Grand Rounds
Shivani V. Reddy, M.D.
University of Louisville
Department of Ophthalmology and Visual Sciences
Patient Presentation

CC: Left Eye Pain

HPI: 31 y/o WF presents to the ER with 5 days of
pain/pressure OS . She describes the pain as 8/10, deep
and stabbing in quality with gradual worsening over the 5
day period. Denies blurry vision, photophobia or foreign
body sensation.
History
POHx: episode of OD pain 7 months prior
- CT orbits with OD superior rectus, lateral rectus and
lacrimal gland enlargement
- resolved with Prednisone 60 mg PO Q.day x 2 weeks
myopia
PMHx: migraines, anxiety
FAMHx: no known thyroid or autoimmune diseases
ROS: URI which she recovered from 3 weeks prior
MEDS: benadryl, protonix, flexaril, depakote
ALLERGIES: lortab, toradol, sulfa antibiotics
Exam
8
20/20
VA
TP
20/20-2
12
4→3
P
4→3
no RAPD
EOM: -1 restriction in all gazes with pain OS
no diplopia
MRD 1: 4mm OU
no lid lag
no proptosis OS
Exam
OD
OS
LIDS/LASHES
WNL
WNL
CONJ
WNL
WNL
CORNEA
WNL, no staining
WNL, no staining
IRIS
WNL
WNL
LENS
WNL
WNL
FUNDUS EXAM:
c/d: 0.3 with sharp rim OU
MVP wnl OU
PHYSICAL EXAM: no
cervical/submandibular
LAD
CT SCAN
Enlargement of superior oblique muscle OS, no lacrimal gland involvement
Summary
32 y/o WF presents with 5 days of OS pain worsened on EOM
with minor movement restriction in all gazes. Ant segment and
fundus exam WNL. CT scan shows swelling of superior oblique
muscle. She had a similar episode OD previously that resolved upon
treatment with corticosteroids
DDx:
 Thyroid Eye Disease
 Autoimmune Disease
 Orbital malignancy
 Infectious (orbital cellulitis)
 NSOI
Laboratory Workup
- ESR
- CRP
- CBC
WNL
WNL
WNL
ANA negative
ACE WNL
Thyroid Function Tests
- Free T4 WNL
- T3
WNL
- TSH
WNL
- TSI
negative
- T-Perox negative
Summary
32 y/o WF presents with 5 days of OS pain worsened on EOM with
minor movement restriction in all gazes. Ant segment and fundus exam
WNL. CT scan shows swelling of superior oblique muscle. She had a
similar episode OD previously that resolved upon treatment with
corticosteroids.
Negative Workup
DDx:
 Thyroid Eye Disease
 Autoimmune Disease
 Orbital malignancy
 Infectious (orbital cellulitis)
 NSOI
Treatment
 Started on oral Prednisone 1mg/kg with ranitidine
3 day follow-up
 Pain and EOM restriction resolved
 Started on slow taper
 No recurrences as of 2 weeks ago per telephone
follow-up
Nonspecific Orbital Inflammation
(NSOI)
 Also known as:
 Inflammatory orbital pseudotumor
 Idiopathic orbital inflammatory syndrome
 Benign process characterized by polymorphous lymphoid
infiltrate +/- fibrosis of varying degrees
 No known local or systemic cause
 Diagnosis of exclusion
 Controversial pathogenesis, likely cell mediated
NSOI
 typically unilateral in adults, but upto 1/3 bilateral in
 children
 5 main locations in order of frequency:
 Lacrimal gland (darcryoadenitis)
 Extraocular muscles (myositis)
 50% with tendon involvement
 Anterior orbit
 +/- tenons involvement (ring sign)
 Orbital apex
 Diffuse
 Sclerosing subtype with marked orbital fibrosis
NSOI
 Variable presentation depending on location
 Most typical feature is deep-rooted boring retro-orbital
pain
 Other common features
 EOM restriction +/- pain
 Proptosis
 Conjunctival Inflammation
 Chemosis
 Upper eyelid erythema
 Children commonly present with uvietis, disc edema
and eosinophilia
NSOI
Lab findings:
 Elevated ESR
 CBC with eosinophilia
 + ANA levels
 Mild CSF pleocytosis
 Histological Findings
 Pleomorphic cellular infiltrate with lymphocytes,
plasma cells and eosinophils, later stages with
fibrotic changes
 Sclerosing subtype shows very little inflammation
NSOI
Dacryoadenitis with marked inflammation and expansion along the
lateral orbital wall. Diffuse gland enlargement with blurring of margins
NSOI
Extraocular muscle inflammation with tubular enlargement 2/2 tendon involvement
medial rectus > superior muscle complex > lateral rectus > inferior rectus
NSOI
Diffuse orbital involvement showing fat enhancement (asterix’s)
Diagnosis
 Based on a combination of clinical symptoms, labs and imaging
 Biopsy if - diagnosis uncertain, atypical presentation, poor response to initial
medical treatment
Treatment
Mild cases
 Observation
 NSAIDS + PPI
Moderate - Severe Cases
 Corticosteroids are mainstay of therapy at 1mg/kg
dosing
 Slow taper to ensure complete suppression of
inflammation
Refractory Cases & Sclerosing Variant
 Immunomodulator therapy
 Cyclosporine, cyclophosphamide, methotrexate
 Low dose radiation
Response/Prognosis
 78% with +ve initial response BUT only 37% cured , 52%
disease recurrence
 Patients with optic neuropathy 2/2 compression showed 95%
response rate
 Sclerosing subtype tends to show less of a treatment response
 Per 2007 review of 56 published biopsy proven NSOI cases
 34% have complete resolution
 43% with partial resolution
 23% refractory
Ophthal Plast Reconstr Surg 2013;29:286–289)
• Prospective, noncomparitive interventional case series
• 47 patients with acute idiopathic orbital inflammation
• Dacryoadenitis – 31
• Myositis – 12
• Diffuse – 4 cases
• Patients injected with 2-4 ml betamethasone suspension through a 22
gauge needle into the inflamed gland, around the inflamed muscle and
periocularly in diffuse cases
• After injection, NSAIDS + topical steroid treatment for 2 weeks
•F/U was weekly x 1 month, every 3 months x 1 year, then yearly
• Dacryoadenitis – 31 cases (4 recurrent)
• 25 cases 2ml suspension, 6 cases 4ml suspension
• Mean age 26.4 years , F>M (24:7)
• Complete response - 1-2 weeks
• No recurrences/complications
• Myositis - 12 cases (1 recurrent)
• 2ml suspension
• Mean age 27.4 years , M>F (9:3)
• Complete response - 1-2 weeks
• 1 recurrence 14 months post with LR inflammation- resolved after inj#2
• No other recurrences/complications
• Diffuse Inflammation – 4 cases (2 recurrent)
• 4 ml suspension
• Mean age 29.2 years, all men
• Complete response - 1-4 weeks
• 1 recurrence 9 months post , resolved after inj #2
• No other recurrences/complications
THANK YOU
References
 BCSC Section 4. Ophhtalmic Pathology and Intraocular tumors
 BCSC Section 8. Orbit, Eyelids and Lacrimal System
 Ding ZX, Lip G, Chong V. Idiopathic orbital pseudotumor. Clinical Radiology
2011;66:886-892
 Kapur R, Sepahdari AR, Mafee MF, et al. MR imaging of orbital inflammatory
syndrome, orbital cellulitis, and orbital lymphoid lesions: the role of diffusionweighted imaging. AJNR Am J Neuroradiol 2009;30:64-70
 Mombaerts I, Schingmann RO, Goldschmeding R, et al. Are systemic
corticosteroids useful in the management of orbital pseudotumors? Ophthalmol.
1996;103:521-528
 Ahn Yuen SJ, Rubin PAD. Idiopathic Orbital Inflammation Distribution, Clinical
Features, and Treatment Outcome. Arch Ophthalmol. 2003;121:491-499
 Swamy BN, McCluskey P, Nemet A, Crouch R, Martin P, Benger R, Ghabriel R,
Wakefield D. Idiopathic orbital inflammatory syndrome: Clinical features and
treatment outcomes. Br J Ophthalmol 2007;91:1667-1670