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Idiopathic Orbital Inflammation (Pseudotumor)
H. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Idiopathic orbital inflammation (IOI)
              • Synonyms

                • Orbital pseudotumor (or simply "pseudotumor")
              • Definitions

                • Nonspecific orbital inflammation, often without known etiology or systemic illness
                • Tolosa-Hunt
                  • Variant apical form extending into cavernous sinus
                • Sclerosing orbital inflammatory pseudotumor
                  • Variant form with chronic progressive fibrosis
                • IgG4-related disease (IgG4-RD)
                  • Variant form, recently identified, with plasma cell-mediated inflammation
                  • Accounts for minority of IOI
                  • May be classified as distinct entity

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. McNab AA et al: IgG4-Related Ophthalmic Disease. Part II: Clinical Aspects. Ophthal Plast Reconstr Surg. 31(3):167-78, 2015
                                            2. McNab AA et al: IgG4-related ophthalmic disease. Part I: background and pathology. Ophthal Plast Reconstr Surg. 31(2):83-8, 2015
                                            3. Bijlsma WR et al: Risk factors for idiopathic orbital inflammation: a case-control study. Br J Ophthalmol. 95(3):360-4, 2011
                                            4. Mendenhall WM et al: Orbital pseudotumor. Am J Clin Oncol. 33(3):304-6, 2010
                                            5. Schuknecht B et al: Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia. Eur J Radiol. 69(3):445-53, 2009
                                            6. Katsimpris JM et al: Isolated lateral rectus myositis as a manifestation of idiopathic orbital inflammation. Klin Monbl Augenheilkd. 225(5):510-3, 2008
                                            7. Swamy BN et al: Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes. Br J Ophthalmol. 91(12):1667-70, 2007
                                            8. Brannan PA et al: Sclerosing idiopathic orbital inflammation. J Pediatr Ophthalmol Strabismus. 43(3):183-4, 2006
                                            9. Gordon LK: Diagnostic dilemmas in orbital inflammatory disease. Ocul Immunol Inflamm. 11(1):3-15, 2003
                                            10. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                            11. Jacobs D et al: Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 13(6):347-51, 2002
                                            12. Wasmeier C et al: Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome--are they the same disease? J Neurol. 249(9):1237-41, 2002
                                            13. Yuen SJ et al: Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am. 15(1):121-6, 2002
                                            14. Bernardino CR et al: Angle-closure glaucoma in association with orbital pseudotumor. Ophthalmology. 108(9):1603-6, 2001
                                            15. Maalouf T et al: What has become of our idiopathic inflammatory pseudo-tumors of the orbit? Orbit. 18(3):157-166, 1999
                                            16. Weber AL et al: Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am. 37(1):151-68, xi, 1999
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Idiopathic orbital inflammation (IOI)
                                                        • Synonyms

                                                          • Orbital pseudotumor (or simply "pseudotumor")
                                                        • Definitions

                                                          • Nonspecific orbital inflammation, often without known etiology or systemic illness
                                                          • Tolosa-Hunt
                                                            • Variant apical form extending into cavernous sinus
                                                          • Sclerosing orbital inflammatory pseudotumor
                                                            • Variant form with chronic progressive fibrosis
                                                          • IgG4-related disease (IgG4-RD)
                                                            • Variant form, recently identified, with plasma cell-mediated inflammation
                                                            • Accounts for minority of IOI
                                                            • May be classified as distinct entity

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. McNab AA et al: IgG4-Related Ophthalmic Disease. Part II: Clinical Aspects. Ophthal Plast Reconstr Surg. 31(3):167-78, 2015
                                                                                      2. McNab AA et al: IgG4-related ophthalmic disease. Part I: background and pathology. Ophthal Plast Reconstr Surg. 31(2):83-8, 2015
                                                                                      3. Bijlsma WR et al: Risk factors for idiopathic orbital inflammation: a case-control study. Br J Ophthalmol. 95(3):360-4, 2011
                                                                                      4. Mendenhall WM et al: Orbital pseudotumor. Am J Clin Oncol. 33(3):304-6, 2010
                                                                                      5. Schuknecht B et al: Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia. Eur J Radiol. 69(3):445-53, 2009
                                                                                      6. Katsimpris JM et al: Isolated lateral rectus myositis as a manifestation of idiopathic orbital inflammation. Klin Monbl Augenheilkd. 225(5):510-3, 2008
                                                                                      7. Swamy BN et al: Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes. Br J Ophthalmol. 91(12):1667-70, 2007
                                                                                      8. Brannan PA et al: Sclerosing idiopathic orbital inflammation. J Pediatr Ophthalmol Strabismus. 43(3):183-4, 2006
                                                                                      9. Gordon LK: Diagnostic dilemmas in orbital inflammatory disease. Ocul Immunol Inflamm. 11(1):3-15, 2003
                                                                                      10. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                                                                      11. Jacobs D et al: Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 13(6):347-51, 2002
                                                                                      12. Wasmeier C et al: Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome--are they the same disease? J Neurol. 249(9):1237-41, 2002
                                                                                      13. Yuen SJ et al: Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am. 15(1):121-6, 2002
                                                                                      14. Bernardino CR et al: Angle-closure glaucoma in association with orbital pseudotumor. Ophthalmology. 108(9):1603-6, 2001
                                                                                      15. Maalouf T et al: What has become of our idiopathic inflammatory pseudo-tumors of the orbit? Orbit. 18(3):157-166, 1999
                                                                                      16. Weber AL et al: Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am. 37(1):151-68, xi, 1999