COMPLICATIONS O F C H R O N I C CYCLITIS R O N A L D E. S M I T H , M.D.,
W I L L I A M A. G O D F R E Y ,
AND S A M U E L J. KIMURA, San Francisco,
M.D.,
M.D.
California
Recently, we described the clinical fea tures, course, and visual prognosis of chronic cyclitis 1 (variously termed pars planitis, 2 peripheral uveitis, 3 and vitritis 4 ). The 100 patients described in that report 1 had a variety of ocular complica tions. We document these complications in the present study.
ocular examination by at least two of us (the final examination). Details of the ex amination techniques, grading systems, and classification of disease into mild, moderate, and severe categories on the basis of vitreous reaction were recorded previously. 1
M A T E R I A L AND M E T H O D S
There were 44 females and 56 males; their ages at onset ranged between 4 and 58 years (median and arithmetic mean of 23 years); 99 patients were white, and one was black. The interval from onset of symptoms to the final examination here ranged from four to more than 20 years with a median and mean of 10.5 years. Most cases were bilateral, yielding 182 affected eyes. The disease in each eye was classified either as severe, moderately severe, or mildly se vere. In 76 eyes, cataracts were usually locat ed posterior or anterior subcapsularly, or both, or posterior cortically (Table 1).
The methods used in this study were recorded previously. 1 Briefly, cases with the diagnosis of chronic cyclitis were retrieved from the Uveitis Survey files here. All cases had a minimum follow-up of four years from the onset of the illness and all patients met the following criteria: (1) gradual onset in one or both eyes with blurred vision or floaters as the initial symptom; (2) absent or minimal photo phobia; (3) minimal anterior chamber reaction; and (4) presence of vitreous opacities. Exudate over the pars plana-ora serrata region inferiorly, a unique feature of this disease, was often present, espe cially in more severe disease. However, since earlier clinical examinations did not always include scierai depression, the presence of exudate was not used as a cri terion. The first 100 patients available for follow-up underwent a repeat, complete From the Francis I. Proctor Foundation for Re search in Ophthalmology and the Department of Ophthalmology, University of California, San Fran cisco Medical Center, San Francisco, California. This study was supported in part by research grant 2 Pol EY 00310, National Institutes of Health; Public Health Service grant EY 51582-01 (Dr. Smith); and Public Health Service grant 1 F0 3 EY 51530 (Dr. Godfrey). Reprint requests to Ronald E. Smith, M.D., De partment of Ophthalmology, University of Southern California School of Medicine, LAC-USC Medical Center, 1200 N. State St., Los Angeles, CA 90033.
RESULTS
TABLE 1 C O M P L I C A T I O N S O F C H R O N I C CYCLITIS I N 182 E Y E S *
Complications Cataract Macular degeneration Band keratopathy Glaucoma Retinal detachment Retinoschisis Vitreous hemorrhage Retinal hemorrhage Retinitis pigmentosalike changes Vessels leaving the disk margin
No. of Eyes
Percent of Total
76 51 17 15 10 7 6 5 2
42 28 9 8 5 4 3 3 1
2
1
*More than one complication occurred in some eyes.
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Thirty-four cataracts were noted at the initial examination. Twenty eyes were aphakic at the final examination, having undergone surgery in the interval be tween the first visit and the final examina tion. Several cataracts appeared to be re lated to the long-term use of corticosteroids. Cataracts developed more frequent ly in eyes with more severe or prolonged disease (Table 2). Macular degeneration secondary to chronic cystoid macular edema was ob served in 51 eyes at the final examination. Seventy-eight eyes had macular edema or degeneration, or both, at some time dur ing the course of the disease. The rela tionship between severity of disease, du ration of activity, and macular changes is noted in Table 2. Eleven patients (17 eyes) had band keratopathy at the final examination. Glauco ma was observed in 15 eyes, and was probably related to corticosteroids in 11 eyes. Intraocular pressure increased after cataract extraction in two eyes and was associated with retinal detachment in two other eyes. Only six eyes required medi
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cation for glaucoma at the final examina tion. We noted retinal detachment and retinoschisis in ten and seven eyes, respective ly. Vitreous hemorrhage was the present ing manifestation of the disease in one eye and occurred in five other eyes. No bleeding sites were found despite careful searches of the fundus. Retinal detach ment did not occur in these eyes. Intraretinal hemorrhages were noted in the active stages of the disease in five eyes; there were no sequelae. Pigmentary changes similar to those found in retinitis pigmentosa (Fig. 1) were found in two eyes. In both in stances, the fundus changes developed during the course of the patient's disease and were not present at the initial exami nation. No associated changes in vessel caliber or optic nerve were noted. Retinal vessels leaving the disk margin were also observed (Fig. 2). DISCUSSION
The major causes of visual morbidity in chronic cyclitis at the time of the initial
TABLE 2 INCIDENCE O F CATARACTS, MACULAR DEGENERATIONS, SEVERITY, AND DURATION OF DISEASE IN EYES WITH COMPLICATIONS* Duration of Disease, yrs
Severity of Disease
4-10
Mild Moderate Severe
11-15
Mild Moderate Severe
16-20
Mild Moderate Severe
21 or more
Mild Moderate Severe
*From Smith, Godfrey, and Kimura.1
No. of Eyes None 26 24 50 4 13 16 33 1 1 9 11 0 0 3 3
Macular Degeneration
Cataracts
None 2 ( 8%) 9 (38%)
None 10 (38%) 16 (67%)
2 (50%) 6 (46%) 7 (44%)
3 (75%) 5 (38%) 9 (56%)
1 (100%) 0 4 (44%)
0 1 (100%) 7 (78%)
0 0 1 (33%)
0 0 3 (100%)
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Table 3 Causes of Decreased Visual Acuity of 6/12 (20/40) or less at First and Final Examinations
First examination Final examination
No. of Eyes
Macular Disease
Lens Opacities
Vitreous Opacities
70
42 (60%)
4 ( 6%)
27 (39%)
69
51 (74%)
13 (19%)
6 ( 9%)
loss of the foveal reflex with a wet appear ance of the posterior pole and numerous glistening highlights reflected from the irregularly thickened, edematous retina (Fig. 3, top left). Cystoid macular changes similar to those associated with the Irvine-Gass postcataract syndrome and Fig. 1 (Smith, Godfrey, and Kimura). Bone spicule other forms of severe uveitis frequently pigmentation similar to retinitis pigmentosa was ensue in the later stages of chronic edema. seen in this 20-year-old white man. Fluorescein angiography reveals early leakage from small perifoveal capillaries examination and at the final examination and into adjacent retinal tissue during the here were compiled (Table 3). late stages of the fluorescein pass (Fig. 3, Macular edema and resultant macular top right and bottom). Serous elevations degeneration are frequent and serious 2,5 of the neuroepithelium or pigment epi complications of this syndrome. Of all thelium are rarely encountered. Often, the eyes with visual acuity of 6/12 (20/40) abnormal leakage from multiple areas oc or less, 74% had macular disease. Clini curs throughout the fundus, including the cally, early macular edema appears as a far periphery. 4 , 5 Involvement of the peripapillary retina frequently gives the ap pearance of papilledema. If macular edema persists for several months, chronic macular changes devel op with permanent impairment of central vision to varying degrees. Alterations in the pigment epithelium with dispersion, clumping, and patchy loss of pigment are the clinical findings in such maculae (Fig. 4). No fluorescein leakage was present in these cases, but multiple pigment epithe lium window defects occurred. The mac ular changes in chronic cyclitis are not unique to this syndrome but are rather the final common pathway for macular edema Fig. 2 (Smith, Godfrey, and Kimura). Vessels leaving the disk have been distorted by fractional of any etiology. Heavy vitreous reaction, evidence of effect of the peripheral inflammatory lesions (black spots are artifacts). severity of the disease process, appears to
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Fig. 3 (Smith, Godfrey, and Kimura). Although the macular area in this 19-year-old woman reveals only loss of foveal reflex before fluorescein angiography (top left), the early fluorescein phase (top right) shows dilation and leakage from the perifoveal capillary net (arrows). Later, the more charac teristic pattern of cystoid maculopathy developed (bottom).
predispose to the development of macular edema. However, many eyes with mini mal vitreous reaction developed sympto matic macular edema, demonstrating that the vitreous reaction alone is not a reli able indication for the development of macular disease. Of importance to the clinician manag ing patients with chronic cyclitis is the finding that vitreous opacities were re sponsible for the visual loss in only 9% of all eyes with visual acuity of 6/12 (20/40) or less at the final examination (Table 3). This compares to 74% of visually im paired eyes that had decreased acuity caused by macular changes. Vitreous cells, floaters, and opacities gradually dis-
appear or become less important clinical ly in time, whereas macular edema may lead to chronic, permanent macular changes even when the disease process is inactive. Furthermore, the uniformly good functional results in eyes that did not develop macular complications impli cate macular disease as the most impor tant factor in determining the prognosis. Cataract formation is an inevitable complication of the chronic inflammatory process. 2 · 3 Eyes with increased evidence of vitreous disease and a prolonged course tended to develop more cataracts. However, the role of corticosteroids must not be overlooked. In several cases, the development and progression of lens
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Fig. 4 (Smith, Godfrey, and Kimura). Disruption of smooth pigment epithelial pattern is present in the macular area of this 45-year-old woman with permanent macular dysfunction due to chronic mac ular edema and finally macular degeneration. Arrow points to the foveal area (black line is artifact).
opacities appeared to be related to the use of these drugs. Despite their frequent appearance, most cataracts did not affect visual function. The opacities were con sidered dense enough to have caused vis ual acuity of 6/12 (20/40) or less in only 6% of eyes initially and in 19% of eyes at the final examination (Table 3). Surgery was performed in 20 eyes, usually with out complications and with visual results dependent on macular function. Band keratopathy was clinically indis tinguishable from that classically associ ated with juvenile arthritis and iridocyclitis. Glaucoma is probably not a complica tion of the disease process, but the result of vigorous corticosteroid therapy or a surgical procedure such as cataract ex traction. Corticosteroid therapy was prob ably responsible for increased intraocular pressure in 11 of the 15 affected eyes. Cataract extraction caused glaucoma in two other eyes. Retinal detachment occurred less fre
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quently in our series than in the series (22 to 51%) reported by Brockhurst and asso ciates. 3 · 6 The subspecialty interests of their clinic probably account for the marked difference in the incidence of this complication. The repair and prognosis of such detachments have been well described. 6 Vitreous traction secondary to long-standing vitreous inflammation, band formation, and subsequent retinal hole formation probably account for most retinal detachments. 6 Vitreous hemorrhage in chronic cyclitis has been described as an infrequent com plication 2 and was observed in six eyes in our series. Neovascularization of the inferiorly organized exudate in the ora serrata region has been described as the possible cause of such hemorrhage. Vitre ous hemorrhages in our series cleared with no complications such as detach ments. Retinal vessels leaving the disk margin with displacement of the macula occurred in two of our patients, probably as the result of peripheral vitreous traction bands. Chronic cyclitis must be consid ered along with retrolental fibroplasia, ocular toxacara canis, Coats' disease, and familial exudative vitreoretinopathy in the differential diagnosis of heterotropic macula. Bone spicule pigmentation resembling retinitis pigmentosa has been reported as an unusual complication of chronic cycli tis, 2 and developed in two eyes in our series. The extensive reactions in pigment epithelium in such cases suggest a diffuse inflammatory process as a possible cause of chronic cyclitis. SUMMARY
Follow-up examinations, ranging from four to more than 20 years, were per formed on 100 patients with chronic cy clitis whose ages at onset were from 4 to 58 years. Cataracts were found in 42% of eyes and macular disease secondary to
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macular edema in 28% of eyes. Band keratopathy, glaucoma, retinal detach ment, retinoschisis, vitreous hemorrhage, retinal hemorrhage, and vessels leaving the disk margin were also noted. The complications resulting in de creased vision in chronic cyclitis were macular edema in active cases and macu lar degenerative changes in the late inac tive stages. Of all eyes with final visual acuity of 6/12 (20/40) or less, 74% had permanent, late macular changes secon dary to earlier cystoid macular edema. Vitreous opacities or cells, or both, caused decreased visual acuity in the early ac tive stages of chronic cyclitis but were not major factors in the ultimate visual prognosis in the late inactive stages. At the final examination, vitreous opacities caused a visual loss in only 9% of the eyes that had visual acuity of 6/12 (20/40) or less. It was difficult to determine whether
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corticosteroids caused cataract formation and glaucoma. ACKNOWLEDGMENT
Barry Blum, M.D., permitted us to use Figure 3.
REFERENCES 1. Smith, R. E., Godfrey, W. A., and Kimura, W. J.: Chronic cyclitis. 1. Course and visual prognosis. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77:760, 1973. 2. Welch, R. B., Maumenee, A. E., and Wahlen, H. E.: Peripheral posterior segment inflammation, vitreous opacities, and edema of the posterior pole: pars planitis. Arch. Ophthalmol. 64:540, 1960. 3. Brockhurst, R. J., Schepens, C. L., and Okamura, I. D.: Uvéitis. 2. Peripheral uveitis. Clinical description, complications, and differential diagno sis. Am. J. Ophthalmol. 49:1257, 1960. 4. Gass, J. D. M.: Fluorescein angiography in endogenous intraocular inflammation. In Aronson, S. B. (ed.): Clinical Methods in Uveitis. St. Louis, C. V. Mosby, 1968, p p . 204-209. 5. Maumenee, A. E.: Clinical entities in "uveitis." An approach to the study of intraocular inflamma tion. Am. J. Ophthalmol. 69:1, 1970. 6. Brockhurst, R. J., and Schepens, C. L.: Uveitis. 4. Peripheral uveitis. The complication of retinal detachment. Arch. Ophthalmol. 80:747, 1968.