Clinical characteristics of serpiginous choroidopathy in North India

Share Embed


Descrição do Produto

Clinical Characteristics of Serpiginous Choroidopathy in North India VISHALI GUPTA, MS, ANITA AGARWAL, MS, AMOD GUPTA, MS, P. BAMBERY, MD, AND SUBINA NARANG, MS

● PURPOSE: To describe clinical characteristics of serpig-

inous choroidopathy in the North Indian population. ● DESIGN: Retrospective cohort-based study. ● METHODS: Records of 86 patients who obtained a diagnosis of serpiginous choroidopathy at presentation or after a minimal follow-up of 2 years were retrieved. Group I included 62 patients who were diagnosed as having serpiginous choroidopathy at the initial presentation. Group II included 20 patients who initially presented as having multifocal placoid pigment epitheliopathy resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and had progressed over years to serpiginous choroidopathy, while group III had 4 patients with mixed presentations. Statistical analysis was done using the chi-square and Mann–Whitney U test. A review of the clinical, historical, fundus photographic, and fluorescein angiographic features was performed. The demographic and clinical features, number of recurrences, final visual acuity, and final description of healed lesions were documented. ● RESULTS: The median age at presentation was 30 years in group I, 29 years in group II, and 31 years in group III. The disease was bilateral in five (15%) patients in group I, 12 (60%) in group II, and all four in group III. All the affected eyes in groups II and III and 58 eyes (87%) in group I had recurrences over 2 to 8 years of follow up. Compared with group I, group II was characterized by more bilaterality (P ⴝ .001), less central foveal involvement (P ⴝ .001), better final visual acuity (P ⴝ .001), the presence of healed multifocal scars (P ⴝ .001), and less subretinal fibrosis (P ⴝ .02). Four patients in group III showed mixed features. ● CONCLUSIONS: Serpiginous choroidopathy in our population was seen in young patients and had three distinct Accepted for publication March 12, 2002. From the Departments of Ophthalmology (V.G., A.G., S.N.), Internal Medicine (P.B.), Postgraduate Institute of Medical Education and Research, Chandigarh, India; Department of Ophthalmology and Visual Sciences, Vanderbilt University Medical Center, Nashville, Tennessee (A.A.). Reprint requests to Amod Gupta, MS, Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160 012, India; fax: (⫹91)172-747837; e-mail: [email protected] 0002-9394/02/$22.00 PII S0002-9394(02)01501-5

©

2002 BY

presentations that seemed to affect the choriocapillaris primarily. Our patients appeared to have a variation of serpiginous choroidopathy, typical of the Asian-Indian population, that had some important differences from that reported in Caucasians. (Am J Ophthalmol 2002; 134:47–56. © 2002 by Elsevier Science Inc. All rights reserved.)

S

ERPIGINOUS CHOROIDOPATHY IS A PROGRESSIVE RE-

current inflammatory disease of the choroid and choriocapillaris that involves the retina secondarily.1 The disease typically occurs in otherwise healthy young to middle-aged adults. The lesions appear as gray-white to yellow with ill-defined edges; classically begin in a peripapillary location, and spread centrifugally with multiple recurrences. The fluorescein angiographic appearance is distinctive, with the acute lesions showing hypofluorescence early and progressive late staining.1– 4 Isolated peripheral lesions have also been described.5–7 Acute posterior multifocal placoid pigment epitheliopathy (APMPPE), on the other hand, is an acute inflammatory disorder of the outer retina affecting young adults who present with sudden visual loss with a frequent history of an antecedent viral-like illness. The disease is generally benign with rapid resolution of the fundus lesions and good visual recovery.8 –11 Recently, Jones and associates12 have described six patients with a new entity named relentless placoid choroiretinitis with features similar to APMPPE and serpiginous choroidopathy. In our population from North India, based on the initial presentation, we describe three variable presentations of serpiginous choroidopathy that were categorized into (1) serpiginous choroidopathy; (2) multifocal serpiginous choroidopathy, where lesions initially presented as multifocal pigment epitheliopathy and later progressed to serpiginous choroidopathy; and (3) mixed variety with eyes showing features of serpiginous, multifocal, and geographic serpiginous choroidopathy that is characterized by wavelike progression of one or more placoid area of inner choroidal inflammation.

ELSEVIER SCIENCE INC. ALL

RIGHTS RESERVED.

47

had a detailed ocular examination followed by fundus photography and fluorescein angiography at presentation and subsequently as and when required, and detailed comparisons of the initial and follow-up fundus photographs and fluorescein angiograms were made. Laboratory tests, including purified protein derivative (PPD) skin test, chest x-ray, and Venereal Disease Research Laboratory (VDRL)/Treponema pallidum heamagglutination (TPHA) tests were done for all patients. Systemic corticosteroids constituted the mainstay of therapy in both the groups and were administered whenever the center of the macula was involved or threatened. Initially, high-dose oral corticosteroids (1 mg/kg/day) were given for no longer than 1 month after which they were tapered and discontinued if possible. In patients with exacerbations of the disease during the tapering schedule, a higher dosage was resumed for another month or until the disease quieted and then was tapered to a dose just above the threshold at which the disease reactivated. In patients requiring chronic suppression of the disease, a maintenance dose of 10 mg/day was administered. Treatment with immunosuppresive agents was added if there was no response or worsening after 2 to 4 weeks. The best-corrected visual acuity (BCVA) was assessed for all the groups and was converted to the logarithm of the minimal angle of resolution (logMAR) score, as described by Ferris and associates13 which is closely approximated by the logarithm of the reciprocal of traditional Snellen visual acuity. The two groups (I and II) were compared using the chi-square test for statistical significance. Statistical testing was conducted at the .05 significance levels. The median values for age and visual acuity were compared using the Mann–Whitney U test. Only groups with more than 30 eyes were included for the analysis. The third group, comprised of eight eyes of four patients with mixed findings, was not included for further analysis.

METHODS IN A RETROSPECTIVE COHORT STUDY, PATIENTS WITH A

diagnosis of serpiginous choroidopathy were identified from the clinic and photography records of the Uveitis Clinic population at the Postgraduate Institute of Medical Education and Research, Chandigarh, India. The patients’ histories and details of the initial ophthalmic examinations were reviewed. Fundus photographs as well as the angiograms were reviewed to confirm the clinical diagnosis. Only patients with a minimum follow-up of 2 years were included in the study. The patients were retrospectively categorized into three groups. Group I included patients with an atypical presentation of serpiginous choroidopathy fulfilling the following criteria: (1) the presence of typical active grayish-white lesions with jigsaw puzzle contours developing adjacent to the optic nerve head or in the macula with pseudopodia-like extensions; (2) fluorescein angiograms in the active stage that showed early hypofluorescence with eventual progressive hyperfluorescence seen in late frames of the angiograms (the progressive hyperfluorescence helped to distinguish late staining of the edge of an inactive lesion from late leakage and staining of the edge of an active lesion, as the active lesions tend to show progressive hyperfluorescence); and (3) after resolution, the characteristic atrophy of the outer retina and inner choroid with circumscribed irregular hyperpigmented borders. Group II included patients with initial presentation of multifocal pigment epitheliopathy that initially mimicked APMPPE and were diagnosed on the basis of the following criteria: (1) multiple gray-white round-to-oval lesions at the level of the outer retina and inner choroid in and around the posterior pole and mid-periphery; (2) the lesions that were uniformly hypofluorescent during early dye transit became hyperfluorescent in the late stages of fluorescein angiography, with no progressive edge; (3) during follow-up, multiple recurrences with spread of the individual lesions, confluence of the multifocal lesions, or appearance of new focal lesions occurred in all the eyes; and (4) a final picture that resembled a milder version of chronic serpiginous choroidopathy with variable atrophy of outer retina and inner choroid, which was true of all affected eyes. In addition, four patients with different findings in two eyes were excluded from these groups and placed in group III. This was done to allow the comparison of patients with homogenous findings in the subgroup analysis. In all the groups, only patients with follow-up data, including the detailed description of initial episode as well as subsequent history regarding recurrent episodes of inflammation, if any, were included in the study. Patients who did not show any activity or progression in at least one of the eyes during follow-up with us were excluded from the study. The duration of the disease between onset and presentation was recorded for all the patients. All patients 48

AMERICAN JOURNAL

RESULTS ONE HUNDRED TWENTY-SIX PATIENTS WITH A CLINICAL

history and features suggestive of serpiginous choroidopathy or those with initial presentation as multifocal pigment epitheliopathy and who later progressed to serpiginous choroidopathy were seen at our Uveitis clinic between the years 1989 and 1997. Thirty eight of 126 (33.3%) patients were seen at an inactive stage, and 2 were lost to follow up and were excluded from the study. Of the remaining 86 patients, 62 were diagnosed as serpiginous choroidopathy based on the above-mentioned criteria and were included in group I, while group II included 20 patients who initially presented as multifocal pigment epitheliopathy and progressed to serpiginous choroidopathy. Four of these patients had mixed findings in the two eyes and have been described separately in group III. There were 42 men (67.7%) and 20 women (32.3%) in OF

OPHTHALMOLOGY

JULY 2002

TABLE 1. Demographic and Ocular Profiles of Group I and II Patients Group I n ⫽ 67

Age (years) Range Mean Median Gender Males Females Bilaterality Diseases onset—presentation interval (days) Median Range Prodrome Anterior segment inflammation Vitritis Central foveal Involvement Recurrences Visual acuity Range Initial Final Mean Initial Final Median Initial Final Final clinical features Healed geographic scar Associated healed multifocal scars Subretinal fibrosis Follow-up (years) Range Median

Group II n ⫽ 32

95% Confidence Interval

P Value

Odds Ratio

.20 .47

— —

.44

0.52

0.15, 0.17

⬍.001*

0.05

0.01, 0.21

18–57 31.97 ⫾ 9.8 30

11–45 28.86 ⫾ 7.8 29

42 (67.7%) 20 (32.2%) 5 (8.06%)

16 (80.0%) 4 (20.0%) 12 (60.0%)

7.0 4–9 0 10 (14.9%) 30 (44.78%)

6.5 4–8 2 (6.25%) 4 (12.5%) 12 (37.50%)

.06





.19 .98 .49

— 1.22 1.35

— 0.35, 4.26 0.57, 3.20

54 (80.60%) 58 (86.57%)

15 (46.88%) 32 (100%)

⬍.001* .07

4.7 0.00

1.87, 11.82 0.00, 0.92

0.2–2.9 0.0–2.9

0.1–2.5 0.9–1.8

1.03 ⫾ 0.6 0.66 ⫾ 0.5

0.81 ⫾ 0.4 0.17 ⫾ 0.5

.06 ⬍.001

— —

⫺9.23, 40.03 0.31, 0.67

0.80 0.50

0.80 0.28

.52 .01†

67 (100%) 12 (17.9%) 17 (25.37%)

32 (100%) 32 (100%) 1 (3.13%)

— ⬍.001* .02*





3–8 5

2–7 4.5

.06

⫺1.72, 7.94 —

*Significant by chi square test. † Significant by Mann–Whitney test for significance.

group I, with ages ranging from 18 to 57 years with a median age of 30 years; the 25th percentile was 25 years; and the 75th percentile was 35 years. Group II included 16 men (80%) and 4 women (20%) with ages ranging from 11 to 45 years with a median age of 29 years; the 25th percentile was 24 years; and 75th percentile was 32.50 years. None of the patients in group I and only 2 patients in group II had prodromal upper respiratory tract infection. There was no difference in the two groups in the duration of follow-up, with group I patients having a median follow-up of 5 years, while those of group II having a median follow-up of 4.5 years (Table 1). All the 67 affected eyes of 62 patients in group I presented initially with grayish-white lesions developing adjacent to the disk or in the macula. The disease was VOL. 134, NO. 1

bilateral in 5 (8.1%). None of these patients had simultaneous involvement of both eyes at presentation. These lesions showed early hypofluorescence and late progressive hyperfluorescence of the active borders. Sixteen eyes (23.4%) in addition also developed multiple round-to-oval lesions at the level of outer retina and inner choroid during the course of the disease. The spread of the initial lesions was centrifugal (outward progression from optic disk) in 41 eyes (61.2%) and centripetal (i.e., beginning in the macula and progressing towards optic disk) in 10 eyes (14.9%), while in the rest it was not recorded. In group II, all 32 affected eyes of 20 patients showed multiple gray-white round-to-oval lesions at the level of the outer retina and inner choroid in the posterior pole and mid-periphery. The disease was bilateral in 12 patients

SERPIGINOUS CHOROIDOPATHY

49

(60%); of these, only 4 (20%) had bilateral disease at initial presentation. The lesions were hypofluorescent during early dye transit and became hyperfluorescent in the late stage of fluorescein angiography. The comparison between groups I and II is shown in Table 1. The age and gender did not differ significantly between the two groups. Bilaterality was significantly higher in group II patients. There was no difference in the interval between disease onset and presentation in the two groups. There was also no significant difference between the two groups in the presence of anterior segment inflammation and vitritis. The central foveal involvement was found to be significantly higher in group I patients. There was no significant difference between the two groups in the number of recurrences. All eyes in both the groups had healed geographic scars. Associated multifocal scars were seen in a significantly higher number of group II eyes. Subretinal fibrosis was significantly higher in group I patients. One eye in group I developed subretinal neovascularization during follow-up, two eyes showed periphlebitis on fluorescein angiography, and one eye had a branch retinal vein occlusion. None of the patients in group II showed the angiographic evidence of periphlebitis, subretinal neovascularization, or branch retinal vein occlusion. The geographic scars in both groups of patients mainly showed the atrophy of the outer retina with irregular hyperpigmented borders. Twenty-five eyes (37.31%) in group I and eight eyes (25%) in group II, in addition, also showed evidence of geographic atrophy of outer choroid with exposure of large choroidal vessels with no significant difference between the two. None of the eyes showed atrophy of large choroidal vessels. The initial and final visual acuities are listed in Table 1. Median final visual acuity was significantly better among patients in group II. All patients received oral corticosteroids. Three patients in group I, in addition, also received immunosuppressive agents (oral azathioprine [two patients] and intravenous cyclophosphamide [one patient]). Response to treatment was considered good if a patient could achieve a final visual acuity of 20/40 or better. At initial presentation, only five eyes in group I and 18 eyes in group II had a visual acuity of 20/40 or better. Final visual acuity of 20/40 or better could be achieved in 38 of 67 eyes (56.7%) in group I and 32 of 40 eyes (80%) in group II. Group III was comprised of four patients who had different presentations in the two eyes. The details are listed in Table 2. The disease was bilateral in all. Patient 1 showed the presence of multifocal pigment epitheliopathy in the right eye, which over a period of time progressed to serpiginous choroidopathy while the left eye had healed geographic scar. Patients 2 and 4 showed a new pattern in their left and right eyes, respectively. In these patients, the disease initially presented as one or more confluent areas of inner choroidal inflammation in the posterior pole. These patches showed wavelike progression in both the eyes, eventually resembling serpiginous choroidopathy. Patient 50

AMERICAN JOURNAL

2 showed acute and subacute multifocal lesions in the right eye, while the left eye showed wavelike progression of five placoid lesions (Figures 1A–1H). The second eye of patient 4 showed multifocal pigment epitheliopathy (MPE) progressing to serpiginous choroidopathy. Patient 3 had a serpiginous choroidopathy-like picture in the left eye and the right eye presented initially as MPE progressing to serpiginous choroidopathy. Three patients were treated with systemic corticosteroids, while one (patient 3) received both systemic corticosteroids and immunosuppressives (intravenous cyclophasphamide). Final visual acuity ranged from Log 0.0 to Log 1.20 (mean 0.36 ⫾ 0.41, median 0.35). Final pictures in all showed the presence of a healed geographic scar with healed multifocal lesions in three eyes and subretinal fibrosis in one. Laboratory tests including a PPD skin test, chest x-ray, and VDRL/TPHA, were unremarkable in all the groups. As per records, none of the patients had any history suggestive of cutaneous herpes zoster immediately prior to, during, or after developing visual symptoms.

CASE REPORT A 29-YEAR-OLD MAN PRESENTED IN AUGUST 1991 WITH

complaints of decreased vision in the left eye of 2 weeks’ duration. The BCVA was 20/20 in the right and 20/80 in the left eye. Funduscopy of the left eye revealed a yellowish-white subretinal lesion with irregular edges in the posterior pole and inferotemporal retina (Figure 2A) with a healed pigmented scar temporal to the macula. A fundus fluorescein angiogram revealed the lesion to be hypofluorescent in the early phase with late hyperfluorescence. A diagnosis of serpiginous choroidopathy was made, and the patient was managed with oral corticosteroids and intravenous cyclophosphamide. The lesions healed and visual acuity in the left eye improved to 20/60. Four years later, he presented with complaints of decreased vision and floaters in the right eye. Ocular examination revealed a BCVA of 20/80 in the right eye and 20/20 in the left eye. Funduscopy revealed multiple yellowish-white well-circumscribed round-to-oval lesions, mainly in the posterior pole area. On fluorescein angiography, the lesions were hypofluorescent during early dye transit and hyperfluorescent in the late phase (Figures 2B and C). The juxtapapillary area was uninvolved and the presentation was like MPE. The patient was given oral corticosteroids at a dose of 1.5 mg/kg body weight. The visual acuity improved to 20/60 in the right eye and the lesions resolved within 3 weeks (Figure 2D). Ten months later, the patient had a recurrence in the right eye and the initial discrete lesions in the right eye became confluent, involving the juxtapapillary area. On fluorescein angiography the lesions were hypofluorescent in the early phase with hyperfluorescent edges in the late phase (Figure 2E). This recurrence once again was managed with oral corticosteroids, which were OF

OPHTHALMOLOGY

JULY 2002

TABLE 2. Group III Demographic & Ocular Profile of Patients Visual Acuity (logMAR) Pt. Age No. (years) Sex Initial* Final

1.

29

M

0.6

1.2 2.

33

M

0.6

0.8

3.

29

M

0.6

0.6

4.

44

F

0.6

1.0

Duration of Anterior Follow-up Segment (months) Inflammation Vitritis

Description of Lesion

0.2 OD: MPE with progression to serpiginous choroidopathy 1.2 OS: Healed geographic scar 0.5 OD: serpiginous choroidopathy with isolated lesion 0.5 OS: isolated placoid lesions with wavelike progression 0.0 OD: MPE lesions progressing to serpiginous choroidopathy 0.0 OS: serpiginous choroidopathy

26

36

120

0.5 OD: single large placoid lesion with wavelike progression 0.0 OS: MPE progressing to serpiginous choroidopathy

72

































Treatment

Recurrences

Final Clinical Features

Corticosteroids



Corticosteroids



Hyperpigmented geographic scar OU OD: healed multifocal lesions Healed geographic scar OU with exposure of large choroidal vessels

Corticosteroids ⫹ Cyclophosamide



Corticosteroids



Geographic scars with hyperpigmented borders OU Associated healed multifocal scars OD OD: geographic scar

OS: multifocal healed lesions with subretinal fibrosis

F ⫽ Female; M ⫽ Male; MPE ⫽ multifocal pigment epitheliopathy; OD ⫽ right eye; OS ⫽ left eye; OU ⫽ both eyes. *Initial visual acuity represents the visual acuity at the time the eye was first affected.

maintained at a dose of 10mg/day for a period of 18 months. Eight years later, the patient maintains a visual acuity of 20/20 in the right eye and 20/20 in the left with geographic scarring of the fundus in both eyes (Figures 2F and G).

DISCUSSION SERPIGINOUS CHOROIDOPATHY, A CHRONICALLY RECUR-

ring disease, characteristically begins in the juxtapapillary area as one or more jigsaw puzzle-shaped zones of graywhite discoloration that is often in continuity with a zone of retinal pigment epithelium (RPE) and choroidal atrophy that surrounds all or a portion of the optic disk.1– 4,14 Within a few weeks, the gray lesions become pale and atrophic while the active edge advances further to involve adjacent areas and, eventually, the fundus shows multiple jigsaw-puzzle-shaped areas of chorioretinal atrophy.15 In our population we found that serpiginous choroidopathy had a presentation that was different in several aspects from what has been described in the literature. In Caucasians, the disease usually becomes evident in fourth VOL. 134, NO. 1

to fifth decade of life,1– 4,14 though rarely has it been reported in younger patients.16 In the Indian population, the disease was seen in younger patients with a median age of 30 years (range, 18 –57 years). Other features that were different in our population were the unilaterality of the disease, which is reported to be mostly bilateral in Caucasians,2,5,14,16 the presence of anterior segment inflammation, the low frequency of subretinal neovascularization (i.e., 15% compared to 56% in previously reported series17), and preservation of large choroidal vessels. Patients with APMPPE, on the other hand, usually have bilateral posterior creamy white lesions. The disease runs a self-limited monophasic course with good visual recovery.8 –11 In group II of our series, 20 patients (32 eyes) presented with multiple creamy white round-to-oval lesions in and around the posterior pole that initially could not be differentiated from APMPPE. The fluorescein angiographic appearance showed early hypofluorescence and late hyperfluorescence of the lesions. The disease, however, was bilateral only in 12 (60%) patients. Whether we were seeing a variant of APMPPE in the Indian population or a different disease was not clear at initial

SERPIGINOUS CHOROIDOPATHY

51

presentation and hence these patients were labeled as having MPE. However, on follow-up, the eyes with multifocal lesions showed emergence of a disease pattern different from APMPPE. While recurrence of APMPPE is rare and has been documented only in a few in52

AMERICAN JOURNAL

stances,11,18 –20 in our patients isolated MPE lesions showed ameboid spread into contiguous areas over a period of several months to years. Recurrence of disease is a wellknown feature of serpiginous choroidopathy and occurs at the edge of a preexisting lesion and spreads with fingerlike OF

OPHTHALMOLOGY

JULY 2002

FIGURE 1. (A) Group III (patient 2): Fundus photograph right eye showing an irregular confluent grayish white juxtapapillary lesion involving the posterior pole with an active grayish white edge more marked superiorly and inactive centrally with an isolated temporal lesion of active choroidopathy (arrow). (B), (C) Group III (patient 2): Fundus fluorescein angiogram right eye showing irregular filling and hypofluorescence of lesions in the early phase with late hyperfluorescence of active edges. (D) Group III (patient 2): Right eye 7 years after onset shows extensive peripapillary atrophy and hyperpigmentation. (E) Group III (patient 2): Fundus fluorescein angiogram right eye 7 years later. Note progression of the lesion (shown in Figure 1C leading to extensive atrophy of retinal pigment epithelium [RPE] and choroidal vessels). (F) Group III (patient 2): Fundus fluorescein angiogram of the left eye at presentation showing five placoid lesions; the central three are becoming confluent and showing hyperfluorescence of the lesions with central hypofluorescence. The temporal lesions are hyperfluorescent. These lesions showed wavelike progression. (G) Group III (patient 2): Fundus photograph of same eye as in (F) 10 months later showing healed pigmented geographic lesions with atrophy of outer retina and inner choroid resulting in exposure of large choroidal vessels in the posterior pole. (H) Group III (patient 2): Corresponding fundus fluorescein angiogram, with left eye showing hyperfluorescence from the large choroidal vessels shown in (G).

projections into adjacent areas. While most cases of serpiginous choroidopathy begin in the juxtapapillary area, the majority of our patients in group II presented with lesions in and around the posterior pole. After multiple recurrences, the peripapillary retina was involved subsequently in many of these eyes. In the acute stage, the multifocal active lesions of APMPPE and serpiginous choroiditis are often indistinguishable and in such patients a history of antecedent illness is evidence in favor of APMPPE. However, only two of our patients in group II had a prodromal illness. In the absence of prodrome, the diagnosis of APMPPE was presumptive in our patients. In group III patients, contralateral eyes had different presentations. In APMPPE, despite extensive alternations in the pigment, the RPE cells and the photoreceptors apparently recover and the return of vision to 20/30 is typical. Gass 8 in a series of 30 patients followed for at least 1 year, found that only two of 59 eyes failed to obtain a visual acuity of 20/30 or better. However, other authors have reported poor visual outcome in recurrent APMPPE on long-term follow-up.11 The visual prognosis in serpiginous choroidopathy is poor if the fovea is involved due to extensive loss of choriocapillaris and choroidal atrophy. The final visual status in our patients was variable, and a final visual acuity of 20/40 or better was achieved in 56.7% of eyes in group I patients and 80% of eyes in group II patients. Recently, Jones and associates12 have described six patients with an unusual clinical entity resembling APMPPE and serpiginous choroidopathy, but with a proVOL. 134, NO. 1

gressive clinical course and widespread distribution of lesions. They termed the entity “relentless placoid chorioretinitis” and believed that it represented a variant of serpiginous choroidopathy. Our patients in group II resembled the patients described in their series with respect to the presence of initial yellow-white placoid lesions, demonstrating early hypofluorescence with later staining, multiple recurrences with growth of previous lesions, appearance of new lesions, and good therapeutic response to oral corticosteroids. However, bilaterality of the disease was less common in our patients (i.e., 12 [60%] of 20 patients compared to their series where 5 of the 6 [83.3%] patients had bilateral disease). The differential diagnosis in these patients would include retinal pigment epithelitis and other causes of choroiditis. The lesions of retinal pigment epithelitis are more subtle, smaller in size, and do not show recurrences.21 Other causes of choroiditis including sarcoidosis, tuberculosis, Vogt–Koyanagi–Harada disease, and sympathetic ophthalmia were discounted by the lack of any other supportive evidence. History, review of fundus photographs, and fluorescein angiograms along with laboratory investigations narrowed the differential diagnosis to APMPPE or serpiginous choroiditis in our patients. Corticosteroids constituted the mainstay of therapy in both the groups. In a recently published article, Jabs and associates22 have highlighted the scarcity of data in literature regarding the treatment of serpiginous choroidopathy. Laatikainen and associates16 have reported a good clinical response to corticosteroids in a small case series. One small case series of five eyes reported resolution of acuity within 2 weeks of initiation of triple immunosuppression.23 In our experience, we found that systemic corticosteroids were beneficial for most of our patients who responded within 2 to 4 weeks of initiation of the therapy. Subretinal fibrosis is an uncommon complication of posterior uveitis and has been described more often in patients with severe or recurrent multifocal choroiditis24 and Vogt–Koyanagi–Harada disease.25–28 Chisholm and associates2 have described subretinal fibrosis as a long-term complication of serpiginous choroiditis in 19 eyes of 20 patients, all of whom had bilateral disease. In our series, the occurrence of subretinal fibrosis was higher in group I, with 17 eyes (25.37%) showing subretinal fibrosis, compared with only 1 eye (3.13%) in group II. In addition, group II patients showed significantly higher bilaterality, less central foveal involvement, and better final visual acuity. Many other comparisons between the two groups had borderline P values which do not necessarily rule out the possibility that true differences exist as the relatively large P values could simply be related to small sample size. Our results indicate that serpiginous choroidopathy has variable presentations in the North Indian population. Both groups I and II represent the variations of serpiginous choroidopathy in the Indian population. Group I patients more closely resembled the classic form that has been

SERPIGINOUS CHOROIDOPATHY

53

described in the Caucasians, although the important differences observed in the Indian population included unilaterality, relatively younger age, presence of associated anterior segment inflammation, low rate of large choroidal 54

AMERICAN JOURNAL

vessel exposure, and low rate of subretinal neovascularization. This form of disease appears to be milder compared with patients with typical serpiginous choroiditis in Caucasians. In addition, group II represents a subgroup of OF

OPHTHALMOLOGY

JULY 2002

FIGURE 2. (A) Group III (patient 3): Fundus photograph of left eye at presentation showing yellowish-white lesion in the posterior pole with active inferior yellowish-white irregular serpiginous lesion in lower temporal mid-periphery (arrow). Temporal to the fovea, there is extensive scarring with pigmentary changes. (B) Group III (patient 3): Fundus fluorescein angiogram of the right eye at presentation showing multifocal lesions in the posterior pole that are hypofluorescent in dye transit. Note transmission hyperfluorescence in the center of large hypofluorescent lesion indicating an area of relative healing. (C) Group III (patient 3): Fundus fluorescein angiogram of the right eye in late phase showing that initial hypofluorescent lesions have become hyperfluorescent in the late phase. The central hyperfluorescent patch shows progressive hyperfluorescence in the late phase indicating the presence of biphasic lesions. (D) Group III (patient 3): Fundus photograph of the right eye 3 weeks later showing resolution of lesions with pigmentary changes. (E) Group III (patient 3): Fundus fluorescein angiogram of the right eye in late dye transit phase showing hyperfluorescent lesions with areas of blocked fluorescence caused by overlying pigment. (F), (G) Group III (patient 3): Fundus photographs of the right eye (F) and left eye (G) 8 years later showing healed pigmented lesions. Note extensive pigmentation in the lower temporal quadrant in (G) when compared to (A).

patients with serpiginous choroidopathy who present with acute multifocal lesions resembling APMPPE. This form of disease also appears to be milder, at least initially, compared with patients with typical serpiginous choroiditis. The lesions need not necessarily begin at the optic disk. Multiple recurrences, both angiographic and ophthalmoscopic enlargement of individual lesions, contiguous spread from preexisting lesions, and confluence of multifocal lesions were observed over a period of time. There was considerable overlap between these two patterns, and a third subset of patients showed mixed features in the opposite eyes. In our experience at a tertiary care referral institute, we did not see any typical case of APMPPE, as has been described in Caucasians. All patients that were initially diagnosed as having APMPPE showed progression resembling serpiginous choroiditis during follow-up. In an epidemiologic study from South India of 1273 uveitis cases seen over 2 years, 69 were diagnosed as geographic helicoid peripapillary choroiditis, while only 5 were diagnosed as having APMPPE.29 However, the course of disease in these 5 patients is not known. All of our patients represent variations of serpiginous choroiditis, some of whom have lesions that can be misinterpreted as APMPPE, but true APMPPE remains a distinct and separate entity that is, perhaps, not commonly seen in the Indian population. ACKNOWLEDGMENT

We thank Dr. V. K. Gupta, Head, Division of Design and Experiments, IASRI, New Delhi for the statistical assistance. VOL. 134, NO. 1

REFERENCES 1. Gass, JDM. Inflammatory diseases of the retina and choroid. In: Stereoscopic atlas of macular diseases: diagnosis and treatment. 4th ed. St. Louis: CV Mosby, 1997;158 –165. 2. Chisholm IH, Gass JDM, Hutton WL. The late stage of serpiginous (geographic) choroiditis. Am J Ophthalmol 1976;82:343–351. 3. Hamilton AM, Bird AC. Geographical choroidopathy. Br J Ophthalmol 1974;58:784 –797. 4. Schatz H, Maumenee AE, Patz A. Geographic helicoid peripapillary choroidopathy: Clinical presentation and fluorescein angiographic findings. Trans Am Acad Ophthalmol Otolaryngol 1974;78:747–761. 5. Hardy RA, Schatz H. Macular geographic helicoid choroidopathy. Arch Ophthalmol 1987;105:1237–1242. 6. Jampol LM, Orth D, Daily MJ, Rabb MF. Subretinal neovascularization with geographic (serpiginous) choroiditis. Am J Ophthalmol 1979;88:663– 689. 7. Mansour AM, Jampol LM, Packo KH, Hrisomalos NF. Macular serpiginous choroiditis. Retina 1988;8:125–131. 8. Gass JDM. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1968;80:177–185. 9. Ryan SJ, Maumenee AE. Acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol 1972;74:1066 – 1074. 10. Heath J. D, Spalton DJ. Acute posterior multifocal placoid pigment epitheliopathy. Int Ophthalmol Clin 1995; 35:93– 105. 11. Damato BE, Nanjiani M, Foulds WS. Acute posterior multifocal placoid pigment epitheliopathy: a follow up study. Trans Ophthalmol Soc U K 1983;103:517–522. 12. Jones BE, Jampol LE, Yannuzzi LA et al. Relentless placoid chorioretinitis. A new clinical entity or an unusual variant of serpiginous chorioretinitis? Arch Ophthalmol 2000;118: 931–938. 13. Ferris FL, Kaskoff A, Bresnick GH et al. New visual acuity charts for clinical research. Am J Ophthalmol 1982;94;91– 96. 14. Abu el-Asrar AM. Serpiginous choroiditis. Int Ophthalmol Clin 1995;35:87–91. 15. Weiss H, Annesley WH, Jr, Shields JA, Tomer T, Christopherson K. The clinical course of serpiginous choroidopathy. Am J Ophthalmol. 1979;87:133–142. 16. Laatikainen L and Erkkila H. Serpiginous choroiditis. Br J Ophthalmol 1974;58:777–783. 17. Blumenkranz MS, Gass JDM, Clarkson JG. Atypical serpiginous choroiditis. Arch Ophthalmol 1982;100:1773–1775. 18. Lyness AL, Bird AC. Recurrences of acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol 1984;98:203–207. 19. Lewis RA, Martonyi CL. Acute posterior multifocal placoid pigment epitheliopathy: a recurrence Arch Ophthalmol 1975;93:235–238. 20. Williams DF, Mieler WF. Long-term follow up of acute posterior multifocal placoid pigment epitheliopathy. Br J Ophthalmol 1989;73:985–990. 21. Krill AE, Deutman AF. Acute retinal pigment epithelitis. Am J Ophthalmol 1972;74:193–206. 22. Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of Immunosuppressive drugs in patients with ocular inflammatory disorders. Recommendations of an expert panel. Am J Ophthalmol 2001;131:679. 23. Hooper PL, Kaplan HJ. Triple agent immunosuppression in serpiginous choroiditis. Ophthalmology 1991;98:944 –952. 24. Gass JDM, Margo CE, Levy MH. Progressive subretinal

SERPIGINOUS CHOROIDOPATHY

55

gressive subretinal fibrosis and uveitis. Br J Ophthalmol 1984;68:667– 673. 28. Kuo IC, Rechdouni A, Rao NA, Johnston RH, Margolis TP, Cunningham Jr. ET. Subretinal fibrosis in patients with Vogt–Koyanagi–Harada disease. Ophthalmology 2000;107: 1721–1728. 29. Biswas J, Narain S, Dedashis D, Ganesh SK. Pattern of uveitis in a referral uveitis clinic in India. Int Ophthalmol 1996;20:223–228.

fibrosis and blindness in patients with multifocal granulomatous chorioretinitis. Am J Ophthalmol 1996;122:76 – 85. 25. Salvador F, Garcia Arumi J, Mateo C, et al. Multifocal choroiditis with progressive subretinal fibrosis. Ophthalmologica 1994;208:163–167. 26. Cantrill HL, Folk JC. Multifocal choroiditis associated with progressive subretinal fibrosis. Am J Ophthalmol 1986;101: 170 – 80. 27. Palestine AG, Nussenblatt RB, Parver LM, Knox DL. Pro-

56

AMERICAN JOURNAL

OF

OPHTHALMOLOGY

JULY 2002

Lihat lebih banyak...

Comentários

Copyright © 2017 DADOSPDF Inc.