Choroidal Granulomas in Sistemic Sarcoidosis
Transcript of Choroidal Granulomas in Sistemic Sarcoidosis
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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.
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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
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(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.
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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.
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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.
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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
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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
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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.
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47CHOROIDAL GRANULOMAS IN SYSTEMIC SARCOIDOSIS DESAI ET AL