1. Eastern Ophthalmic Pathology Society September 13-15, 2018
Idiopathic Orbital Inflammation (Inflammatory Orbital Pseudotumor) with Bone Erosion and Extension into the Paranasal Sinuses
Alan D. Proia, M.D., Ph.D.
Eastern Ophthalmic Pathology Society September 13-15, 2018
Proia/DUMC

2. Clinical History
Woman in middle 30s with four month history of gradually worsening:
Tearing, crusty discharge, itching, and photophobia in her right eye
Difficulty focusing and eye strain after closing and then reopening her eyes
Diplopia
Pain in right eye during up, down, left, and right gaze
Protrusion of right eye
One month history of nausea when looking at objects close to her face
Proia/DUMC

3. Examination Proptosis O.D.
Exophthalmometry: 23 mm O.D. and 20 mm O.S.
Intraocular pressure 23 mmHg O.D. and 19 mmHg O.S.
Full extraocular movements but pain in right eye during left, right, up, and down movement
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5. Computed Tomography
2.8 x 1.9 x 3.2 cm mass involving the intraconal and extraconal nasal right orbit and extending to the orbital apex
Anterior displacement of the globe
Mass abutting the globe with loss of fat margin; effacement of the medial rectus muscle, portions of the fat plane, and the superior oblique muscle
Erosion of the mass through the orbital floor into the superior maxillary sinus and into the ethmoid sinus through the lamina papyracea
Proia/DUMC

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7. Surgery and Follow-up Anterior orbitotomy with biopsy
1.0 x 0.9 x 0.3 cm aggregate of tissue
Dense fibrous connective tissue with numerous lymphocytes and macrophages; rare cells expressing cytokeratins
One month later – anterior orbitotomy with biopsy of orbit and right ethmoid and maxillary sinuses
Diagnosis: Idiopathic orbital inflammation/idiopathic inflammatory pseudotumor
Proia/DUMC

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17. CD3
CD20
CD138
Kappa
Lambda
Proia/DUMC

18. IgG
IgG4
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19. Idiopathic Orbital Inflammation
Enlarged structure or mass in the orbit that is of unknown cause and is manifest histologically by nonspecific inflammation with varying degrees of fibrosis
Other names: orbital pseudotumor, idiopathic orbital pseudotumor, inflammatory orbital pseudotumor, idiopathic orbital inflammatory syndrome, non-specific orbital inflammation
Proia/DUMC

20. Idiopathic Orbital Inflammation
Incidence
11% of lesions at Wills Eye Hospital Philadelphia, PA
8.5% at Erasmus University Medical Center, Rotterdam, Netherlands
5.2% at The University of Texas M.D. Anderson Cancer Center, Houston, TX
Classified by anatomic distribution
Mombaerts: dacryoadenitis, myositis, diffuse (several structures involved), or perineuritis
Proia/DUMC

21. Idiopathic Orbital Inflammation
All age groups
Slight predominance in male gender
Most common symptoms and signs
Pain, diplopia, decreased vision, periorbital swelling, proptosis, restricted ocular motility
Histology
Nonspecific chronic inflammatory infiltrate of lymphocytes, plasma cells, histiocytes, eosinophils with degranulation, sometimes neutrophils
Proia/DUMC

22. Idiopathic Orbital Inflammation
Histology, continued
Eosinophils may be prominent in children
Lymphoid follicles with germinal centers may be present, most common in chronic stage of disease
Degree of fibrosis is variable
T-lymphocytes predominate over B-lymphocytes
Polyclonal
Variants: Granulomatous IOI and idiopathic sclerosing orbital inflammation
Proia/DUMC

23. Idiopathic Orbital Inflammation
Extraorbital extension
Bone erosion: 16 prior reports
Predominantly in diffuse IOI
Dacryoadenitis and myositis more common in patients without bone erosion
Through fissures and foramina without eroding through bone: 32 reported cases
Clifton and coworkers found extraorbital extension in CT scans of 8/90 (9%) of patients with biopsy confirmed IOI
Proia/DUMC

24. Extraorbital Extension
Conclusion from review of the literature
Not possible to predict a priori which patient with diffuse IOI will exhibit bone erosion
Treatment similar to IOI without extension
Corticosteroids
Radiotherapy, immunosuppressive drugs, and/or surgical debulking in non-responsive IOI
Proia/DUMC

25. Inflammatory Osteolysis
From: G Mbalaviele, et al., Inflammatory osteolysis: a conspiracy against bone, J Clin Invest 127: , 2017
Green = pro-osteoclast factors and red = anti-osteoclast factors
Proia/DUMC