character. Br. J. Ophthalmol. 23:433, 1939. 9. Franceschetti, A.: A curious affection of the fundus oculi. Helicoid peripapillary chorioretinal degeneration. Its relation to pigmentary paravenous chorioretinal degeneration. Doc. Ophthalmol. 16:81, 1962.
