CHOROIDAL GRANULOMAS IN SYSTEMIC SARCOIDOSIS

UDAY R. DESAI, MD,* KHALED A. TAWANSY, MD,* BRIAN C. JOONDEPH, MD, RHETT M. SCHIFFMAN, MD, MS*
Purpose: To evaluate the clinical course, including response to therapy, of patients with macular and peripapillary choroidal granulomas secondary to systemic sarcoidosis. Methods: This is a retrospective case study and literature review. Nine patients with choroidal granulomas were identied. Eight patients had a tissue biopsy conrming sarcoidosis; one was diagnosed from clinical history and typical gallium scan. Ocular examinations included fundus examination, uorescein angiography, and visual eld examination. Eight patients had magnetic resonance imaging (MRI) scans looking for intracranial granulomas. Treatment consisted of oral prednisone in eight patients (one with concomitant subconjunctival triamcinolone); one patient received no treatment because of good vision and granuloma in the nasal retina. Variables studied included visual acuity (VA), response of granulomas to treatment, time to recurrence, and associated anterior segment ndings. Results: Eight of nine patients had a solitary lesion whereas one had multifocal involvement. The granulomas ranged in size from one half to four disk diameters. Eight patients had blurry vision; one was asymptomatic. All nine patients had hilar adenopathy and/or pulmonary parenchymal disease. No patient had nonocular neurologic symptoms and in eight patients who underwent MRI examination no intracranial granulomas were detected. Of the eyes that were treated (n 8) all had decrease in the size of the choroidal mass at an average of 4 months of treatment. Two had complete resolution. Mean follow-up was 29.2 months. At the time of initial diagnosis only one patient had an active anterior uveitis. Five of nine patients had at least one recurrence. Mean time to recurrence was 7.6 months after discontinuing oral prednisone. The VA at presentation ranged from 20/30 to 20/300. Final VA was 20/30 or better in all patients. Conclusions: Choroidal granulomas related to systemic sarcoidosis respond well to oral corticosteroids. They may recur but good vision can be maintained. They are not typically associated with concomitant iritis and also do not appear to be associated with intracranial granulomas. RETINA 21:40 47, 2001

arcoidosis is an inammatory disease of unknown etiology whose histologic hallmark is the presence of noncaseating granulomas composed of epithelioid cells and Langerhans giant cells. Any organ system
From *Eye Care Services, Henry Ford Health Sciences Center, and VitreoRetinal Consultants, P.C., St. Johns Hospital Medical Center, Detroit, Michigan. Reprint requests: Uday Desai, MD, Eye Care Services, Henry Ford Health System, 2799 W. Grand Boulevard, K-10, Detroit, MI 48202.

may be affected, but more frequent involvement may be seen in the lungs, liver, skin, central nervous system (CNS), and eyes.1 The incidence of ocular disease in biopsy-proven sarcoidosis has ranged from 26 to 63% in recent studies.2 4 These same studies show uveitis affecting between 28 and 74% of patients with ocular sarcoidosis. James et al have found the most common ocular manifestation to be anterior uveitis, which occurs in 60% of patients with eye disease.5 Posterior segment ndings, which are seen in approx40

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imately 25% of patients with ocular involvement, include vitritis, retinal vasculitis, chorioretinitis, and granulomas of the retina, optic nerve, or choroid.6 12 Five and one half percent of patients with ocular sarcoidosis have been noted to have choroidal granulomas.6 If only patients with posterior segment involvement are examined the incidence of choroidal granulomas rises to 12%.13 Patients with posterior segment disease have been shown to have twice the incidence of CNS involvement when compared to the whole population of patients with sarcoidosis.6,13 Whether all types of posterior disease are associated with CNS manifestations is uncertain. Similarly, it is uncertain whether all patients with posterior segment sarcoidosis are a homogeneous group. To further dene the characteristics of sarcoidosis-related choroidal granulomas, including their potential to be associated with CNS involvement, we performed the following retrospective study. Patients and Methods We reviewed the charts of all patients with a diagnosis of sarcoidosis who were seen at the ophthalmology department of Henry Ford Hospital between 1990 and 1995. Eight patients were identied who had a creamy-white elevated choroidal mass in the macula or peripapillary area. An additional patient was included in this analysis from a local practice. All nine patients were African American. Age at diagnosis ranged from 31 to 68 years (mean, 48). Eight of nine patients had negative puried protein derivative (PPD) tests with controls. The patient with a positive PPD also had a positive tissue biopsy for sarcoidosis and his response to steroid treatment was more typical for sarcoidosis. Eight patients had a positive tissue biopsy conrming sarcoidosis. One patient was diagnosed from her clinical history and typical gallium scan. Fluorescein angiography (FA) and perimetry were obtained routinely in all patients, as indicated by the clinical course. Magnetic resonance imaging (MRI) scans were performed on eight of nine patients to rule out intracranial granulomas. Patient follow-up was a minimum of 1 year. Mean follow-up was 29.2 months. Indication for treatment was any decrease in visual acuity (VA) that was secondary to the presence of the granuloma. The cause of the visual decrease was either a mass effect on the macula or optic nerve or an accumulation of subretinal uid in these areas. Treatment modalities included oral prednisone in all treated patients and subconjunctival triamcinolone injection in one patient. One patient did not require treatment. Treatment was tapered based on clinical response. Angiotensin converting enzyme (ACE) levels were

available at presentation in all patients and on resolution of the granulomas in seven patients. Detailed clinical summaries of three representative cases are described. Case Reports Case 1
A 39-year-old man presented with subcutaneous skin nodules and swollen wrists in April 1992. Skin biopsy revealed noncaseating granulomas. Chest x-ray showed interstitial nodules and ACE was 99 U/mL (normal 1170). He was treated with 80 mg of prednisone orally, which was tapered according to his clinical response. In May 1993 he was referred by his optometrist for fundus evaluation. Visual acuity was 20/25 bilaterally and he had early nuclear sclerotic cataracts. Funduscopy of the right eye revealed two creamy-white choroidal masses. The rst lesion, which was located inferonasal to the disk, was 1 disk diameter (DD). The second lesion, which was present under the superotemporal arcade, measured 2 DD. The left fundus had an area of punctate pigment epithelial defects in the inferotemporal periphery. On FA, the masses showed early choroidal hypouorescence followed by late leakage and staining. Oral prednisone was again started at 80 mg/d and was tapered on a monthly basis as long as the lesions were regressing. In April 1994 he had photopsia and metamorphopsia. Vision had dropped to 20/50 in the right eye and there was an increase in the size of the granuloma under the superotemporal arcade. Increasing exudation with subretinal uid encroaching on the foveal avascular zone was seen. Subretinal hemorrhage was noted, and FA demonstrated a subretinal neovascular membrane (Figures 1 and 2). No inammation was found in the anterior segment or vitreous. The patient was treated with prednisone 80 mg/d for 1 week. This was followed by a decremental tapering of the dose, which betted the clinical improvement of the granulomas. By August the membrane had disappeared, the lesions had completely regressed, and vision returned to 20/25. On reevaluation in March 1995, he had blurred vision. Visual acuity was unchanged, and there was a new granuloma nasal to the optic nerve in the left eye that measured 1 DD. He was restarted on 40 mg/d of prednisone and the lesion regressed over the next 2 months, leaving a choroidal scar. He has since been weaned off steroids with no recurrence to date.

Case 2
A 31-year-old man was seen in March 1993 complaining of blurred vision in the right eye. He had a history of pulmonary sarcoidosis diagnosed 2 years earlier after presenting with bronchitis. His chest x-ray showed hilar adenopathy and a transbronchial biopsy revealed noncaseating granulomas. When rst examined, he was taking 10 mg/d of oral prednisone. His VA was 20/25 in the right eye and 20/20 in the left. Anterior segments were quiet and without evidence of prior inammation. In the center of the right macula was a choroidal granuloma that was 2 mm wide and 2 mm in thickness. The periphery of the right fundus was unaffected. Funduscopy of the left eye was unremarkable. Fluorescein angiography of the right eye showed hypouorescence from blockage of the choroidal vasculature with late leakage and staining. The patient was observed for the next 6 months; the lesion remained relatively stable. In September 1993, VA deteriorated to 20/60 in the right eye, and there was elevation of the neurosensory retina

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Fig. 1. Superotemporal macula of the right eye shows cream-colored choroidal granuloma. Subretinal hemorrhage is present on nasal side of mass. A retinochoroidal anastomosis is seen in the center of the mass. This manifests as a retinal arteriole diving into the granuloma.

Fig. 3. Choroidal granuloma located in the macula of the right eye. Surrounding the yellow granuloma is a ring of neurosensory retinal elevation.

(Figure 3), with uorescein leakage extending into the fovea. The patient was treated with 80 mg of oral prednisone, which was tapered according to the clinical response. The lesion decreased in size over the subsequent 2 months and vision improved to 20/25 in the right eye. By February 1994, the prednisone was discontinued. He had retinal pigment epithelial atrophy in the area of former elevation (Figure 4). Vision has remained stable since. Anterior segment and vitreous has remained free of inammation throughout his course.

Case 3
A 48-year-old woman was seen in January 1991 after referral by her internist. She had a history of systemic sarcoidosis for the last

5 years. She had presented with bronchitis and a chest x-ray showed bilateral hilar adenopathy. Biopsy of skin nodules revealed noncaseating granulomas. She had been treated intermittently with oral prednisone for skin plaques, and was starting hydroxychloroquine therapy. She had a history of high myopia, Fuchs cornealdystrophy, and anterior uveitis in the right eye. Visual acuity was 20/20 bilaterally and color vision was normal. Both corneas had mild guttata. No synechiae were observed. Anterior chambers were quiet and the lenses were clear. The right fundus had retinal pigment epithelial atrophy extending from the optic nerve and along the inferotemporal arcade. The patient was observed with routine examinations until July 1994, when she had deteriorating vision in the right eye. Visual acuity had dropped to 20/25 and there was a 1 relative afferent pupillary defect. Color vision remained normal, but perimetry showed an enlarged blind spot.

Fig. 2. Mid-arteriovenous uorescein angiography shows hyperuorescence of the granuloma. The subretinal neovascular membrane responsible for the subretinal hemorrhage is demonstrated by the white arrows.

Fig. 4. Pigment mottling and atrophy of the pigment epithelium is seen in the center of the macula as the granuloma has resolved. The dark shadows in the superonasal macula are a photographic artifact.

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Fig. 5. Optic nerve is edematous with blurred disk margins. Yellow white choroidal elevation is seen in the peripapillary retina inferotemporal to the optic nerve.

Fig. 6. After oral steroids, the peripapillary choroidal granula is seen to decrease in size and the optic nerve is better dened.

There was choroidal elevation in the area of previous atrophy with accumulation of subretinal uid. The optic nerve was hyperemic with blurring of the inferior margin. Fluorescein angiogram showed massive leakage from the optic nerve. No anterior or posterior segment inammation was seen. She received a subconjunctival injection of 20 mg of triamcinolone in the inferotemporal quadrant and was started on 80 mg/d of oral prednisone. Vision remained stable on monthly examinations. The prednisone was tapered over 8 months. In November 1995, she was taken off hydroxychloroquine by her dermatologist. She then had grayness in the right nasal visual eld. Visual acuity had dropped to 20/200 in the right eye and there was a 3 right afferent pupillary defect. There was massive elevation of the granuloma to 4.0 mm in thickness with associated optic nerve edema. Vitritis was not present. Prednisone was restarted at 80 mg/d. Seven weeks later vision improved to 20/40 and the granuloma decreased in size. Head MRI showed no evidence of intracranial sarcoidosis. By January 1996 the choroidal mass had resolved but a macular scar remained (Figures 5 and 6).

Results Nine patients were identied with a diagnosis of sarcoidosis with the presence of a choroidal granuloma in the macula or peripapillary region (Table 1). Five patients were women and four were men. All were African American. Age ranged from 31 to 68 years with a mean of 48 years. The eight patients who had a positive tissue biopsy for sarcoidosis had the diagnosis made before the presentation of the choroidal granuloma. The most common sites for biopsy included the bronchioles and the skin. The patient diagnosed with sarcoidosis clinically was diagnosed after the development of the ocular ndings. Eight of nine patients had a solitary choroidal gran-

uloma around the posterior pole. Two were peripapillary and three were subfoveal. One patient each had a granuloma located along a temporal arcade, in the temporal macula, and in the nasal midperiphery. One patient had a multifocal presentation with granulomas located in the superotemporal and inferonasal quadrants of the right eye. He subsequently developed a granuloma in the nasal midperiphery of his left eye while under observation. The granulomas ranged in size from 0.5 to 4 DD. One of nine patients was visually asymptomatic. This was a 44-year-old man who presented with a complaint of burning eyes, which was related to lacrimal insufciency. Funduscopy showed an asymptomatic granuloma along the superotemporal arcade. The remaining eight patients had blurred vision. Three described metamorphopsia, two described seeing halos, and two described para central scotomas. Systemic features of sarcoidosis included hilar adenopathy and/or pulmonary parenchymal disease in nine patients, cutaneous granuloma in three patients, lacrimal dysfunction in one patient, and hypercalcemia in one patient. Fluorescein angiography in the acute untreated lesions showed early choroidal hypouorescence due to blockage from the mass. By the arteriovenous phase, there were multifocal spots of hyperuorescence from the lesion, which continued to leak throughout the remainder of the angiogram. This leakage would pool in the subretinal space in areas of neurosensory retinal detachment. Also, late staining of the choroidal mass was observed. Fluorescein angiography in treated lesions displayed retinal pigment epithelium window defects. In the two cases of peripapillary involvement marked leakage was observed from the optic nerve.

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Table 1. Patient Characteristics

Age, yr/ Sex Location 20/50 20/60 20/20 185/27 34/26 185/120 20/20 71/57 241/145 20/40 20/25 20/25 20/25 20/200 20/30 134/43 57 7 20/25 99/93 Other Organ Involvement

Eye

Tissue for Diagnosis

Anterior Segment Involvement

Treatment Modality and Duration, mo

ACE Level, Worst Final /mL, Snellen Snellen Initial/at Acuity Acuity Resolution

Posterior Segment Complications

Time to Recurrence, mo 7 None 4 3 6 18 None None Chorioretinal atrophy None

39/M

Skin

None

31/M Inferior peripapillary and Lung, skin inferotemporal arcade Superior parafoveal Peripapillary and parafoveal Temporal macula Foveal Nasal arcades Inferotemporal macula Skin Lungs None Prednisone, 11 Prednisone, 8 Lung Prednisone, 2 20/40

Bronchial

None

Superotemporal arcade Skin, lungs, Prednisone, 4 and inferior macula peripheral nodes Subfoveal Lung Prednisone, 6

48/F

Skin

Remote iritis

Subretinal heme, CNVM, chorioretinal scar Retinal pigment epithelial defects, pigment clumps Chronic mass with macular star exudate

44/M

Lacrimal

None

Peribulbar 20/200 20/30 Depo-Medrol, prednisone, 8 Prednisone, 2 20/50 20/25

44/F

Bronchial

None

Lung, lacrimal, peripheral nodes Lung Prednisone, 4

53/F

Bronchial

Panuveitis

20/100 20/30

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38/F

Bronchial

None

Lungs, kidneys, hypercalcemia

2001

67/F

Gallium scan

Telangiectasis, exudates, chronic masses Shunt vessels, chronic mass, chorioretinal atrophy Vasculitis, BRVO, chorioretinal atrophy Telangiectasis, chorioretinal atrophy, epiretinal membrane Chorioretinal scar

68/M

Skin

Remote iritis Iritis

VOLUME 21

ACE, angiotensin-converting enzyme; CNVM, choroidal neovascular membrane; BRVO, branch retinal vein occlusion.

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Visual eld examination of these two peripapillary lesions showed an enlarged blind spot in one and a paracentral scotoma in the other. One of these two patients had an afferent pupillary defect that resolved with systemic steroid therapy. The second patient did not have an afferent pupillary defect. Both patients with peripapillary granulomas had normal color vision. None of the nine patients had nonocular neurologic symptoms. Eight of nine patients underwent gadolinium enhanced MRI of the head. No intracranial lesions and specically no intracranial granulomas were detected. Treatment dosages for prednisone ranged from 40 to 100 mg per day initial dose, with a tapering of the dose over 3 to 6 months depending on the clinical response. Lesions responded to prednisone therapy within an average of 4 months. Two patients had complete resolution of the lesion, so that no residual mass could be detected, and only mottling of the retinal pigment epithelium remained. Of these two patients, one had a subfoveal granuloma, and presented at the onset of symptoms. The second patient had a granuloma develop in the temporal macula of his only functioning eye. It is presumed therefore that he presented early in the course of the granuloma and was promptly treated with systemic prednisone. This prompt treatment may have played a role in allowing complete resolution of the choroidal masses. The remaining patients had longer times from the onset of symptoms to the initiation of treatment. These seven patients had regression of the choroidal lesions with reabsorption of the subretinal uid, but without complete disappearance of the granulomas. Five of nine patients had at least one recurrence after tapering off prednisone. These were growths of the initial choroidal mass, with increasing subretinal exudation. One patient developed a choroidal granuloma at a new focus in the fellow eye. The mean time to recurrence was 7.6 months after discontinuing prednisone. One patient had active anterior uveitis when the choroidal granuloma appeared. Two patients had an antecedent history of iritis, and another developed panuveitis 20 months after the granuloma had become quiescent. The initial VA at presentation was variable, ranging from 20/30 to 20/300. The patients with the most subfoveal uid and thickest lesions had worse VA. The nal VA was uniformly good, ranging from 20/20 to 20/30 in all patients. The patients with subfoveal lesions had subjective complaints of distorted vision despite good Snellen acuities.

Discussion In our series, anterior uveitis does not appear to be associated with the acute choroidal granuloma. Four patients had a history of anterior uveitis but only one had the uveitis concurrently with the granuloma. This nding is in agreement with Tingey and Gonders review of seven similar cases in which only one patient had a remote history of granulomatous uveitis.14 This lack of associated anterior segment involvement differs from other varieties of posterior segment disease that occur in sarcoidosis. Obenauf and associates found that posterior segment disease was unlikely in the absence of anterior segment involvement.6 In their study the majority of patients with posterior segment disease had chorioretinitis or retinal vasculitis. The higher incidence of anterior uveitis in patients with chorioretinitis or retinal vasculitis has been described by other authors. Duker et al described 11 patients with retinal vasculitis, seven of whom had concomitant anterior uveitis.15 Chorioretinitis associated with sarcoidosis has been shown by Deutsch and Tessler16 and by Lardenoye and associates17 to be consistently accompanied by anterior uveitis. Patients with choroidal granulomas cannot be relied upon to have anterior chamber reaction to serve as an indicator of posterior segment disease. Our patients show that, in fact, unless secondary involvement of the macula or optic nerve is present, choroidal granulomas may go unnoticed. The importance of identifying an asymptomatic choroidal granuloma is open to debate. The uniformly good visual outcome in our patients supports the notion that, even if asymptomatic lesions are allowed to become symptomatic by a lack of identication, VA is not compromised as long as adequate treatment is administered. Alternatively, it may be consequential not to identify an asymptomatic granuloma, if the presence of one may be a harbinger of CNS disease. Whereas certain forms of neurosarcoidosis may result in minimal dysfunction, others have lower 2-year remission rates with increased morbidity and mortality.18 Posterior segment disease in ocular sarcoidosis has been linked to CNS abnormalities.19 Gould and Kaufman noted a much higher rate of neurosarcoidosis in patients with fundus abnormalities when compared to all patients with sarcoidosis (37%2%).13 However, the majority of their patients had retinal periphlebitis or perivenous nodules. In our patients clinical examination and MRI scanning did not disclose any evidence of associated CNS involvement. Whereas it is incorrect to say that CNS disease does not occur in patients with choroidal granulomas, given the small number of

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our patients, it does not appear to be a frequent accompaniment. Given the lack of asymptomatic CNS lesions on MRI scanning we would not advocate routine neuroimaging in patients with choroidal granulomas. Various articles that deal specically with sarcoid granulomas of the choroid have also failed to identify CNS lesions in their patients.14,20 23 Whereas the few patients in the literature and in our study preclude any statistical meaning, it seems prudent to perform neuroimaging only on patients with neurologic symptoms. Other ancillary tests did not have a prominent role in the diagnosis and management of choroidal granulomas. Because eight of our nine patients already were known to have biopsy-proven sarcoidosis, diagnosis was made on clinical examination. Angiotensin converting enzyme levels were not particularly helpful in diagnosing the granulomas as four of the nine patients had normal ACE levels at the time the granuloma was diagnosed. The ACE levels also did not correlate with the size of the granuloma. However, in patients with grossly elevated ACE levels at presentation, a subsequent drop correlated well with attening of the mass. These patients were treated with systemic steroids and the drop in the ACE level probably reected a reduction in extraocular granuloma formation. The FA ndings were nonspecic. The diagnosis of sarcoidosis-related choroidal granulomas cannot be made solely on angiography. Amelanotic choroidal melanoma, choroidal hemangioma, metastatic lesions, and other granulomas are in the differential diagnosis. The FA is useful in identifying associated choroidal neovascularization and can be used to monitor resolution of the new vessels during treatment with oral corticosteroids. Visual eld testing failed to identify any unsuspected lesions of neurosarcoidosis. Whereas it is unlikely to nd visual eld abnormalities in patients who do not have MRI evidence of visual pathway abnormalities it is possible that subtle lesions in the retrobulbar optic nerve or chiasm may result in visual eld abnormalities. Our patients have visual eld abnormalities that correlate with their funduscopic ndings. These ndings indicate that the likelihood of nding CNS involvement by visual eld testing seems to be small in cases of sarcoidosis-related choroidal granulomas. Routine perimetry does not appear to be indicated. In 1982, Marcus and associates reported two patients with biopsy proven sarcoidosis and macular choroidal granulomas.20 Both lesions completely resolved with steroid therapy, leaving retinal pigment epithelial defects that transmitted but did not leak uorescein. Both patients had a nal acuity of 20/20.

Olk and associates described a similar patient with a peripapillary granuloma that completely resolved with systemic steroids.21 In 1984, Campo and Aaberg reported two patients with similar lesions, but only partial resolution of the choroidal granulomas occurred with systemic steroid therapy.22 Final acuities were 20/60 and 20/25, but the rst case had associated granulomatous iritis. There was no apparent correlation between the size of the lesion or duration of therapy and the extent of attening. In the current study, two of nine patients had complete resolution of their granulomas, such that no creamy white subretinal deposit could be seen, and only pigmentary abnormalities remained. The remaining seven patients had partial attening of the masses with resolution of the macular neurosensory detachments when present and excellent visual outcomes. Both patients in this study who had complete disappearance of the granulomas had the initiation of steroids within a week of the onset of their symptoms. Of Marcus et als and Olk et als patients with complete resolution, two of three had treatment initiated within 1 week of treatment.20,21 The third patient had complete resolution even though symptoms were present for 2 months before treatment was begun. Of Campo and Aabergs patients with partial resolution, one was treated 3 months after the onset of symptoms and the second was treated 1 week after the development of symptoms.22 It is possible that earlier treatment may permit more complete reduction of the choroidal mass in these patients, but it is difcult with such a small number of patients to say this with any degree of certainty. In this study, the average time to resolution 4 monthswas comparable to the 3 months reported in a patient of Campo and Aaberg.22 Olk et als patient resolved within 5 months21; the two patients of Marcus et al resolved in 3 weeks and 1 year, respectively.20 Similar to Campo and Aaberg, we had a patient with a choroidal neovascular membrane that disappeared on prednisone therapy; no laser treatment was necessary. Frank and Weiss described a subretinal neovascular membrane in an eye with multiple widespread choroidal granulomas and panuveitis.7 They were able to treat the membrane successfully with laser photocoagulation. Because systemic prednisone was effective in allowing involution of the choroidal neovascular membrane in our patient we would advise such treatment before proceeding to laser photocoagulation. In a 1955 report on pulmonary sarcoidosis, Scadding wrote that if the lesions are in a reversible stage, cortisone can cause dramatic clearing, but there is equally no doubt that, after this dramatic clearing, the

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lesions frequently recur.9 After discontinuing prednisone, ve of our nine patients relapsed at least once, with enlargement of the choroidal mass and detachment of the overlying retina. All had expansion of at choroidal masses and one had a new granuloma form. Because the mean time to recurrence was 7.6 months after discontinuing oral corticosteroids one may get a false sense of security once the granuloma responds to treatment. Patients should be told to carefully monitor their visual function and to report any changes promptly. Recurrences respond to retreatment with systemic steroids and if multiple recurrences occur, multiple treatments with tapering doses of corticosteroids may be necessary. The excellent visual outcomes in our series of choroidal sarcoid granulomas contrast markedly with Laties and Scheies review of 11 cases of optic nerve granulomas in which 5 of 11 had nal vision of count ngers or less, and an additional two had vision of 6/18.24 The worse outcomes in the patients reviewed by Laties and Scheie probably reect the intraneural or extraneural mass effect of optic nerve granulomas and its effect on damaging optic nerve bers. Poor outcome of granulomas of the optic nerve has also been described by others. Kelley and Green described a case of an optic nerve granuloma where signicant necrosis in the optic nerve mass may have contributed to the visual demise.25 Statton et al showed a case where an optic granuloma led to blindness, presumably from mass effect on the optic nerve.26 Our series, the largest of its kind, agrees with previous reports demonstrating a good prognosis and responsiveness to systemic corticosteroids for choroidal granulomas. This should include peripapillary lesions that encroach on the optic nerve but do not inltrate it. These lesions differ from other posterior segment abnormalities in sarcoidosis. They are less likely to be associated with inammation and appear not to have accompanying CNS involvement. These patients demonstrate a high rate of late recurrence and need long-term follow up. Although recurrences may occur, they respond to repeat dosing of oral corticosteroids and good VA can be maintained. Key words: sarcoidosis, choroidal granuloma, uveitis, choroid, steroids. References
1. Stanbury RM, Graham EM, Murray PI. Sarcoidosis. Int Ophthalmol Clin 1995;35:123137.

2. 3. 4. 5. 6.

7.

8. 9. 10. 11. 12.

13. 14. 15. 16. 17.

18. 19. 20. 21.

22. 23.

24.

25. 26.

James DG. Ocular sarcoidosis. Ann NY Acad Sci 1986;465: 551563. Rothova A, Alberts C, Glasius E, et al. Risk factors for ocular sarcoidosis. Doc Ophthalmol 1989;72:287296. Jabs DA, Johns CJ. Ocular involvement in chronic sarcoidosis. Am J Ophthalmol 1986;102:297301. James DJ, Neville E, Langley DA. Ocular sarcoidosis. Trans Ophthalmol Soc UK 1976;96:133139. Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol 1978;86:648 655. Frank KW, Weiss H. Unusual clinical and histopathological ndings in ocular sarcoidosis. Br J Ophthalmol 1983;67:8 16. Bruntse E. Ocular sarcoidosis. Danish Med Bull 1958;5:217 227. Scadding JG. Pulmonary sarcoidosis. Trans Ophthalmol Soc UK 1955;75:173180. Chumbley LC, Kearns TP. Retinopathy of sarcoidosis. Am J Ophthalmol 1972;73:123131. Spalton DJ, Sanders MD. Fundus changes in histologically conrmed sarcoidosis. Br J Ophthalmol 1981;65:348 358. Brod RD. Presumed sarcoid choroidopathy mimicking birdshot retinochoroidopathy. Am J Ophthalmol 1990;109:357 358. Gould H, Kaufman HE. Sarcoid of the fundus. Arch Ophthalmol 1961;65:161164. Tingey DP, Gonder JR. Ocular sarcoidosis presenting as a solitary choroidal mass. Can J Ophthalmol 1992;27:2529. Duker JS, Brown GC, McNamara JA. Proliferative sarcoid retinopathy. Ophthalmology 1988;95:1680 1686. Deutsch TA, Tessler HH. Inammatory pseudohistoplasmosis. Ann Ophthalmol 1985;17:461 465. Lardenoye CW, Van der Lelij A, de Loos WS, et al. Peripheral multifocal chorioretinitis. Ophthalmology 1997;104:1820 1826. Heck AW, Phillips II LH. Sarcoidosis and the nervous system. Neurol Clin 1989;7:641 654. Brinkman CJJ, Rothova A. Fundus pathology in neurosarcoidosis. Int Ophthalmol 1993;17:2326. Marcus DF, Bovino JA, Burton TC. Sarcoid granuloma of the choroid. Ophthalmology 1982;89:1326 1330. Olk RJ, Lipmann MJ, Cundiff HC, Daniels J. Solitary choroidal mass as the presenting sign in systemic sarcoidosis. Br J Ophthalmol 1983;67:826 829. Campo RV, Aaberg TM. Choroidal granuloma in sarcoidosis. Am J Ophthalmol 1984;97:419 427. Denis P, Nordmann J, Laroche L, Saraux H. Branch retinal vein occlusion associated with a sarcoid choroidal granuloma. Am J Ophthalmol 1992;113:333334. Laties AM, Scheie HG. Sarcoid granuloma of the optic disk: evolution of multiple small tumors. Trans Am Ophthalmol Soc 1970;68:219 233. Kelley JS, Green R. Sarcoidosis involving the optic nerve head. Arch Ophthalmol 1973;89:486 488. Statton R, Blodi FC, Hanigan J. Sarcoidosis of the optic nerve. Arch Ophthalmol 1964;71:834 836.