choroidal granulomas in sistemic sarcoidosis

7/27/2019 Choroidal Granulomas in Sistemic Sarcoidosis

1/8

CHOROIDAL GRANULOMAS INSYSTEMIC 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 withchoroidal granulomas were identified. Eight patients had a tissue biopsy confirming sarcoid-

osis; one was diagnosed from clinical history and typical gallium scan. Ocular examinations

included fundus examination, fluorescein angiography, and visual field 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 treat-

ment, time to recurrence, and associated anterior segment findings.

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 weredetected. 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 21:4047, 2001

Sarcoidosis is an inflammatory disease of unknown

etiology whose histologic hallmark is the presenceof noncaseating granulomas composed of epithelioid

cells and Langerhans giant cells. Any organ system

may be affected, but more frequent involvement may

be seen in the lungs, liver, skin, central nervous sys-

tem (CNS), and eyes.1 The incidence of ocular disease

in biopsy-proven sarcoidosis has ranged from 26 to

63% in recent studies.24 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 findings, which are seen in approx-

From *Eye Care Services, Henry Ford Health Sciences Center,and VitreoRetinal Consultants, P.C., St. Johns Hospital MedicalCenter, Detroit, Michigan.

Reprint requests: Uday Desai, MD, Eye Care Services, HenryFord Health System, 2799 W. Grand Boulevard, K-10, Detroit, MI48202.

40


2/8

imately 25% of patients with ocular involvement, in-clude vitritis, retinal vasculitis, chorioretinitis, and

granulomas of the retina, optic nerve, or choroid.612

Five and one half percent of patients with ocular

sarcoidosis have been noted to have choroidal granu-lomas.6 If only patients with posterior segment in-

volvement are examined the incidence of choroidalgranulomas rises to 12%.13

Patients with posterior segment disease have beenshown to have twice the incidence of CNS involve-

ment when compared to the whole population of pa-tients with sarcoidosis.6,13 Whether all types of poste-

rior disease are associated with CNS manifestations isuncertain. Similarly, it is uncertain whether all pa-

tients with posterior segment sarcoidosis are a homo-geneous group. To further define the characteristics of

sarcoidosis-related choroidal granulomas, includingtheir 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 diag-nosis of sarcoidosis who were seen at the ophthalmol-

ogy department of Henry Ford Hospital between 1990and 1995. Eight patients were identified who had a

creamy-white elevated choroidal mass in the maculaor peripapillary area. An additional patient was in-

cluded in this analysis from a local practice. All ninepatients were African American. Age at diagnosis

ranged from 31 to 68 years (mean, 48). Eight of ninepatients had negative purified protein derivative (PPD)tests with controls. The patient with a positive PPD

also had a positive tissue biopsy for sarcoidosis andhis response to steroid treatment was more typical for

sarcoidosis. Eight patients had a positive tissue biopsyconfirming sarcoidosis. One patient was diagnosed

from her clinical history and typical gallium scan.Fluorescein angiography (FA) and perimetry were ob-

tained routinely in all patients, as indicated by theclinical course. Magnetic resonance imaging (MRI)

scans were performed on eight of nine patients to ruleout 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 thegranuloma. The cause of the visual decrease was ei-

ther a mass effect on the macula or optic nerve or anaccumulation of subretinal fluid in these areas. Treat-

ment modalities included oral prednisone in all treatedpatients 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 resolu-tion of the granulomas in seven patients. Detailed

clinical summaries of three representative cases aredescribed.

Case Reports

Case 1

A 39-year-old man presented with subcutaneous skin nodules

and swollen wrists in April 1992. Skin biopsy revealed noncase-

ating 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 re-

vealed two creamy-white choroidal masses. The first 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 pigmentepithelial defects in the inferotemporal periphery. On FA, the

masses showed early choroidal hypofluorescence 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 fluid encroaching on the foveal avascular

zone was seen. Subretinal hemorrhage was noted, and FA demon-

strated a subretinal neovascular membrane (Figures 1 and 2). No

inflammation 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 befitted

the clinical improvement of the granulomas. By August themembrane had disappeared, the lesions had completely re-

gressed, 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 bron-chitis. His chest x-ray showed hilar adenopathy and a transbron-

chial biopsy revealed noncaseating granulomas. When first exam-

ined, 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 inflammation. 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 unaf-

fected. Funduscopy of the left eye was unremarkable. Fluorescein

angiography of the right eye showed hypofluorescence from block-

age 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

41CHOROIDAL GRANULOMAS IN SYSTEMIC SARCOIDOSIS DESAI ET AL


3/8

(Figure 3), with fluorescein 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 inflammation through-

out 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 hydroxychloro-

quine therapy. She had a history of high myopia, Fuchs corne-

aldystrophy, 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 andthere was a 1 relative afferent pupillary defect. Color vision

remained normal, but perimetry showed an enlarged blind spot.

Fig. 1. Superotemporal macula of the right eye shows cream-colored

choroidal granuloma. Subretinal hemorrhage is present on nasal side ofmass. A retinochoroidal anastomosis is seen in the center of the mass.

This manifests as a retinal arteriole diving into the granuloma.

Fig. 2. Mid-arteriovenous fluorescein angiography shows hyperfluo-rescence of the granuloma. The subretinal neovascular membrane re-

sponsible for the subretinal hemorrhage is demonstrated by the whitearrows.

Fig. 3. Choroidal granuloma located in the macula of the right eye.

Surrounding the yellow granuloma is a ring of neurosensory retinal

elevation.

Fig. 4. Pigment mottling and atrophy of the pigment epithelium is

seen in the center of the macula as the granuloma has resolved. Thedark shadows in the superonasal macula are a photographic artifact.

42 RETINA, THE JOURNAL OF RETINAL AND VITREOUS DISEASES 2001 VOLUME 21 NUMBER 1


4/8

There was choroidal elevation in the area of previous atrophy with

accumulation of subretinal fluid. 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 inflammation was seen.

She received a subconjunctival injection of 20 mg of triamcin-

olone 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 field. 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 de-

creased 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 identified with a diagnosis ofsarcoidosis with the presence of a choroidal granu-

loma 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 68years with a mean of 48 years. The eight patients who

had a positive tissue biopsy for sarcoidosis had thediagnosis made before the presentation of the choroi-

dal granuloma. The most common sites for biopsyincluded the bronchioles and the skin. The patient

diagnosed with sarcoidosis clinically was diagnosedafter the development of the ocular findings.

Eight of nine patients had a solitary choroidal gran-

uloma around the posterior pole. Two were peripap-illary and three were subfoveal. One patient each had

a granuloma located along a temporal arcade, in thetemporal macula, and in the nasal midperiphery. One

patient had a multifocal presentation with granulomaslocated in the superotemporal and inferonasal quad-

rants of the right eye. He subsequently developed agranuloma 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. Thiswas a 44-year-old man who presented with a com-plaint of burning eyes, which was related to lacrimal

insufficiency. Funduscopy showed an asymptomaticgranuloma along the superotemporal arcade. The re-

maining eight patients had blurred vision. Three de-scribed metamorphopsia, two described seeing halos,

and two described para central scotomas. Systemic fea-tures of sarcoidosis included hilar adenopathy and/or

pulmonary parenchymal disease in nine patients, cu-taneous granuloma in three patients, lacrimal dysfunc-

tion in one patient, and hypercalcemia in one patient.Fluorescein angiography in the acute untreated le-

sions showed early choroidal hypofluorescence due toblockage from the mass. By the arteriovenous phase,

there were multifocal spots of hyperfluorescence fromthe lesion, which continued to leak throughout the

remainder of the angiogram. This leakage would poolin the subretinal space in areas of neurosensory retinal

detachment. Also, late staining of the choroidal masswas observed. Fluorescein angiography in treated le-

sions displayed retinal pigment epithelium windowdefects. In the two cases of peripapillary involvement

marked leakage was observed from the optic nerve.

Fig. 5. Optic nerve is edematous with blurred disk margins. Yellowwhite choroidal elevation is seen in the peripapillary retina inferotem-

poral 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 defined.



5/8

Table

1.PatientCharacteristics

Age,

yr/Sex

Eye

Tissuefor

Diagnosis

Anterior

Segment

Involv

ement

Location

OtherOrgan

Invo

lvement

Treatment

Modalityand

Duration,mo

Worst

Snellen

Acuity

Final

S

nellen

Acuity

ACELevel,

/mL,

Initial/at

Resolution

PosteriorSegme

nt

Complications

Timeto

Recurrence,

mo

39/M

R

Skin

None

Superotemporalarcade

andinferiormacula

Skin,lu

ngs,

perip

heralnodes

Prednisone,4

20/50

20/25

99/93

Subretinalheme,CN

VM,

chorioretinalscar

7

31/M

R

Bronchial

None

Subfoveal

Lung

Prednisone,6

20/60

20/20

185/27

Retinalpigmentepith

elial

defects,pigmentc

lumps

None

48/F

R

Skin

Remote

iritis

Inferiorperipapillaryand

inferotemporalarcade

Lung,s

kin

Peribulbar

Depo-Medrol,

prednisone,8

20/200

20/30

34/26

Chronicmasswithm

acular

starexudate

4

44/M

R

Lacrimal

None

Superiorparafoveal

Lung,lacrimal,

perip

heralnodes

Prednisone,2

20/50

20/25

185/120

Telangiectasis,exudates,

chronicmasses

3

44/F

L

Bronchial

None

Peripapillaryand

parafoveal

Lung

Prednisone,4

20/40

20/20

71/57

Shuntvessels,chron

ic

mass,chorioretinal

atrophy

6

53/F

L

Bronchial

Panuv

eitis

Temporalmacula

Lung

Prednisone,2

20/100

20/30

241/145

Vasculitis,BRVO,

chorioretinalatrophy

18

38/F

R

Bronchial

None

Foveal

Lungs,

kidneys,

hypercalcemia

Prednisone,8

20/40

20/25

134/43

Telangiectasis,

chorioretinalatrophy,

epiretinalmembran

e

None

67/F

L

Gallium

scan

Remote

iritis

Nasalarcades

Lungs

None

20/25

20/25

57

Chorioretinalscar

None

68/M

L

Skin

Iritis

Inferotemporalmacula

Skin

Prednisone,11

20/200

20/30

7

Chorioretinalatrophy

None

ACE,angiotensin-convertingenzyme;CNVM,choroidalneovascularmemb

rane;BRVO,branchretinalveinocclusio

n.



6/8

Visual field 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 colorvision.

None of the nine patients had nonocular neurologic

symptoms. Eight of nine patients underwent gadolin-

ium enhanced MRI of the head. No intracranial le-

sions and specifically 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 pre-

sented 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 re-

maining patients had longer times from the onset ofsymptoms to the initiation of treatment. These seven

patients had regression of the choroidal lesions with

reabsorption of the subretinal fluid, but without com-

plete 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 granu-

loma at a new focus in the fellow eye. The mean time

to recurrence was 7.6 months after discontinuing pred-

nisone. One patient had active anterior uveitis when

the choroidal granuloma appeared. Two patients hadan 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 fluid and thickest lesions had worse VA.

The final 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 onehad the uveitis concurrently with the granuloma. This

finding is in agreement with Tingey and Gondersreview of seven similar cases in which only one pa-

tient had a remote history of granulomatous uveitis.14

This lack of associated anterior segment involvement

differs from other varieties of posterior segment dis-ease that occur in sarcoidosis.

Obenauf and associates found that posterior seg-ment disease was unlikely in the absence of anterior

segment involvement.6 In their study the majority ofpatients with posterior segment disease had chorioreti-

nitis or retinal vasculitis. The higher incidence ofanterior uveitis in patients with chorioretinitis or ret-

inal vasculitis has been described by other authors.Duker et al described 11 patients with retinal vascu-

litis, seven of whom had concomitant anterior uve-itis.15 Chorioretinitis associated with sarcoidosis has

been shown by Deutsch and Tessler16 and by Larde-noye and associates17 to be consistently accompanied

by anterior uveitis. Patients with choroidal granulo-mas cannot be relied upon to have anterior chamber

reaction to serve as an indicator of posterior segmentdisease.

Our patients show that, in fact, unless secondaryinvolvement of the macula or optic nerve is present,

choroidal granulomas may go unnoticed. The impor-tance of identifying an asymptomatic choroidal gran-

uloma is open to debate. The uniformly good visual

outcome in our patients supports the notion that, evenif asymptomatic lesions are allowed to become symp-

tomatic by a lack of identification, VA is not compro-mised as long as adequate treatment is administered.

Alternatively, it may be consequential not to iden-tify an asymptomatic granuloma, if the presence of

one may be a harbinger of CNS disease. Whereascertain forms of neurosarcoidosis may result in mini-

mal dysfunction, others have lower 2-year remissionrates with increased morbidity and mortality.18 Poste-

rior segment disease in ocular sarcoidosis has beenlinked to CNS abnormalities.19 Gould and Kaufman

noted a much higher rate of neurosarcoidosis in pa-tients with fundus abnormalities when compared to all

patients with sarcoidosis (37%2%).13 However, themajority of their patients had retinal periphlebitis or

perivenous nodules. In our patients clinical examina-tion and MRI scanning did not disclose any evidence

of associated CNS involvement. Whereas it is incor-rect to say that CNS disease does not occur in patients

with choroidal granulomas, given the small number of



7/8

our patients, it does not appear to be a frequentaccompaniment.

Given the lack of asymptomatic CNS lesions onMRI scanning we would not advocate routine neuro-

imaging in patients with choroidal granulomas. Vari-ous articles that deal specifically with sarcoid gran-

ulomas of the choroid have also failed to identifyCNS lesions in their patients.14,2023 Whereas the

few patients in the literature and in our study pre-clude any statistical meaning, it seems prudent to

perform neuroimaging only on patients with neuro-logic symptoms.

Other ancillary tests did not have a prominent rolein the diagnosis and management of choroidal granu-

lomas. Because eight of our nine patients already wereknown to have biopsy-proven sarcoidosis, diagnosis

was made on clinical examination. Angiotensin con-verting enzyme levels were not particularly helpful in

diagnosing the granulomas as four of the nine patientshad normal ACE levels at the time the granuloma was

diagnosed. The ACE levels also did not correlate withthe size of the granuloma. However, in patients with

grossly elevated ACE levels at presentation, a subse-quent drop correlated well with flattening of the mass.

These patients were treated with systemic steroids andthe drop in the ACE level probably reflected a reduc-

tion in extraocular granuloma formation.The FA findings were nonspecific. 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 reso-lution of the new vessels during treatment with oral

corticosteroids.Visual field testing failed to identify any unsus-

pected lesions of neurosarcoidosis. Whereas it is un-likely to find visual field abnormalities in patients who

do not have MRI evidence of visual pathway abnor-malities it is possible that subtle lesions in the retro-

bulbar optic nerve or chiasm may result in visual fieldabnormalities. Our patients have visual field abnor-

malities that correlate with their funduscopic findings.These findings indicate that the likelihood of finding

CNS involvement by visual field testing seems to besmall in cases of sarcoidosis-related choroidal granulo-

mas. Routine perimetry does not appear to be indicated.In 1982, Marcus and associates reported two pa-

tients with biopsy proven sarcoidosis and macularchoroidal granulomas.20 Both lesions completely re-

solved with steroid therapy, leaving retinal pigmentepithelial defects that transmitted but did not leak

fluorescein. Both patients had a final acuity of 20/20.

Olk and associates described a similar patient with aperipapillary granuloma that completely resolved with

systemic steroids.21 In 1984, Campo and Aaberg re-ported two patients with similar lesions, but only

partial resolution of the choroidal granulomas oc-curred with systemic steroid therapy.22 Final acuities

were 20/60 and 20/25, but the first case had associatedgranulomatous iritis. There was no apparent correla-

tion between the size of the lesion or duration oftherapy and the extent of flattening. In the current

study, two of nine patients had complete resolution oftheir granulomas, such that no creamy white subreti-

nal deposit could be seen, and only pigmentary abnor-malities remained. The remaining seven patients had

partial flattening of the masses with resolution of themacular neurosensory detachments when present and

excellent visual outcomes.Both patients in this study who had complete dis-

appearance of the granulomas had the initiation ofsteroids within a week of the onset of their symptoms.

Of Marcus et als and Olk et als patients with com-plete resolution, two of three had treatment initiated

within 1 week of treatment.20,21 The third patient hadcomplete resolution even though symptoms were

present for 2 months before treatment was begun. OfCampo and Aabergs patients with partial resolution,

one was treated 3 months after the onset of symptomsand the second was treated 1 week after the develop-

ment of symptoms.22 It is possible that earlier treat-ment may permit more complete reduction of the

choroidal mass in these patients, but it is difficult withsuch a small number of patients to say this with any

degree of certainty.In this study, the average time to resolution4

monthswas comparable to the 3 months reported in

a patient of Campo and Aaberg.22 Olk et als patientresolved 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 patientwith a choroidal neovascular membrane that disap-

peared on prednisone therapy; no laser treatment wasnecessary. Frank and Weiss described a subretinal

neovascular membrane in an eye with multiple wide-spread choroidal granulomas and panuveitis.7 They

were able to treat the membrane successfully with laserphotocoagulation. Because systemic prednisone was

effective in allowing involution of the choroidal neo-vascular membrane in our patient we would advise such

treatment before proceeding to laser photocoagulation.In a 1955 report on pulmonary sarcoidosis, Scad-

ding 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



8/8

lesions frequently recur.9 After discontinuing pred-nisone, five of our nine patients relapsed at least once,

with enlargement of the choroidal mass and detach-ment of the overlying retina. All had expansion of flat

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 afalse sense of security once the granuloma responds to

treatment. Patients should be told to carefully monitortheir visual function and to report any changes

promptly. Recurrences respond to retreatment withsystemic steroids and if multiple recurrences occur,

multiple treatments with tapering doses of corticoste-roids may be necessary.

The excellent visual outcomes in our series of cho-roidal sarcoid granulomas contrast markedly with

Laties and Scheies review of 11 cases of optic nervegranulomas in which 5 of 11 had final vision of count

fingers or less, and an additional two had vision of6/18.24 The worse outcomes in the patients reviewed

by Laties and Scheie probably reflect the intraneuralor extraneural mass effect of optic nerve granulomas

and its effect on damaging optic nerve fibers. Pooroutcome of granulomas of the optic nerve has also

been described by others. Kelley and Green describeda case of an optic nerve granuloma where significant

necrosis in the optic nerve mass may have contributedto the visual demise.25 Statton et al showed a case

where an optic granuloma led to blindness, presum-ably from mass effect on the optic nerve.26

Our series, the largest of its kind, agrees with pre-vious reports demonstrating a good prognosis and

responsiveness to systemic corticosteroids for choroi-dal granulomas. This should include peripapillary le-

sions that encroach on the optic nerve but do not

infiltrate it. These lesions differ from other posteriorsegment abnormalities in sarcoidosis. They are less

likely to be associated with inflammation and appearnot to have accompanying CNS involvement. These

patients demonstrate a high rate of late recurrence andneed long-term follow up. Although recurrences may

occur, they respond to repeat dosing of oral cortico-steroids and good VA can be maintained.

Key words: sarcoidosis, choroidal granuloma, uve-

itis, choroid, steroids.

References

1. Stanbury RM, Graham EM, Murray PI. Sarcoidosis. Int Oph-

thalmol Clin 1995;35:123137.

2. James DG. Ocular sarcoidosis. Ann NY Acad Sci 1986;465:

551563.

3. Rothova A, Alberts C, Glasius E, et al. Risk factors for ocular

sarcoidosis. Doc Ophthalmol 1989;72:287296.

4. Jabs DA, Johns CJ. Ocular involvement in chronic sarcoid-

osis. Am J Ophthalmol 1986;102:297301.

5. James DJ, Neville E, Langley DA. Ocular sarcoidosis. Trans

Ophthalmol Soc UK 1976;96:133139.6. Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoid-

osis and its ophthalmic manifestations. Am J Ophthalmol

1978;86:648655.

7. Frank KW, Weiss H. Unusual clinical and histopathological

findings in ocular sarcoidosis. Br J Ophthalmol 1983;67:8

16.

8. Bruntse E. Ocular sarcoidosis. Danish Med Bull 1958;5:217

227.

9. Scadding JG. Pulmonary sarcoidosis. Trans Ophthalmol Soc

UK 1955;75:173180.

10. Chumbley LC, Kearns TP. Retinopathy of sarcoidosis. Am J

Ophthalmol 1972;73:123131.

11. Spalton DJ, Sanders MD. Fundus changes in histologically

confirmed sarcoidosis. Br J Ophthalmol 1981;65:348358.12. Brod RD. Presumed sarcoid choroidopathy mimicking bird-

shot retinochoroidopathy. Am J Ophthalmol 1990;109:357

358.

13. Gould H, Kaufman HE. Sarcoid of the fundus. Arch Oph-

thalmol 1961;65:161164.

14. Tingey DP, Gonder JR. Ocular sarcoidosis presenting as a

solitary choroidal mass. Can J Ophthalmol 1992;27:2529.

15. Duker JS, Brown GC, McNamara JA. Proliferative sarcoid

retinopathy. Ophthalmology 1988;95:16801686.

16. Deutsch TA, Tessler HH. Inflammatory pseudohistoplasmo-

sis. Ann Ophthalmol 1985;17:461465.

17. Lardenoye CW, Van der Lelij A, de Loos WS, et al. Peripheral

multifocal chorioretinitis. Ophthalmology 1997;104:1820

1826.18. Heck AW, Phillips II LH. Sarcoidosis and the nervous sys-

tem. Neurol Clin 1989;7:641 654.

19. Brinkman CJJ, Rothova A. Fundus pathology in neurosar-

coidosis. Int Ophthalmol 1993;17:2326.

20. Marcus DF, Bovino JA, Burton TC. Sarcoid granuloma of the

choroid. Ophthalmology 1982;89:1326 1330.

21. Olk RJ, Lipmann MJ, Cundiff HC, Daniels J. Solitary cho-

roidal mass as the presenting sign in systemic sarcoidosis.

Br J Ophthalmol 1983;67:826829.

22. Campo RV, Aaberg TM. Choroidal granuloma in sarcoidosis.

Am J Ophthalmol 1984;97:419427.

23. Denis P, Nordmann J, Laroche L, Saraux H. Branch retinal

vein occlusion associated with a sarcoid choroidal granu-

loma. Am J Ophthalmol 1992;113:333334.24. Laties AM, Scheie HG. Sarcoid granuloma of the optic disk:

evolution of multiple small tumors. Trans Am Ophthalmol

Soc 1970;68:219 233.

25. Kelley JS, Green R. Sarcoidosis involving the optic nerve

head. Arch Ophthalmol 1973;89:486 488.

26. Statton R, Blodi FC, Hanigan J. Sarcoidosis of the optic

nerve. Arch Ophthalmol 1964;71:834 836.


choroidal granulomas in sistemic sarcoidosis

Documents