MAJOR REVIEW
Idiopathic Sclerosing Orbital Inflammation: A Review of
Demographics, Clinical Presentation, Imaging, Pathology,
Treatment, and Outcome
John D. Pemberton, D.O.*†, and Aaron Fay, M.D.*†
*Department of Ophthalmology, Harvard Medical School; and †Ophthalmic Plastic and Reconstructive Surgery,
Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.
Purpose: To characterize clinical features, diagnostics stud-
ies, treatments, and outcomes of patients with histologically
proven idiopathic sclerosing orbital inflammation (ISOI), to
define optimal management for this recalcitrant disease, and to
determine changes in characterization and management by
comparing our results with the last significant literature review.
Methods: A search of the U.S. National Library of Medi-
cine: National Institutes of Health’s electronic database for
cases and case series in the English literature of biopsy-proven
ISOI published between March 1994 and September 2010 was
conducted. A cross-literature review was performed to tabulate
demographics, clinical findings, studies, treatments, and out-
comes, which were compared with the ISOI data published by
Rootman et al. (1994).
Results: Sixty-one cases, 71 eyes from 17 published re-
ports, met inclusion criteria. No ethnic, sex, or comorbidity
predilection was established. Patients typically presented in
the fourth decade with proptosis (73%), pain (49%), and
normal vision (44%). Orbital imaging and histopathology
were sparsely reported. Most common treatments involved
systemic corticosteroids either alone (34%) or combined
with other modalities (51%).
Conclusions: Characteristics of the disease remain un-
changed, and best management was not determined due to
inconsistent reporting methods across the literature. Collabora-
tion with established groups (i.e., European Group On Graves
Orbitopathy (EUGOGO), International Thyroid Eye Disease
Society (ITEDS)) or the formation of a new group of physi-
cians and scientists to help develop a systematic approach for
future reporting and evaluation was proposed.
(Ophthal Plast Reconstr Surg 2012;28:79–83)
P rior to Rootman’s 1994 article, idiopathic sclerosing orbital
inflammation (ISOI) was considered by most a variant of
the more common idiopathic orbital inflammation, more com-
monly called orbital pseudotumor at that time.1,2 His system-
atic review, the largest at the time, solidified the current
Accepted for publication September 16, 2011.
The authors have no financial or conflict of interest to disclose.
Address correspondence and reprint requests to Dr. Aaron Fay, M.D.,
Director, Ophthalmic Plastic and Reconstructive Surgery, Massachusetts
Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, U.S.A.
E-mail: aaron_fay@meei.harvard.edu
DOI: 10.1097/IOP.0b013e318238ecf7
Ophthal Plast Reconstr Surg, Vol. 28, No. 1, 2012
paradigm that ISOI is a unique clinicopathologic entity and that
it represents approximately 7.8% of all inflammatory lesions of
the orbit.1,2 Interestingly, no single or combined therapy was
found to be more effective than another.
ISOI is characterized clinically as an aggressive, insid-
ious, chronic, and progressive sclerosing process. Histologi-
cally, it is a dense fibrotic tissue with paucicellular infiltrate
consisting of lymphocytes (predominately T lymphocytes) as
well as a few eosinophils, histiocytes, and plasma cells.1,3,4
Although ISOI is thought to be limited to the orbit, extraorbital
extension and other associated fibrosclerosing disorders such as
retroperitoneal fibrosis, Riedel’s thyroiditis, mesenteritis, scle-
rosing cholangitis, and mediastinal fibrosis have been reported
in association with a similar orbital condition.4 –18 The inflam-
matory and immune process has not been well defined, and
treatment remains nonspecific. Since 1994, there have been 61
documented cases of biopsy-proven ISOI in 17 articles
reporting various treatment regimens and results.8 –24 We have
systematically reviewed those 17 articles to determine whether
significant changes in diagnosis and management have oc-
curred since 1994.
METHODS
The U.S. National Library of Medicine: National Institutes of
Health electronic database was searched for cases or series in the
English literature of ISOI published between March 1994 and Septem-
ber 2010. Search terms used were: Idiopathic Sclerosing Orbital In-
flammation, Idiopathic Sclerosing Pseudotumor, and Sclerosing Orbital
Inflammation. Only histologically diagnosed cases were included;
those with insufficient clinical documentation were excluded. Of these,
61 defined histologic criteria for inclusion. Data were extracted from
each article, and Excel spreadsheets were created to help in data
manipulation. Demographic fields included age, sex, ethnicity, lateral-
ity, and coexisting disease. Clinical fields included symptoms, signs,
treatment regimens, and outcomes. Ancillary data (radiography, histol-
ogy, immunohistochemistry) were tabulated as well. Due to inconsis-
tency between the published reports, we grouped treatment outcomes in
one of 3 categories: 1) Good: Resolution of symptoms and signs with
no reported recurrence; 2) Partial: Symptoms and signs improved but
not completely resolved, with or without recurrence; and 3) Poor: No
documented improvement with or without progression.
RESULTS
Seventy-five cases from 19 published reports were identified.8 –24
Thirteen cases reported by Swamy et al.25 in 2007 were excluded as the
demographic, clinical, and outcome data were mixed with an additional
11 cases of idiopathic, granulomatous, and vasculitic orbital pseudo-
tumor, making specific data extraction and evaluation difficult. The
Sharma et al.26 2006 case report was excluded because the diagnosis of
79
J. D. Pemberton and A. Fay Ophthal Plast Reconstr Surg, Vol. 28, No. 1, 2012

TABLE 1. Demographic data in 61 patients with ISOI TABLE 2. Vision, symptoms, and signs in 61 patients
Age
with ISOI
Mean 48 Vision (n ⫽ 71 (eyes), N/S 13)
Range 5 to 83 Range 20/20 to NLP
Sex male/female 33/28 20/20 31
Affected eye 20/30–20/60 16
Right 32 20/80–20/400 8
Left 19 Count fingers or worse 3
Bilateral 10
Ethnicity (7/61) Yes No N/S*
Caucasian 7 Symptoms
Black 1 Pain 30 8 23
Pacific Islander 1 Diplopia 21 2 38
Co-existing disease (7/61) Blurred vision 19 1 51
Dermatomyositis 1 Swelling 10 1 60
Nasopharyngeal carcinoma and COPD 1 Inflammation 8 1 62
Hemolytic anemia, diabetes, and HTN 1 Hypesthesia 3 68
Hypertension 2 Epiphora 1 1 69
Drug abuser 1 Signs
Retroperitoneal fibrosis and hypothyroidism 1 Proptosis 52 9 10
Not specified 54 Restricted ocular motility 39 21 11
COPD, chronic obstructive pulmonary disease; HTN, hypertension. Ptosis 20 16 35
Afferent pupillary defect 18 28 25
Color vision deficit 10 18 43
Lid edema/chemosis 7 64
sclerosing orbital inflammation was presumed from a previous abdom- Globe displacement 5 1 65
inal biopsy that demonstrated sclerosing mesenteritis. After exclusions, Lagophthalmos 1 70
61 cases of biopsy-proven idiopathic sclerosing orbital inflammation in Lid retraction 1 70
71 eyes from 17 published reports remained.8 –24 NLP, no light perception; N/S, not specified.
The demographic data for 61 patients with biopsy-proven ISOI
are presented in Table 1. There were approximately equal numbers of
males and females (33 males/28 females) with a mean age of 48 years
The most common quadrant of involvement was the lateral and/or
(standard deviation could not be calculated due to lack of detail in
superior (33/61). Extraorbital extension was documented in 15 cases,
original publications). The disease was predominately unilateral (51/
with 38 periorbital locations, with the most common being the infra-
61), and the right eye was affected (32/51) most commonly. Ethnicity
temporal fossa (7/38). There were also 3 cases of bony erosion through
and comorbidities were scarcely reported.
the greater wing of the sphenoid.
Presenting vision, symptoms, and signs are shown in Table 2.
The pathology and immunohistochemical analyses are pre-
Vision measured from 20/20 to no light perception with normal vision
sented in Table 4. All 61 cases were biopsy-proven. Symptom to biopsy
(20/20) being frequently reported (31/71, 44%); 66% were 20/60 or
time was documented in 16/61 cases and ranged from 2 weeks to 24
better. The most common complaint was pain (30/61, 49%), and the
months (mean 8 months, standard deviation 7.7 months). The pathol-
severity was defined in 17 of the 30 cases (57%): severe (9/17, 53%),
moderate (4/17, 24%), and mild (4/17, 24%). Patients also reported
diplopia (21/61, 34%), blurred vision (19/71, 27%), and swelling
(10/71, 14%). The most common physical sign was proptosis (52/71, TABLE 3. Radiographic findings, orbital location, and
73%); reported measurements (11/52, 21%) ranged from 2 mm to 7 mm extraorbital extension in 61 patients with biopsy
(mean 3.82 mm, standard deviation 1.41 mm). Hsuan et al.19 docu- proven ISOI
mented proptosis in 26/31 cases (84%) with a range from 1 mm to 10
Location of lesion
mm (mean 3.1 mm). (Relative proptosis was reported, even in bilateral Anterior orbit 26/61
cases, making the absolute axial displacement difficult to ascertain.) Lacrimal 15/61
Cases of ocular motility restriction (39/71, 55%) were also common, Mid-orbit (intra/extra-conal) 34/61
and the patterns, although sparsely reported (14/71, 20%), were hori- Apical/posterior orbit 7/61
zontal (5/14, 36%), vertical (2/14, 14%), and both (7/14, 50%). Globe Ocular muscle 24/61
displacement (6/71, 8%) was superior (2/6, 33%), medial (1/6, 17%), Diffuse 19/61
inferomedial (1/6, 17%), and inferior (1/6, 17%). Extra-orbital extension (15 cases, 38 locations)
Radiographic findings, orbital location, and extraorbital exten- Infratemporal fossa 7
Intracranial 5
sion are presented in Table 3. It is striking that few of the publications
Pterygopalatine fossa 4
described imaging characteristics in detail. There were 17 orbital MRI Cavernous sinus 4
studies and 58 CTs done in 71 eyes of 61 patients. The lesion was Infra-orbital fissure 3
described most commonly as homogeneous and diffuse with ill-defined Maxillary sinus 3
borders in 7/17 MRIs and 10/58 CTs. Each report used a unique system Preseptal 3
to report the locations of lesions. We created 6 location categories Infra-orbital canal 2
(Table 3). Most common locations were anterior and lacrimal (41/61), Sphenoid sinus 2
midorbit (34/61), and the posterior orbit and extraocular muscles Frontal sinus 1
(31/61). The extraocular muscles involved were superior and lateral Ethmoid sinus 1
Nasal cavity 1
recti (10/24), medial and inferior recti (5/24), lateral and inferior recti
Superior orbital fissure 1
(2/24), superior oblique, medial, and superior recti (1/24), superior Nasolacrimal duct 1
rectus and levator (1/24), lateral rectus (1/24), and not specified (4/24).

80 © 2012 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 28, No. 1, 2012 Review of Idiopathic Sclerosing Orbital Inflammation

TABLE 4. Pathologic and immunohistochemical data TABLE 6. Steroids only versus Steroids plus* additional
in 61 patients with biopsy proven ISOI therapy in patients with biopsy proven ISOI
Biopsy-proven ISOI 61 Range Mean Mode
Symptoms until biopsy (16 cases, 45 N/S)
Range 2 weeks to 24 months Symptoms to tx. (months)
Mean 8 months Steroids only (20 pts) 1 to 48 7 4
Mode 4 months Steroids plus (27 pts) 1 to 240 20 4
Pathologic description Dosage (mg)
Sclerosis 49 (12 N/S) Steroids only (21 pts) 30 to 150 59 60
Lymphocytes 45 (16 N/S) Steroids plus (22 pts) 25 to 100 55 60
Paucity of cellular infiltrate 36 (25 N/S) Duration of tx. (months)
Plasma cells 28 (33 N/S) Steroids only (18 pts) 1 to 144 12 1
Eosinophils 21 (40 N/S) Steroids plus (22 pts) 1 to 120 9 2
Histiocytes 17 (44 N/S) Taper length (months)
Neutrophils 11 (50 N/S) Steroids only (2 pts) 1 to 4 2.5 N/A
Lymphoplasmacytic 5 (56 N/S) Steroids plus (4 pts) 1.5 to 3 2.1 2
Giant cells 4 (57 N/S) Good Partial Poor
Immunohistochemistry (5 cases, 6 N/S,
50 N/D) Outcome
CD 3⫹ 4 Steroids only (21 pts) 9 5 7
CD 20⫹ 5 Steroids plus (31 pts, 1 N/S) 8 18 5
CD 45⫹ 1
CD 68 ⫹ 1 *Steroid plus, steroids plus either surgery, radiation, chemotherapy, biologic
IgG4⫹ 1 agents or combination of these; pts, patients; tx, treatment; N/A, not applicable. Good:
Symptoms and signs resolved, no recurrence. Partial: Symptoms and signs improved
N/S, not specified; N/D, not done. but not resolved or recurred. Poor: No documented change or progression.

ogy described sclerosis (49/61), lymphocytes (45/61) (in 5/61 cases,

predominantly T cells), and paucity of inflammatory infiltrate (36/61). trexate, and infliximab (1/61); steroids, intralesional steroids, ritux-
Immunohistochemistry was rarely performed (11/61) and rarely imab, and CyberKnife radiation (1/61); and steroids, radiation, and
reported (5/11). cyclophosphamide (1/61). In a review of all cases in which steroids
Treatments and outcomes (Table 5) were evaluated to define the were used (53/61), symptoms onset to initiation of therapy was docu-
therapeutic modalities for the management of idiopathic sclerosing mented (47/53) and not documented (6/53). The range was 3 days to
orbital inflammation. There were 15 different treatment regimens: 240 months (mean 20.2 months). Dosage was documented in 43/53
steroids only (21/61); steroids and surgery (8/61); steroids and radiation cases, and the range was 25 to 150 mg per day (mean of 54.5 mg).
(6/61); steroids, radiation, and surgery (4/61); no treatment (4/61); Duration of therapy was documented in 40/61 cases; duration ranged
surgery only (3/61); steroids and azathioprine (3/61); steroids, radia- from 3 days to 144 months (mean 10.6 months). Taper was documented
tion, and cyclosporin (2/61); intralesional steroids only (1/61); steroids in 6/61 cases; taper ranged from 1 to 4 months (mean 2.3 months).
and cyclophosphamide (2/61); steroids and intralesional steroids (1/ Patients who received only systemic steroids were compared with all
61); steroids, cyclophosphamide, and cyclosporin (1/61); steroids, cases in which systemic steroids were used in addition to other
surgery, and cyclophosphamide (1/61); steroids, azathioprine, metho- therapeutic modalities (Table 6). The systemic steroid only group
(21/61) reported symptoms to treatment from 3 days to 48 months
(mean 7 months). Steroid dosage ranged from 30 to 150 mg (mean 59
TABLE 5. Treatments and outcomes of 61 patients mg) with duration of therapy from 1 to 144 months (mean 12 months).
with biopsy proven ISOI (n ⫽ 56 patients [5 N/S]) The steroid plus other modalities group (32/61) reported symptoms to
treatment from 1 month to 240 months (mean 20 months). Steroid
Good Partial Poor dosage ranged from 25 to 100 mg (mean 55 mg) with duration from 3
Only steroid 9 5 7 days to 120 months (mean 9 months). Tapering schedule was rarely
Only intralesional steroids 1 reported for either group. Treatment outcome for the steroid only group
Only surgery 1 1 1 was good (9/21), partial (5/21), and poor (7/21). The outcome for the
Steroid plus additional therapy(s) 8 18 5 steroid plus other modalities group was good (8/32), partial (18/32),
Steroid plus, breakdown (31 pts) and poor (5/32). The overall response, regardless of treatment regimen,
Surgery 3 5 was good (19/61), partial (24/61), and poor (13/61). Thirteen adverse
Radiation 5 1
side effects were reported among 8 patients. Five of the 8 cases were
Radiation and surgery 1 3
Azathioprine 2 1 on systemic steroids with reported side effects of weight gain (3/8);
Radiation and cyclosporin 2 psychosis, osteoporosis, heart failure, hypertension, or cataracts (2/8);
Cyclophosphamide 1 1 and (1/8) euphoria. The remaining 3 cases reported neutropenia with
Intralesional steroids 1 cyclophosphamide, altered taste with azathioprine, and dry eye from
Cyclophosphamide and cyclosporin 1 radiation therapy. Length of follow-up data was determined for 11 case
Surgery and cyclophosphamide 1 reports and averaged 21.6 months with 6 having no recurrence, 3
Azathioprine, methotrexate, and infliximab 1 having improvement but still with persistent disease, and 2 progressing
Rituximab, intralesional, and CyberKnife 1
despite therapy. The Chen et al.20 report of 9 patients had an average
Radiation and cyclophosphamide 1
follow-up of 32.4 months with 6 having no recurrence, 3 having poor
N/S, not specified; pts, patients. Good: Symptoms and signs resolved, no response; however, whether this was persistent disease versus recur-
recurrence. Partial: Symptoms and signs improved but not resolved and/or rence was not clarified. The Hsuan et al.19 report of 31 patients had an
recurrence. Poor: No documented improvement and/or there was progression.
average follow-up of 29 months with recurrence not being specified.

© 2012 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. 81
J. D. Pemberton and A. Fay Ophthal Plast Reconstr Surg, Vol. 28, No. 1, 2012

TABLE 7. Comparison of authors’ results with Rootman et al.1

Authors’ results (61 pts) Rootman (16 pts)
Age: Range (mean) 5–83 (mean 48) 8 to 81 (mean 42)
Sex: Male/female 33M/28F 11M/5F
Affected eye 32 R, 19 L, 10 b/l 5 R, 9 L, 2 b/l
Vision 71 eyes, N/S 13 18 eyes
20/20 31 (44%) 7 (39%)
20/25–20/60 16 (22%) 10 (56%)
20/200–20/400 8 (11%) 0
Count fingers or worse 3 (4%) 1 (5%)
Length of symptoms to biopsy 8 months 24 months
Symptoms and signs (%)
Pain 51% 81%
Diplopia 34% 50%
Blurred vision 31% 13%
Swelling 16% 56%
Inflammation 13% 69%
Epiphora 2% 25%
Proptosis 72% 69%
Restricted ocular motility 55% 56%
Ptosis 28% 31%
Lagophthalmos 1% 6%
Radiographic lesion location
Anterior orbit/lacrimal 67% Anterior 75%
Mid-orbit(intra/extra-conal) 56% Midplane 56%
Apical/posterior orbit/ocular muscle 51% Posterior 56%
Most common quadrant Superior and lateral Superior and lateral
Most common description Homogeneous, ill-defined border Homogeneous, ill-defined borders
N/S, not specified; b/l, bilateral; pts, patients.

DISCUSSION IgG4-positive orbital inflammation may be either a distinct

Idiopathic sclerosing orbital inflammation was thought
by many to be a continuum of nonspecific orbital inflammation
that followed a linear progression from acute to chronic with
fibrosis representing end stage disease.27–29 Rootman and oth-
ers1,2,30,31 theorize that ISOI is a distinct entity that requires
special attention and aggressive management. They feel that the
disease is one potential presentation of disease under the
heading idiopathic multifocal fibrosclerosis, which typically
includes retroperitoneal fibrosis, sclerosing cholangitis, medi-
astinal fibrosis, and Riedel’s thyroiditis.1– 8,30,31 Rootman et
al.1 demonstrated in 1994 the histopathological similarities
between ISOI and retroperitoneal fibrosis and concluded that
they are manifestations of a single disease.4
The incidence of ISOI is unclear from published litera-
ture, but Rootman et al.1 reported that ISOI accounted for 7.8%
of all inflammatory orbital lesions and 1.4% of all orbital
masses at his institution from 1976 to 1991. We compared our
results with the case series findings reported in 1994 by Root-
man et al.1 (Table 7). The results were very similar in most
regards; however, the length of symptoms to biopsy was 33%
shorter for our series, suggesting that surgeons have been
biopsying sooner since Rootman published his case series.
Rootman’s description of the histopathology was similar to our
results as we both noted this entity to have sclerosis with
paucicellular chronic infiltrate of lymphocytes (T cells being
more common than B cells), plasma cells, histiocytes, and a
few neutrophils and eosinophils. One of our cases published by
Mehta et al.10 reported a patient having immunoglobulin G4-
(IgG4)-positive plasma cells that have been reported in other
IgG4-related fibrosclerosing disorders such as sclerosing
cholangitis and retroperitoneal fibrosis. Because none of the
other cases in our review or in Rootman’s series looked for this
finding, it is difficult to determine the role IgG4 has in ISOI.
entity (in which those cases would be culled from the ISOI
pool) or an additional defining characteristic of ISOI. In either
case, defining the role of IgG4 testing may prove beneficial to
focusing our management; Khosroshahi et al.32 reported in
June 2010 that Rituximab therapy led to a rapid decline of
serum IgG4 levels and prompt clinical improvement in IgG4-
related systemic disease.
The myriad treatment regimens, limited reports, nonuni-
form reporting of clinical outcomes, and overwhelming use of
steroids in the majority of cases made determining optimal
management impossible. Rootman1 also found that he could
not endorse one approach over another in his series. Brannan33
also reported in his brief 2007 review that a consistent, effec-
tive treatment course is lacking. Newer treatments have been
reported since 1994 (and were included in this review). Inflix-
imab was used in one case but was administered along with
methotrexate and systemic steroid and had incomplete resolu-
tion of findings.21 Another case, also in our review, reported
“successful” use of CyberKnife radiosurgery, but it was used in
conjunction with systemic steroids and rituximab, also without
complete resolution.22 In both of these cases, the technology
was new for the treatment of ISOI, but the effect could not
be isolated, and outcomes were suboptimal. Ultimately,
organized treatment protocols will be needed to determine
optimal management.
It is disheartening to think that we have gained little
ground in trying to define and manage ISOI. For over 2
decades, idiopathic sclerosing orbital inflammation has been
recognized as a distinct entity with unique clinical features that
needed further evaluation and more focused diagnostic strate-
gies and therapeutic regimens.1,2,30,31 However, this current
review summarizing all cases since 1994 offers little insight in

82 © 2012 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 28, No. 1, 2012
pathogenesis, diagnostic studies, and management of this pro-
gressive and destructive disease.
Strategies to further the evaluation, management, and
reporting of idiopathic sclerosing orbital inflammation are
needed. Many have suggested prospective randomized trials,
yet we have not produced one, and the infrequency of the
disease makes it unlikely still. We propose a multidisciplinary
consortium of clinical and research scientists with a common
interest in idiopathic sclerosing orbital inflammation to de-
velop, disseminate, and standardize clinical criteria, modeled
after the 2 major thyroid eye disease groups, the European
Group On Graves Orbitopathy (EUGOGO) and the Interna-
tional Thyroid Eye Disease Society (ITEDS). Another option
would be to combine efforts with these well established groups.
EUGOGO comprises endocrinologists, ophthalmologists, epi-
demiologists, and radiologists working to standardize clinical
assessment reporting to improve the management of patients
with Graves orbitopathy. ISOI, too, requires uniform documen-
tation of initial presentation, subsequent follow-ups, and
therapeutic outcomes, possibly using the ITEDS Vision Inflam-
mation Strabismus Appearance form (VISA) as a model.
ITEDS is a nonprofit society of clinicians and researchers who
are improving our diagnostic and management knowledge of
thyroid eye disease. That society has a patient encounter doc-
ument, the VISA form, which has standardized reporting of
both subjective and objective data for initial and subsequent
examinations. Using these 2 examples as guides, objective
clinical documentation criteria, diagnostic protocols, treatment
regimens, and therapeutic outcome measures may be the best
next step toward understanding and conquering ISOI.
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