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Idiopathic Orbital Inflammation (Pseudotumor)
Bronwyn E. Hamilton, MD; 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 syndrome
                  • Variant apical form extending into cavernous sinus
                • Sclerosing orbital inflammatory pseudotumor
                  • Variant form with chronic progressive fibrosis

              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. Ronquillo Y et al: Nonspecific orbital inflammation. StatPeals, 2021
                                            2. Eissa L et al: Arterial spin labeling and diffusion-weighted MR imaging: utility in differentiating idiopathic orbital inflammatory pseudotumor from orbital lymphoma. Clin Imaging. 71:63-8, 2020
                                            3. Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
                                            4. MarinĂ² M et al: Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis. J Endocrinol Invest. 43(4):401-11, 2020
                                            5. Wu WC et al: Clinical images: IgG4-related orbital pseudotumor. Arthritis Rheumatol. 72(12):2168, 2020
                                            6. Ferreira TA et al: CT and MR imaging of orbital inflammation. Neuroradiology. 60(12):1253-66, 2018
                                            7. Guo J et al: MR-based radiomics signature in differentiating ocular adnexal lymphoma from idiopathic orbital inflammation. Eur Radiol. 28(9):3872-81, 2018
                                            8. Ren J et al: Differentiation of orbital lymphoma and idiopathic orbital inflammatory pseudotumor: combined diagnostic value of conventional MRI and histogram analysis of ADC maps. BMC Med Imaging. 18(1):6, 2018
                                            9. Cunnane MB et al: Imaging of orbital disorders. Handb Clin Neurol. 135:659-72, 2016
                                            10. McNab AA et al: IgG4-related ophthalmic disease. part ii: clinical aspects. Ophthal Plast Reconstr Surg. 31(3):167-78, 2015
                                            11. Bijlsma WR et al: Risk factors for idiopathic orbital inflammation: a case-control study. Br J Ophthalmol. 95(3):360-4, 2011
                                            12. Mendenhall WM et al: Orbital pseudotumor. Am J Clin Oncol. 33(3):304-6, 2010
                                            13. 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
                                            14. Katsimpris JM et al: Isolated lateral rectus myositis as a manifestation of idiopathic orbital inflammation. Klin Monbl Augenheilkd. 225(5):510-3, 2008
                                            15. Swamy BN et al: Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes. Br J Ophthalmol. 91(12):1667-70, 2007
                                            16. Brannan PA et al: Sclerosing idiopathic orbital inflammation. J Pediatr Ophthalmol Strabismus. 43(3):183-4, 2006
                                            17. Gordon LK: Diagnostic dilemmas in orbital inflammatory disease. Ocul Immunol Inflamm. 11(1):3-15, 2003
                                            18. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                            19. Jacobs D et al: Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 13(6):347-51, 2002
                                            20. 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
                                            21. Yuen SJ et al: Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am. 15(1):121-6, 2002
                                            22. Bernardino CR et al: Angle-closure glaucoma in association with orbital pseudotumor. Ophthalmology. 108(9):1603-6, 2001
                                            23. Maalouf T et al: What has become of our idiopathic inflammatory pseudo-tumors of the orbit? Orbit. 18(3):157-66, 1999
                                            24. 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 syndrome
                                                            • Variant apical form extending into cavernous sinus
                                                          • Sclerosing orbital inflammatory pseudotumor
                                                            • Variant form with chronic progressive fibrosis

                                                        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. Ronquillo Y et al: Nonspecific orbital inflammation. StatPeals, 2021
                                                                                      2. Eissa L et al: Arterial spin labeling and diffusion-weighted MR imaging: utility in differentiating idiopathic orbital inflammatory pseudotumor from orbital lymphoma. Clin Imaging. 71:63-8, 2020
                                                                                      3. Madhavan AA et al: Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome. Neuroradiol J. 33(5):424-7, 2020
                                                                                      4. MarinĂ² M et al: Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis. J Endocrinol Invest. 43(4):401-11, 2020
                                                                                      5. Wu WC et al: Clinical images: IgG4-related orbital pseudotumor. Arthritis Rheumatol. 72(12):2168, 2020
                                                                                      6. Ferreira TA et al: CT and MR imaging of orbital inflammation. Neuroradiology. 60(12):1253-66, 2018
                                                                                      7. Guo J et al: MR-based radiomics signature in differentiating ocular adnexal lymphoma from idiopathic orbital inflammation. Eur Radiol. 28(9):3872-81, 2018
                                                                                      8. Ren J et al: Differentiation of orbital lymphoma and idiopathic orbital inflammatory pseudotumor: combined diagnostic value of conventional MRI and histogram analysis of ADC maps. BMC Med Imaging. 18(1):6, 2018
                                                                                      9. Cunnane MB et al: Imaging of orbital disorders. Handb Clin Neurol. 135:659-72, 2016
                                                                                      10. McNab AA et al: IgG4-related ophthalmic disease. part ii: clinical aspects. Ophthal Plast Reconstr Surg. 31(3):167-78, 2015
                                                                                      11. Bijlsma WR et al: Risk factors for idiopathic orbital inflammation: a case-control study. Br J Ophthalmol. 95(3):360-4, 2011
                                                                                      12. Mendenhall WM et al: Orbital pseudotumor. Am J Clin Oncol. 33(3):304-6, 2010
                                                                                      13. 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
                                                                                      14. Katsimpris JM et al: Isolated lateral rectus myositis as a manifestation of idiopathic orbital inflammation. Klin Monbl Augenheilkd. 225(5):510-3, 2008
                                                                                      15. Swamy BN et al: Idiopathic orbital inflammatory syndrome: clinical features and treatment outcomes. Br J Ophthalmol. 91(12):1667-70, 2007
                                                                                      16. Brannan PA et al: Sclerosing idiopathic orbital inflammation. J Pediatr Ophthalmol Strabismus. 43(3):183-4, 2006
                                                                                      17. Gordon LK: Diagnostic dilemmas in orbital inflammatory disease. Ocul Immunol Inflamm. 11(1):3-15, 2003
                                                                                      18. Yuen SJ et al: Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol. 121(4):491-9, 2003
                                                                                      19. Jacobs D et al: Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol. 13(6):347-51, 2002
                                                                                      20. 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
                                                                                      21. Yuen SJ et al: Idiopathic orbital inflammation: ocular mechanisms and clinicopathology. Ophthalmol Clin North Am. 15(1):121-6, 2002
                                                                                      22. Bernardino CR et al: Angle-closure glaucoma in association with orbital pseudotumor. Ophthalmology. 108(9):1603-6, 2001
                                                                                      23. Maalouf T et al: What has become of our idiopathic inflammatory pseudo-tumors of the orbit? Orbit. 18(3):157-66, 1999
                                                                                      24. Weber AL et al: Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am. 37(1):151-68, xi, 1999