CLINICAL SCIENCES

Idiopathic Orbital Inflammation

Distribution, Clinical Features, and Treatment Outcome
Sonia J. Ahn Yuen, MD, PhD; Peter A. D. Rubin, MD

Objective: To evaluate the distribution and clinical as therapy (n = 8), steroids and nonsteroidal anti-inflam-
well as treatment outcome characteristics of idiopathic matory agents (n = 6), nonsteroidal anti-inflammatory
orbital inflammation with the aim of delineating a more agents alone in mild cases (n=2), and, rarely, radiation
systematic approach to diagnosis and treatment. therapy without steroids (n=1) or surgical debulking alone
(n=1). Of 65 patients, 41 (63%) represented treatment suc-
Methods: A 10-year retrospective review of patients with cesses, with complete symptom relief at the time of the
idiopathic orbital inflammation treated at one institution. last follow-up, and 24 (37%) represented treatment fail-
ures, with partial or no relief of symptoms. Treatment fail-
Results: Ninety eyes in 65 patients (22 men and 43 wom- ures were often characterized by recurrence of inflamma-
en) were studied. Diagnoses were isolated dacryoadenitis tion after a period of quiescence (58%) and unremitting,
(n=21), isolated myositis (n=19), concurrent dacryoad- recalcitrant inflammation (38%); 1 patient ultimately re-
enitis and myositis (n=5), orbital apex syndrome (n=6), quired an exenteration.
and idiopathic inflammation involving the preseptal re-
gion, supraorbital region, sclera, Tenon capsule, orbital fat, Conclusion: Systemic steroid with a slow taper has been
or optic nerve (n=14). The mean age at presentation was the established first-line treatment for idiopathic orbital
45 years. Pain and periorbital swelling were the most com- inflammation, but refractory cases accounted for a sig-
mon clinical features and were observed in 45 (69%) and nificant portion of treatment failures in our study, re-
49 (75%) patients, respectively. Seventeen patients (26%) flecting the need for a more systematic approach to the
had bilateral involvement. Biopsy was performed in 19 pa- study of this multifaceted disease and for therapeutic al-
tients (29%) with atypical presentations or who failed to ternatives to systemic steroids.
respond to the initial therapy. Patients were treated with
steroids alone (n=45), steroids and subsequent radiation Arch Ophthalmol. 2003;121:491-499

I
DIOPATHIC ORBITAL inflamma- tive diseases.11,12 It accounts for 4.7% to 6.3%
tion, also known as orbital pseu- of orbital disorders.8,13,14
dotumor, is defined as a benign, Idiopathic orbital inflammation has
noninfective clinical syndrome highly variable clinical features, ranging
characterized by features of non- from a diffuse to very focal process tar-
specific inflammatory conditions of the or- geting specific orbital tissues, such as the
bit without identifiable local or systemic lacrimal gland, extraocular muscles, and
causes. It was first described in 1903 by orbital fat. This space-occupying infiltrat-
Gleason1 and by Busse and Hochheim2 and ing orbital process is typically character-
characterized as a specific clinicopatho- ized by an abrupt onset of pain, propto-
logical entity in 1905 by Birch-Hirsch- sis, and inflammatory signs and symptoms,
feld,3,4 who described it as an orbital mass such as swelling and erythema. Presenta-
that simulated a neoplasm but was histo- tions vary according to the specific loca-
logically inflammatory. Since the initial de- tion and the degree of inflammation, fi-
scription, many classification schemes have brosis, and mass effect. Ptosis, chemosis,
From the Department of been applied to idiopathic orbital inflam- motility dysfunction, and optic neuropa-
Ophthalmology (Dr Yuen) and
mation based on the location of the inflam- thy may also be found. Entrapment, com-
the Orbital and Cosmetic
Surgery Service (Dr Rubin), matory process, the histopathological char- pression, and destruction of orbital tis-
Massachusetts Eye and Ear acteristics, and the stage of inflammation.5-10 sues may occur in patients with extensive
Infirmary, Boston. The authors Idiopathic orbital inflammation is the third sclerosis. Unilateral presentation is typi-
have no relevant financial most common orbital disease, following cal, but bilateral presentations are not un-
interest in this article. Graves orbitopathy and lymphoprolifera- common. Symptoms most commonly de-

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 velop acutely (hours to days) with patients reporting a January 1991 to April 2001. The study protocol was approved
datable onset. In the minority of patients, presentation may by the institutional review board at MEEI. The diagnosis of id-
occur over weeks (subacute) or may occur insidiously over iopathic orbital inflammation was made based on the follow-
a period of months (chronic). Pediatric idiopathic orbital ing clinical criteria: benign, noninfective, inflammatory con-
dition of the orbit without identifiable local or systemic causes.
inflammation is characterized by a number of features that
Radiological workup was performed for many patients but was
differ from the adult presentation. Bilateral manifestation, not a necessary diagnostic criterion for the study. Likewise, his-
as well as uveitis, disc edema, and eosinophilia, appear to topathological confirmation was not a diagnostic requirement
be more common in the pediatric population.15-17 for the study.
The pathogenesis of idiopathic orbital inflamma- Of 79 patients, 6 with the eventual final diagnosis of We-
tion has remained elusive. Idiopathic orbital inflamma- gener granulomatosis or Graves orbitopathy were excluded from
tion has been associated with several infectious pro- the analysis; an additional 8 patients with a follow-up period
cesses, including upper respiratory tract infections and of less than 5 weeks were also excluded from the study. A lower
flulike viral illness, but the exact nature of these asso- limit for the follow-up period was set at 5 weeks because re-
ciations is not clear.15,16,18-20 Several lines of evidence point sponse to treatment is usually evident by this time. All the re-
maining 65 patients met the criteria for idiopathic orbital in-
to immune-mediated processes as the likely underlying
flammation and had at least 5 weeks of follow-up.
ocular mechanism. Associations of idiopathic orbital in- These patients were managed according to the following
flammation with a number of systemic immunologic dis- treatment algorithm: at the initial manifestation, patients were
orders, including Crohn disease, systemic lupus ery- typically treated with a high-dose oral steroid (1.0-1.5 mg/kg
thematosus, rheumatoid arthritis, diabetes mellitus, per day) for 1 to 2 weeks with taper during the ensuing 5 to 8
myasthenia gravis, and ankylosing spondylitis, have been weeks. In the event of rebound of symptoms during steroid taper
reported in several studies.9,21-26 Mombaerts and Koorn- or recurrence of symptoms after a period of quiescence, ste-
neef,27 for instance, found in their series that 10% of 58 roid dosage was increased (with a slower taper) or restarted for
patients with idiopathic orbital inflammation had con- typically no longer than 10 to 12 weeks. Alternatively, when
current autoimmune disease. In addition, idiopathic or- the disease course was atypical or refractory to systemic ste-
roids, biopsy was performed for definitive diagnosis. Radia-
bital inflammation typically responds favorably to sys-
tion treatment was typically reserved for patients who did not
temic corticosteroid treatment and successful outcomes respond to or were intolerant to systemic steroid therapy. Ra-
have been reported with other immunosuppressive agents, diation therapy typically consisted of low-dose irradiation (usu-
such as cyclophosphamide,28 methotrexate,29 and cyclo- ally 15 to 20 Gy fractioned over 10 days). Treatment outcome
sporine.30 Interestingly, 2 other disorders with a predi- was considered a “success” if the patient had complete relief
lection for the orbit, namely Graves orbitopathy and ocu- of symptoms at the time of the last follow-up and a “failure” if
lar myasthenia gravis, are also immune-mediated. the patient had no or only partial relief of symptoms at the time
An autoimmune process has been suggested as the of the last follow-up.
ocular mechanism for idiopathic orbital inflammation by
Atabay et al,31 who reported the presence of circulating RESULTS
autoantibodies against eye muscle antigens in patients
with orbital myositis. Although autoimmunity is a plau- Ninety eyes in 65 patients (22 men and 43 women) with
sible idea, it is not clear whether these autoantibodies are a final diagnosis of idiopathic orbital inflammation and
specific to idiopathic orbital inflammation or are also pres- at least 5 weeks of follow-up were included in the data
ent in other forms of inflammation, such as scleritis and analysis. Patients were followed for a mean of 20 months
uveitis. In addition, the typical unilateral presentation of (range, 1 month to 9 years). Each patient was evaluated
idiopathic orbital inflammation argues against the no- at least twice.
tion that autoimmunity is the primary mechanism. Al-
ternatively, Mombaerts et al32 and Rootman et al33 pro- DISTRIBUTION OF SUBTYPES
posed aberrant immune-mediated production of
fibrogenic cytokines leading to aberrant wound healing The 65 patients had isolated dacryoadenitis (n=21), iso-
as the ocular mechanism underlying the process of fi- lated myositis (n = 19), concurrent dacryoadenitis and
brosis in sclerosing orbital inflammation. myositis (n=5), orbital apex syndrome (n=6), and idio-
Although benign, idiopathic orbital inflammation pathic inflammation involving the preseptal region, su-
may have a clinically malignant course, with severe vi- praorbital region, sclera, Tenon capsule, orbital fat, or
sion loss and oculomotor dysfunction. Spontaneous re- the optic nerve (n = 14). The frequency of these sub-
mission may occur without any therapy, but systemic cor- types is given in the Table. The mean age at manifesta-
ticosteroids are the cornerstone of therapy in the acute tion was 45 years (range, 2.5 weeks to 89 years).
phase. Despite the generally favorable response to ste-
roid therapy, relapses and persistent inflammation com- CLINICAL FEATURES
plicate the clinical course and treatment. Therefore, id-
iopathic orbital inflammation poses a considerable Pain was the most common symptom and was evident
diagnostic and therapeutic challenge. in at least 45 patients (69%). Diplopia was less frequent
but present in at least 20 patients (31%). Periorbital edema
METHODS was the most common sign and was present in at least
This study is a retrospective review of 79 patients with the di- 49 patients (75%), followed by red eye, proptosis, and
agnosis of idiopathic orbital inflammation who were treated at chemosis occurring in at least 31 (48%), 21 (32%), and
the Massachusetts Eye and Ear Infirmary (MEEI), Boston, from 19 (29%) of patients, respectively (Figure 1). Seven-

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 50
Frequency of Different Subtypes of
Idiopathic Orbital Inflammation 45

40
No. (%) of Patients
Subtype (N = 65) 35

Dacryoadenitis 21 (32)

No. of Patients
30
Myositis 19 (29)
Dacryoadenitis and myositis 5 (8) 25
Orbital apex* 6 (9)
20
Other† 14 (22)
15
*Orbital apex syndrome with or without involvement of the superior orbital
fissure and cavernous sinus. 10
†Idiopathic inflammation in the supraorbital region, orbital fat, sclera,
Tenon capsule, or optic nerve. 5

0
Pain Diplopia Edema Red Eye Chemosis Proptosis Uveitis Abnormal
teen patients (26%) had bilateral involvement, either con- Fundus
currently or sequentially. Twenty-six patients (40%) Initial Symptoms and Signs

showed involvement of the extraocular muscles, either Figure 1. Frequency of symptoms and signs of idiopathic orbital inflammation.
as the primary presentation, as in myositis, or as a less
prominent part of a more diffuse presentation. The most
frequently involved muscle was the medial rectus (n=18; tic nerve and may have intracranial extension into the
31%), followed by the superior rectus (n = 15; 25%), lat- cavernous sinus.
eral rectus (n=14; 24%), and inferior rectus (n=12; 20%).
Biopsy was performed in 19 patients (29%) with
atypical presentations or who failed to respond to the ini-
NEUROIMAGING AND BIOPSY
tial therapy. Of the biopsy specimens, 14 showed a be-
nign nonspecific inflammatory pattern, and the remain-
Neuroimaging studies were obtained for 63 patients
ing 5 showed a sclerosing pattern, with dense fibrosis as
(97%). The most common neuroimaging study was the
the primary feature.
computed tomographic (CT) scan, which was obtained
for 59 patients (91%). Magnetic resonance images (MRIs)
TREATMENT OUTCOME
were obtained for 10 patients, 7 in combination with a
CT scan. Ultrasonograms were obtained only for 3 pa-
Of 65 patients included in the study, 45 (69%) were treated
tients, 2 in combination with a CT scan.
with steroids alone, 8 (12%) with steroids and subse-
Radiological findings allowed subtypes of idio-
quent radiation therapy, 6 (9%) with steroids and non-
pathic orbital inflammation to be more precisely classi-
steroidal anti-inflammatory agents, 1 atypical case (2%)
fied according to the specific orbital tissues involved as
with radiation therapy and nonsteroidal anti-
follows5,6,10,11,34:
inflammatory agents, 2 mild cases (3%) with nonsteroi-
• Lacrimal gland: diffuse, oblong enlargement of the lac- dal anti-inflammatory agents alone, and 1 rare case (2%)
rimal gland with preservation of the shape of the gland with surgical debulking alone; treatment was deferred in
that may be accompanied by an inflammatory reaction the remaining 2 cases (per patient request or pending
in the periglandular tissue, blurring the gland margin. completion of workup). Of 65 patients studied, 41 (63%)
• Extraocular muscles: enlargement of the extraocular represented treatment successes and had complete symp-
muscles (single or multiple; with or without the in- tom relief; 24 (37%) represented treatment failures, with
volvement of the associated tendons) accompanied by 23 patients experiencing only partial relief and 1 patient
some spillover of the inflammatory process into orbital experiencing no relief. These treatment outcome results
fat bordering the muscle, blurring the margin of the may reflect an overall higher rate of steroid failures than
muscle. that generally observed in the community because MEEI
• Orbital fat: diffuse infiltration in the orbital fat, envel- serves as a referral center, which would introduce some
oping the globe, that may involve the optic nerve sheath degree of selection bias toward more complicated and re-
complex. calcitrant disease. The stringent definition for treat-
• Preseptum, sclera, episclera, Tenon capsule, and uvea: in- ment success used in our study, ie, complete relief of
flammation and enlargement of the tissues. symptoms may also inflate the overall higher failure rate.
• Optic nerve: inflammation of the optic nerve sheath with Treatment outcomes are shown for different subtypes of
thickening of the margin of the nerve and streaky den- idiopathic orbital inflammation (Figure 2) and treat-
sities in the contiguous orbital fat. ment modes (Figure 3).
• Orbital mass: orbital mass of heterogeneous composi-
tion, occasionally invading extraorbital structures, ex- TREATMENT FAILURE ANALYSIS
tending along the optic nerve sheath from the globe to
the optic canal. The clinical course for many patients in our study was
• Orbital apex and cavernous sinus: inflammatory process complicated by incomplete or no resolution of inflam-
at the apex may compress, obliterate, or displace the op- mation, ie, treatment failure. For 14 (58%) of 24 pa-

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 14 8
Success Steroid Intolerant
Failure Steroid Dependent
12 7

6
10

No. of Patients
No. of Patients

8
4
6
3

4
2

2 1

0 0
Dacryoadenitis Myositis Dacryoadenitis Orbital Apex Other Success Failure
and Myositis Treatment Outcome
Subtypes
Figure 4. The frequency of patients with steroid intolerance (adverse
Figure 2. Frequency of treatment outcomes for different subtypes of orbital reaction to steroid therapy) and steroid dependence (recurrence of or
pseudotumor. Success is complete resolution of symptoms at the time of the increase in symptoms during or after steroid taper) is shown for each
last follow-up. Failure is partial or no resolution of symptoms at the time of treatment outcome. Success is complete resolution of symptoms at the time
the last follow-up. of the last follow-up. Failure is partial or no resolution of symptoms at the
time of the last follow-up.

40
Success
Failure respectively, of the 24 patients who failed to respond to
35
treatment. Steroid dependence and steroid intolerance
also occurred in 12% and 2%, respectively, at some point
30
in the clinical course of patients who ultimately had suc-
25
cessful outcomes (Figure 4).
No. of Patients

Age was not a predictor of treatment failure because

20 the mean age of patients successfully treated (42 years) was
comparable to that of patients who failed to respond to treat-
15 ment (45 years). Likewise, sex was not significantly asso-
ciated with treatment failure because the male-female ra-
10 tio remained comparable for the treatment success group
(13:28) and the treatment failure group (9:15). Inflamma-
5
tion involving the orbital apex was relatively uncommon
0
in our study but was associated with the poorest treat-
Steroids Steroids Steroids Radiation NSAIDs Surgical None ment outcome; 4 (66%) of 6 patients with this subtype failed
and and and Debulking
Radiation NSAIDs NSAIDs to respond to treatment. Sclerosing orbital inflammation
Treatment Mode
also appeared to be associated with poor outcome be-
cause all 4 cases in our study failed to respond to treat-
Figure 3. Frequency of treatment outcome for different treatment modes. ment and 1 patient ultimately required an exenteration.
Success is complete resolution of symptoms at the time of the last
follow-up. Failure is partial or no resolution of symptoms at the time of the
A more detailed characterization of treatment fail-
last follow-up. NSAIDs indicates nonsteroidal anti-inflammatory drugs. ure was beyond the primary scope of this study and re-
mains to be further explored. Our study did not system-
atically address several questions that are important in
tients who failed to respond to treatment, inflammation defining more fully the pattern of recalcitrant disease and
recurred after a seemingly favorable response to treat- thereby improving treatment outcome, such as: Is there
ment with a period of quiescence. For 9 (38%) of 24 pa- a particular window period of peak recurrence after the
tients, the disease course was characterized by unremit- initial presentation, and would an aggressive treatment
ting, recalcitrant inflammation, with no real relief provided targeted at this window ultimately improve outcome?
by treatment; 1 of these patients ultimately required an Does a more prompt initiation of treatment reduce the
exenteration. rate of recurrence and treatment failure? Is there an op-
Systemic steroid therapy has traditionally been the timal course of steroid treatment or tapering for more dif-
mainstay of treatment for idiopathic orbital inflamma- ficult cases that would ultimately reduce the rate of re-
tion. However, many patients in our study, particularly currence?
those who ultimately failed to respond to treatment, had
unsatisfactory outcomes from systemic steroids. Re- COMMENT
bound of symptoms during steroid taper (ie, steroid de-
pendence) and adverse reaction to steroid therapy (ie, In our study, idiopathic orbital inflammation occurred more
steroid intolerance) occurred in 8 (33%) and 3 (13%), frequently in middle-aged women. The true incidence of

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 idiopathic orbital inflammation in the literature is diffi- failed to respond to the initial therapy. A benign, non-
cult to assess, given the wide spectrum of manifestations specific inflammatory pattern was the general finding in
and the lack of a universally accepted definition of the dis- our study, with the exception of 4 cases, which showed
ease entity. However, the peak incidence reported in the dense fibrous connective tissue as the predominating com-
literature appears to be predominantly in the adult popu- ponent with little inflammatory infiltrate (ie, the scle-
lation, typically in middle-aged persons, with pediatric cases rosing form). The dense collagenization seen in the scle-
accounting for only 6% to 17% of the total incidence.5,6 No rosing form is considered by some authors to be the end
strong sex predilection has been reported for idiopathic or- stage of the histological continuum of idiopathic orbital
bital inflammation. However, a 2:1 predominance in wom- inflammation, with the earlier stage characterized by
en,35 as well as a rare instance of familial occurrence,36 has greater lymphocytic component that then progresses to-
been reported for orbital myositis. In our study, an overall ward fibrosis in the later chronic stage.42,43 Others, how-
1.8:1 predominance in women was observed across the dif- ever, support the notion that idiopathic sclerosing in-
ferent subtypes; the highest predominance in women (5:1) flammation of the orbit is a unique clinicopathological
was observed for the orbital apex syndrome group. Dac- entity distinct from idiopathic orbital inflamma-
ryoadenitis and myositis were the most common sub- tion.33,44,45 In either case, the sclerosing form runs an in-
types observed in our study. sidious, frequently progressive course that replaces and
Our patients typically had unilateral periorbital pain damages orbital structures through cicatricial entrap-
and edema at the initial visit, which is consistent with ment. It tends to be more aggressive than the nonscle-
the clinical features reported in the literature. Other fea- rosing forms and appears to have a poor therapeutic out-
tures such as diplopia, redness, chemosis, and proptosis come, consistent with the findings in our study.
occurred less frequently in our study. Restriction, com- Systemic steroid therapy with a slow taper has been
pression, and destruction of orbital tissues occurred rarely the established first-line treatment, but refractory cases
in our study and only in patients with extensive sclero- accounted for a significant portion of treatment failures
sis and poor treatment outcomes. Medial rectus was the in our study. Steroid dependence and intolerance were
most commonly involved extraocular muscle in our study, prominent features of the patients who failed to re-
followed by superior rectus and lateral rectus; inferior spond to treatment. Steroid therapy, in general, hastens
rectus was the least frequently involved. In their studies clinical resolution in the acute phase. However, as ob-
of orbital myositis, Mombaerts and Koornneef27 and Si- served in our study, there are serious shortcomings to
atkowski et al37 reported that medial and lateral rectus steroid therapy. Although many patients with idio-
were most commonly involved. pathic orbital inflammation do have favorable re-
Radiological imaging studies allow tissue charac- sponses to steroid therapy, incomplete resolution is com-
terization and localization without surgical interven- mon. Steroid resistance and dependence as well as
tion and thereby have become invaluable diagnostic tools. potential adverse reactions, such as mood changes, hy-
Computed tomography is the preferred imaging mode perglycemia, dyspepsia, and weight gain, further com-
for idiopathic orbital inflammation and was by far the most plicate the clinical course and therapy. Mombaerts et
common imaging mode used in our study. Idiopathic or- al27,32,46,47 report a low cure rate (37%) and a high recur-
bital inflammation is typically seen on CT scans as a fo- rence rate (52%) obtained with steroid therapy in their
cal or diffuse mass, usually poorly demarcated and en- retrospective studies and question the value of steroids
hancing with contrast. Common CT findings include as the primary treatment modality. They propose that sys-
enhancement with contrast medium, infiltration of ret- temic steroid therapy not be used as the initial step but
robulbar fat, proptosis, extraocular muscle enlarge- be reserved as therapy in selected patients who have as-
ment, muscle tendon or sheath enlargement, apical fat sociated optic neuropathy or who may benefit from rapid
edema, optic nerve thickening, uveal-scleral thicken- though possibly transient symptomatic relief. They point
ing, edema of the Tenon capsule, and lacrimal gland in- out the need for a controlled prospective study that com-
filtration.33 Tendons of the extraocular muscles may be pares the efficacy of different therapeutic modalities. How-
involved or spared.38 Magnetic resonance imaging is gen- ever, until more information on the efficacy of these al-
erally used, either alone or in combination with CT, in ternative treatment modalities becomes available, systemic
patients with extraorbital or intracranial extensions. In- steroid therapy appears to be the best-accepted first-line
tracranial extension and bone destruction have been re- treatment for idiopathic orbital inflammation. Nonste-
ported but are rare findings, usually occurring with the roidal anti-inflammatory drugs, such as ibuprofen, may
sclerosing process.39,40 Echography may be useful as an be used as an alternative to or in combination with ste-
alternative if CT or MRI is not readily available.37 roid therapy. Mannor et al48 recommend a trial of non-
The histopathological spectrum of idiopathic or- steroidal anti-inflammatory drugs for up to 3 weeks or
bital inflammation is typically nondiagnostic, wide, and until clinical resolution, with steroids reserved for re-
diverse, ranging from the typical diffuse polymorphous fractory cases.
infiltrate to the atypical granulomatous inflammation, tis- Reports of alternatives to steroid therapy, such as
sue eosinophilia, and infiltrative sclerosis. In the ab- immunosuppressive chemotherapy, are fairly limited in
sence of systemic fibroinflammatory, granulomatous, and the idiopathic orbital inflammation literature. Pulsed che-
vasculitic disease, these atypical presentations are con- motherapy consisting of either cyclophosphamide or chlo-
sidered to be subclasses of idiopathic orbital inflamma- rambucil combined with prednisone has been reported
tion.41 Histopathological confirmation was sought in our to be effective in the treatment of idiopathic orbital in-
study for patients who had atypical presentations or who flammation refractory to both steroid and radiation

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 1. History and physical examination: 4. After the identifiable causes have been ment, which include increased soft tissue inflammation
• Similar episodes in the past? considered, initiate treatment for presumed that is generally self-limited. At approximately 20 Gy of
• Chronicity and duration of disease idiopathic orbital inflammation:
• Trauma, infection, systemic disorders • For mild clinical presentation:
irradiation, the only ocular structure with a significant
(including cancer), and Observation only or NSAIDs potential risk is the lens, because dry eye, retinopathy,
immunological compromise • For moderate to severe clinical and optic neuropathy are not generally observed with ra-
2. Consider infection and trauma: presentation: diation doses of less than 30 to 40 Gy.
• CBC and cultures Systemic prednisone 1.0 to 1.5 mg/kg
per day for 1 to 2 weeks with taper
Surgical resection may be an effective alternative to
• Radiologic evaluation of orbit and
sinus (typically CT) over 5 to 8 weeks medical and radiation therapy for more localized le-
• Trial of systemic antibodies Close monitoring of clinical sions.53 It is not a strong therapeutic option for diffuse,
progression and potential adverse fibrotic orbital lesions that cannot be removed because
3. Consider identifiable local and systemic
reaction to steroids
potential causes, according to clinical of the involvement of vital structures. The ultimate sur-
suspicion: 5. If there are persistent or recurrent episodes
that are refractory to systemic steroid
gical option of exenteration is indicated for idiopathic or-
• Thyroid orbitopathy
Thyroid function test therapy, strongly consider biopsy for bital inflammation in patients with irretrievable visual
CT scan of orbit definitive diagnosis loss accompanied by pain inadequately controlled by
• Sarcoidosis 6. If biopsy findings are consistent with medical or radiation therapy.
ACE, lysozyme, and chest idiopathic orbital inflammation, consider
Idiopathic orbital inflammation is a clinical diag-
radiograph the following for cases that are refractory
• Wegener granulomatosis to systemic steroids:
nosis of exclusion characterized by highly variable clini-
ANCA studies • If initially favorable response to the cal, radiological, and histopathological presentations. A
Pulmonary and renal function steroid therapy, restart systemic steroid stepwise algorithm is presented in Figure 5 to provide
evaluation with slower taper a more systematic approach to the diagnosis and treat-
• Neoplasm • If steroid intolerant, nonresponsive, or
CBC and systemic workup dependent, consider radiation therapy
ment of this disease.
MRI of orbit and brain Low-dose external beam irradiation, By definition, idiopathic orbital inflammation ex-
typically 1500 to 2000 cGy cludes lesions with identifiable local or systemic etiol-
fractionated over 10 days ogy. Differential diagnosis includes the following: infec-
7. If refractory to both medical and radiation tions, inflammatory reaction to trauma or a foreign body,
therapy, consider:
• Repeat work-up for other causes
thyroid dysfunction, vasculitis (such as Wegener granu-
• Chemotherapy lomatosis, polyarteritis nodosa, and giant cell arteritis),
• Surgical debulking if the lesion is easily sarcoidosis, neoplasm, and arteriovenous fistulas and mal-
accessible or if there is a severely formations. Because idiopathic orbital inflammation is
progressive and disabling clinical course
a diagnosis of exclusion, meticulous physical examina-
Figure 5. Algorithm for decision pathways in the diagnosis and treatment of tion and history taking are important initial steps. Par-
idiopathic orbital inflammation. ACE indicates angiotensin-converting ticular attention to the following is essential: previous simi-
enzyme; ANCA, antineutrophil cytoplasmic antibody; CBC, complete blood
count; CT, computed tomography; MRI, magnetic resonance imaging; and
lar episodes, antecedent trauma or infection, chronicity
NSAIDs, nonsteroidal anti-inflammatory drugs. and duration of symptoms, associated constitutional
symptoms, systemic disorders (including cancer), and risk
factors for potential immunological compromise.
therapy.28 Methotrexate and intravenous immunoglob- Infection must be considered. The orbit may be the
ulin have similarly been found to be effective in treating site of specific infections, most commonly by paranasal
idiopathic orbital inflammation that did not respond to sinusitis or contiguous spread from infections of the face,
steroids.29 The role of chemotherapy in the treatment of teeth, a penetrating foreign body in cases of trauma, and
idiopathic orbital inflammation remains to be further ex- septicemia. Infection most commonly begins in the eth-
plored. moid sinus, with extension of the inflammatory infil-
Radiation therapy was used in our study to treat pa- trate into the orbital space. In mild cases, the process may
tients with idiopathic orbital inflammation who were un- be limited to the preseptal space. In more severe cases,
responsive to steroid therapy, became steroid depen- the inflammatory infiltrate may extend into the extra-
dent, or had intolerable adverse reactions to steroids. conal space with subperiosteal involvement. Extension
Radiation therapy was used as the initial treatment in only into the intraconal space across the periorbita is rare but
1 atypical case in which Graves orbitopathy and atypi- does occur and requires very aggressive and prompt treat-
cal lymphoma were strongly considered in the differen- ment. It is important to be mindful of atypical micro-
tial diagnosis. Radiation therapy did not significantly affect organisms, including fungal infections such as mucor-
the treatment of idiopathic orbital inflammation in our mycosis and aspergillosis, in patients with diabetes and
study, because 8 of 9 patients who underwent radiation immunocompromised patients.10
therapy ultimately failed to respond to treatment. This Other potential causes must be evaluated and
is not surprising given that the patients who ultimately ruled out according to clinical suspicion. Thyroid orbi-
undergo radiation therapy tend to have more compli- topathy, also known as Graves orbitopathy, is the most
cated or atypical disease. However, several studies have common cause of exophthalmos and is an important
reported favorable outcomes with radiation therapy, in- consideration in the workup of patients with idiopathic
dicating that radiation therapy remains a viable treat- orbital inflammation. Thyroid orbitopathy has charac-
ment option for idiopathic orbital inflammation.49-52 Ra- teristic manifestations such as eyelid retraction, eyelid
diation therapy is an attractive alternative to steroid lag, proptosis, restrictive extraocular myopathy, and
therapy, particularly given the relatively few potential ad- optic neuropathy. Radiological findings include
verse effects of low-dose external-beam radiation treat- enlargement of extraocular muscles and an increase in

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 orbital fat volume. These findings may occur in the Neoplasm is also an important consideration in the
euthyroid setting in the absence of any objective thy- evaluation of idiopathic orbital inflammation, espe-
roid dysfunction as the initial presentation of the dis- cially in patients with a solitary orbital lesion. Imaging
ease process or after adequate control.54 A number of studies may show mass lesions, bony changes, and in-
clinical and radiological features distinguish idiopathic tracranial extension. In the adult population, metastatic
orbital inflammation from thyroid orbitopathy. 55 lesions and lymphoma are high in the differential diag-
Abrupt onset of pain and inflammatory signs, such as nosis, and systemic workup would be indicated. Breast,
periorbital erythema and swelling, are typical early lung, prostate, and kidney are common primary sites for
manifestations of idiopathic orbital inflammation. In metastastic orbital tumors. The possibility of chronic in-
contrast, Graves disease has a slower, more insidious flammatory reaction around a true neoplasm should also
course, and extraocular motility dysfunction and visual be considered.
disabilities tend to occur later in the disease process. In Differential diagnosis of pediatric idiopathic or-
addition, pain is not a prominent feature of Graves orbi- bital inflammation is somewhat different from that for
topathy. Radiological findings for idiopathic orbital the adult population and includes the following: orbital
inflammation are typically unilateral and may involve cellulitis, trauma, foreign body, dermoid and epider-
any of the orbital structures, including the extraocular moid cysts, capillary hemangioma, lymphangioma, and
muscles, muscle tendons, orbital fat, perineural connec- dacryoadenitis, as well as neoplasms such as rhabdo-
tive tissues, Tenon capsule, and sclera. Graves orbitopa- myosarcoma, retinoblastoma, and neuroblastoma and thy-
thy, in contrast, has findings that are typically bilateral, roid orbitopathy.17 The most serious consideration is neo-
and the primary focus is on the enlargement of extra- plasia, and a low threshold for biopsy is indicated.
ocular muscles and increased orbital fat volume. Rhabdomyosarcoma is the most common pediatric pri-
Wegener granulomatosis is a necrotizing, granulo- mary orbital malignancy and is commonly accompa-
matous inflammation and vasculitis that affects the res- nied by radiographically evident bony changes, which are
piratory and renal systems; there is ocular involvement very rare in idiopathic orbital inflammation. Retinoblas-
in about 50% of cases.10,56 Wegener granulomatosis is a toma is also an important consideration because it is the
rare disease but an important differential diagnosis for most common intraocular tumor of children, with 10%
idiopathic orbital inflammation because of its associa- of cases having orbital extension.17 Thyroid orbitopathy
tion with high morbidity and mortality. Bilateral eye is exceedingly rare in children and is much lower in the
pain, proptosis, redness, and ocular motility dysfunc- differential diagnosis than it is for idiopathic orbital in-
tion are common clinical features. Ocular and orbital flammation in an adult. Dermoid and epidermoid cysts
manifestations of Wegener granulomatosis include con- and capillary hemangioma are not uncommon orbital le-
junctivitis, marginal ulcerative keratitis, scleritis, uve- sions in newborns and infants. Ruptured dermoid cysts
itis, retinal vasculitis and optic neuropathy, dacryadeni- can provoke a significant granulomatous inflammatory
tis, and nasolacrimal duct obstruction. Histological reaction in the surrounding orbital tissue. Lymphan-
findings consist of necrotizing, granulomatous inflam- gioma is usually slow growing but can also have a ful-
mation and vasculitis. Serum levels of antineutrophil minant inflammatory presentation on rupture and hem-
cytoplasmic antibodies that display a cytoplasmic immu- orrhage.
nofluorescent staining pattern (c-ANCA) are elevated in After the local and systemic causes have been ruled
about 90% of patients with active Wegner granulomato- out, treatment for presumed idiopathic orbital inflam-
sis and are very helpful in diagnosis and differential mation can be initiated. In patients with a mild clinical
diagnosis. However, it is important to note that c-ANCA presentation, clinical progression may be monitored while
may not be a prominent feature of limited Wegener awaiting test results, or nonsteroidal anti-inflammatory
granulomatosis.56 Systemic workup including pulmo- drug therapy may be initiated. In patients with a mod-
nary and renal function assessment and radiological erate to severe clinical presentation and a strong possi-
evaluation would be indicated if Wegner granulomatosis bility of idiopathic orbital inflammation, systemic corti-
is suspected. costeroid therapy may be initiated as long as suspicion
Sarcoidosis is characterized by systemic granulo- for an infectious etiology is low and there is no other con-
matous inflammation involving the lungs, hilar lymph traindication for steroid therapy. Systemic prednisone can
nodes, eyes, and skin.10 Ocular involvement occurs in 25% be started with an initial dosage of 60 mg to 100 mg per
to 50% of patients and may include infiltration of the lac- day for 1 to 2 weeks and a taper typically over 5 to 6 weeks.
rimal gland, extraocular muscles, orbital fat, optic nerve, Clinical progression and potential adverse reactions need
and uveal tract. Conjunctival granuloma or solitary or- to be closely monitored once systemic corticosteroid
bital granuloma may also be found. Clinical features in- therapy has been initiated. In the event of persistent or
clude pain, proptosis, oculomotor dysfunction, uveitis, recurrent episodes that are refractory to systemic ste-
and vision loss. Radiological findings include bilateral hi- roid therapy, biopsy (open biopsy or, if applicable, fine
lar adenopathy and parenchymal pulmonary involve- needle aspiration) should be strongly considered for de-
ment. Noncaseating granulomas are the classic histo- finitive diagnosis, particularly if the lesion is easily ac-
logical finding. Angiotensin-converting enzyme and cessible (eg, lacrimal gland). If the biopsy findings are
lysozyme levels can be useful as adjuncts to clinical, ra- consistent with idiopathic orbital inflammation, con-
diological, and histological findings but not as primary sider the following options in patients with a disease
diagnostic tools because of their limited sensitivity and course that is refractory to systemic steroid therapy: (1)
specificity.57,58 if the patient had a favorable response to steroid therapy

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From the Archives of the ARCHIVES

A look at the past . . .

I t is now certain that accommodation does not affect

intraocular tension, so increased accommodative
efforts due to wearing the correcting glass for near can-
not tend to increase the myopia. This is rather due to
the pressure of the extrinsic muscles, whose deleterious
action is favored when objects are held close to the eyes.

Reference: Heine L. On the full correction of myopia.

Arch Ophthalmol. 1902;31:41.

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