1 / 25

Idiopathic Orbital Inflammation with Bone Erosion and Extension into Paranasal Sinuses

This case study discusses a middle-aged woman with symptoms of orbital inflammation, including tearing, crusty discharge, photophobia, and diplopia. Imaging revealed a mass with bone erosion and extension into the paranasal sinuses. Biopsies confirmed idiopathic orbital inflammation. Treatment options and the potential for bone involvement are addressed.

awm
Download Presentation

Idiopathic Orbital Inflammation with Bone Erosion and Extension into Paranasal Sinuses

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. 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

  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

  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

  4. 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

  5. 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

  6. CD3 CD20 CD138 Lambda Kappa

  7. IgG4 IgG

  8. 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

  9. 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

  10. 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

  11. 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

  12. 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 • Predominantly in diffuse IOI

  13. 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

  14. Inflammatory Osteolysis From: G Mbalaviele, et al., Inflammatory osteolysis: a conspiracy against bone, J Clin Invest 127:2030-2039, 2017 Green = pro-osteoclast factors and red = anti-osteoclast factors

More Related