Grand Rounds

1
Grand Rounds
Shivani V. Reddy, M.D. University of
Louisville Department of Ophthalmology and Visual
Sciences
2
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.

3
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
4
Exam
VA TP
P
no RAPD
EOM -1 restriction in all gazes with pain OS
no diplopia MRD 1 4mm OU no lid
lag no proptosis OS

5
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
6
CT SCAN
Enlargement of superior oblique muscle OS, no
lacrimal gland involvement
7
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

8
Laboratory Workup

• ESR WNL ANA
  negative
• CRP WNL ACE
  WNL
• CBC WNL
• Thyroid Function Tests
• Free T4 WNL
• T3 WNL
• TSH WNL
• TSI negative
• T-Perox negative

9
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

10
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

11
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
  

12
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

13
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

14
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

15
NSOI
Dacryoadenitis with marked inflammation and
expansion along the lateral orbital wall. Diffuse
gland enlargement with blurring of margins
16
NSOI
Extraocular muscle inflammation with tubular
enlargement 2/2 tendon involvement medial
rectus gt superior muscle complex gt lateral rectus
gt inferior rectus
17
NSOI
Diffuse orbital involvement showing fat
enhancement (asterixs)
18
Diagnosis

• Based on a combination of clinical symptoms, labs
  and imaging
• Biopsy if - diagnosis uncertain, atypical
  presentation, poor response to initial medical
  treatment

19
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

20
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

21
Ophthal Plast Reconstr Surg 201329286289)

• 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

22

• Dacryoadenitis 31 cases (4 recurrent)
• 25 cases 2ml suspension, 6 cases 4ml suspension
• Mean age 26.4 years , FgtM (247)
• Complete response - 1-2 weeks
• No recurrences/complications
• Myositis - 12 cases (1 recurrent)
• 2ml suspension
• Mean age 27.4 years , MgtF (93)
• Complete response - 1-2 weeks
• 1 recurrence 14 months post with LR
  inflammation- resolved after inj2
• 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

23
THANK YOU
24
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 201166886-892
• Kapur R, Sepahdari AR, Mafee MF, et al. MR
  imaging of orbital inflammatory syndrome, orbital
  cellulitis, and orbital lymphoid lesions the
  role of diffusion-weighted imaging. AJNR Am J
  Neuroradiol 20093064-70
• Mombaerts I, Schingmann RO, Goldschmeding R, et
  al. Are systemic corticosteroids useful in the
  management of orbital pseudotumors? Ophthalmol.
  1996103521-528
• Ahn Yuen SJ, Rubin PAD. Idiopathic Orbital
  Inflammation Distribution, Clinical Features, and
  Treatment Outcome. Arch Ophthalmol.
  2003121491-499
• 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
  2007911667-1670