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Orbital Cavernous Venous Malformation (Hemangioma)
Aparna Singhal, MDH. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Orbital cavernous venous malformation (OCVM)
            • Synonyms

              • Cavernous hemangioma (misnomer)
              • Encapsulated cavernous lesion of orbit
            • Definitions

              • Venous vascular malformation of orbit characterized by endothelial-lined cavernous spaces
              • Pseudoencapsulated morphology distinguishes OCVM from venous malformations elsewhere in head and neck

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                • Reporting Tips

                                                  Selected References

                                                  1. Bonavolontà P et al: Epidemiological analysis of venous malformation of the orbit. J Craniofac Surg. 31(3):759-61, 2020
                                                  2. Faiz K et al: Bleomycin for orbital and peri-orbital veno-lymphatic malformations - a systematic review. Interv Neuroradiol. 27(2):291-7, 2020
                                                  3. International Society for the Study of Vascular Anomalies: 2018 Classification of Vascular Anomalies. Published May 2018. Accessed March 21, 2021. https://www.issva.org/classification
                                                  4. Calandriello L et al: Cavernous venous malformation (cavernous hemangioma) of the orbit: Current concepts and a review of the literature. Surv Ophthalmol. 62(4):393-403, 2017
                                                  5. Purohit BS et al: Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging. Insights Imaging. 7(1):43-68, 2016
                                                  6. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                                  7. McNab AA et al: The natural history of orbital cavernous hemangiomas. Ophthal Plast Reconstr Surg. 31(2): 89-93, 2015
                                                  8. Rootman DB et al: Comparative histology of orbital, hepatic and subcutaneous cavernous venous malformations. Br J Ophthalmol. 99(1):138-40, 2015
                                                  9. Rootman DB et al: Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature. Br J Ophthalmol. 98(7):880-8, 2014
                                                  10. Rootman J et al: Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies. Ophthal Plast Reconstr Surg. 30(2):91-104, 2014
                                                  11. Bonavolontà G et al: An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg. 29(2):79-86, 2013
                                                  12. Osaki TH et al: Immunohistochemical investigations of orbital infantile hemangiomas and adult encapsulated cavernous venous lesions (malformation versus hemangioma). Ophthal Plast Reconstr Surg. 29(3):183-95, 2013
                                                  13. Gupta A et al: Orbital cavernous haemangiomas: immunohistochemical study of proliferative capacity, vascular differentiation and hormonal receptor status. Orbit. 31(6):386-9, 2012
                                                  14. Tian YM et al: Adhesion of cavernous hemangioma in the orbit revealed by CT and MRI: analysis of 97 cases. Int J Ophthalmol. 4(2):195-8, 2011
                                                  15. Madge SN et al: Primary orbital intraosseous hemangioma. Ophthal Plast Reconstr Surg. 25(1):37-41, 2009
                                                  16. Wilms G: Orbital cavernous hemangiomas. AJNR Am J Neuroradiol. 30(1):E7, 2009
                                                  17. Bouguila J et al: [Intraorbital cavernous hemangioma.] Rev Stomatol Chir Maxillofac. 109(5):312-5, 2008
                                                  18. Cheng JW et al: Transconjunctival orbitotomy for orbital cavernous hemangiomas. Can J Ophthalmol. 43(2):234-8, 2008
                                                  19. Jinhu Y et al: Dynamic enhancement features of cavernous sinus cavernous hemangiomas on conventional contrast-enhanced MR imaging. AJNR Am J Neuroradiol. 29(3):577-81, 2008
                                                  20. Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
                                                  21. Chaddad Neto F et al: Giant cavernoma of the orbit: clinical and surgical considerations. Arq Neuropsiquiatr. 65(4A):992-5, 2007
                                                  22. Rootman J: Vascular malformations of the orbit: hemodynamic concepts. Orbit. 22(2):103-20, 2003
                                                  23. Chang EL et al: Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome. Ophthalmology. 109(3):537-41, 2002
                                                  24. Sayit E et al: The role of Tc-99m RBC scintigraphy in the differential diagnosis of orbital cavernous hemangioma. Ann Nucl Med. 15(2):149-51, 2001
                                                  25. Bilaniuk LT: Orbital vascular lesions. Role of imaging. Radiol Clin North Am. 37(1):169-83, xi, 1999
                                                  26. Harris GJ: Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Orbital Society. Am J Ophthalmol. 127(4):453-5, 1999
                                                  27. Thorn-Kany M et al: Cavernous hemangiomas of the orbit: MR imaging. J Neuroradiol. 26(2):79-86, 1999
                                                  28. Koeller KK et al: Orbital masses. Semin Ultrasound CT MR. 19(3):272-91, 1998
                                                  29. Ki WW et al: Diagnosis of orbital cavernous hemangioma with Tc-99m RBC SPECT. Clin Nucl Med. 22(8):546-9, 1997
                                                  30. Ohtsuka K et al: Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol. 123(3):396-8, 1997
                                                  31. Forbes G: Vascular lesions in the orbit. Neuroimaging Clin N Am. 6(1):113-22, 1996
                                                  32. Acciarri N et al: Orbital cavernous angiomas: surgical experience on a series of 13 cases. J Neurosurg Sci. 39(4):203-9, 1995
                                                  33. Rootman J et al: Multidisciplinary approaches to complicated vascular lesions of the orbit. Ophthalmology. 99(9):1440-6, 1992
                                                  34. Fries PD et al: MR imaging of orbital cavernous hemangioma. J Comput Assist Tomogr. 11(3):418-21, 1987
                                                  35. Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982
                                                  36. Harris GJ et al: Cavernous hemangioma of the orbit. J Neurosurg. 51(2):219-28, 1979
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Orbital cavernous venous malformation (OCVM)
                                                            • Synonyms

                                                              • Cavernous hemangioma (misnomer)
                                                              • Encapsulated cavernous lesion of orbit
                                                            • Definitions

                                                              • Venous vascular malformation of orbit characterized by endothelial-lined cavernous spaces
                                                              • Pseudoencapsulated morphology distinguishes OCVM from venous malformations elsewhere in head and neck

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Staging, Grading, & Classification

                                                                                • Gross Pathologic & Surgical Features

                                                                                  • Microscopic Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Consider

                                                                                              • Image Interpretation Pearls

                                                                                                • Reporting Tips

                                                                                                  Selected References

                                                                                                  1. Bonavolontà P et al: Epidemiological analysis of venous malformation of the orbit. J Craniofac Surg. 31(3):759-61, 2020
                                                                                                  2. Faiz K et al: Bleomycin for orbital and peri-orbital veno-lymphatic malformations - a systematic review. Interv Neuroradiol. 27(2):291-7, 2020
                                                                                                  3. International Society for the Study of Vascular Anomalies: 2018 Classification of Vascular Anomalies. Published May 2018. Accessed March 21, 2021. https://www.issva.org/classification
                                                                                                  4. Calandriello L et al: Cavernous venous malformation (cavernous hemangioma) of the orbit: Current concepts and a review of the literature. Surv Ophthalmol. 62(4):393-403, 2017
                                                                                                  5. Purohit BS et al: Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging. Insights Imaging. 7(1):43-68, 2016
                                                                                                  6. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                                                                                  7. McNab AA et al: The natural history of orbital cavernous hemangiomas. Ophthal Plast Reconstr Surg. 31(2): 89-93, 2015
                                                                                                  8. Rootman DB et al: Comparative histology of orbital, hepatic and subcutaneous cavernous venous malformations. Br J Ophthalmol. 99(1):138-40, 2015
                                                                                                  9. Rootman DB et al: Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature. Br J Ophthalmol. 98(7):880-8, 2014
                                                                                                  10. Rootman J et al: Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies. Ophthal Plast Reconstr Surg. 30(2):91-104, 2014
                                                                                                  11. Bonavolontà G et al: An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg. 29(2):79-86, 2013
                                                                                                  12. Osaki TH et al: Immunohistochemical investigations of orbital infantile hemangiomas and adult encapsulated cavernous venous lesions (malformation versus hemangioma). Ophthal Plast Reconstr Surg. 29(3):183-95, 2013
                                                                                                  13. Gupta A et al: Orbital cavernous haemangiomas: immunohistochemical study of proliferative capacity, vascular differentiation and hormonal receptor status. Orbit. 31(6):386-9, 2012
                                                                                                  14. Tian YM et al: Adhesion of cavernous hemangioma in the orbit revealed by CT and MRI: analysis of 97 cases. Int J Ophthalmol. 4(2):195-8, 2011
                                                                                                  15. Madge SN et al: Primary orbital intraosseous hemangioma. Ophthal Plast Reconstr Surg. 25(1):37-41, 2009
                                                                                                  16. Wilms G: Orbital cavernous hemangiomas. AJNR Am J Neuroradiol. 30(1):E7, 2009
                                                                                                  17. Bouguila J et al: [Intraorbital cavernous hemangioma.] Rev Stomatol Chir Maxillofac. 109(5):312-5, 2008
                                                                                                  18. Cheng JW et al: Transconjunctival orbitotomy for orbital cavernous hemangiomas. Can J Ophthalmol. 43(2):234-8, 2008
                                                                                                  19. Jinhu Y et al: Dynamic enhancement features of cavernous sinus cavernous hemangiomas on conventional contrast-enhanced MR imaging. AJNR Am J Neuroradiol. 29(3):577-81, 2008
                                                                                                  20. Smoker WR et al: Vascular lesions of the orbit: more than meets the eye. Radiographics. 28(1):185-204; quiz 325, 2008
                                                                                                  21. Chaddad Neto F et al: Giant cavernoma of the orbit: clinical and surgical considerations. Arq Neuropsiquiatr. 65(4A):992-5, 2007
                                                                                                  22. Rootman J: Vascular malformations of the orbit: hemodynamic concepts. Orbit. 22(2):103-20, 2003
                                                                                                  23. Chang EL et al: Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome. Ophthalmology. 109(3):537-41, 2002
                                                                                                  24. Sayit E et al: The role of Tc-99m RBC scintigraphy in the differential diagnosis of orbital cavernous hemangioma. Ann Nucl Med. 15(2):149-51, 2001
                                                                                                  25. Bilaniuk LT: Orbital vascular lesions. Role of imaging. Radiol Clin North Am. 37(1):169-83, xi, 1999
                                                                                                  26. Harris GJ: Orbital vascular malformations: a consensus statement on terminology and its clinical implications. Orbital Society. Am J Ophthalmol. 127(4):453-5, 1999
                                                                                                  27. Thorn-Kany M et al: Cavernous hemangiomas of the orbit: MR imaging. J Neuroradiol. 26(2):79-86, 1999
                                                                                                  28. Koeller KK et al: Orbital masses. Semin Ultrasound CT MR. 19(3):272-91, 1998
                                                                                                  29. Ki WW et al: Diagnosis of orbital cavernous hemangioma with Tc-99m RBC SPECT. Clin Nucl Med. 22(8):546-9, 1997
                                                                                                  30. Ohtsuka K et al: Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol. 123(3):396-8, 1997
                                                                                                  31. Forbes G: Vascular lesions in the orbit. Neuroimaging Clin N Am. 6(1):113-22, 1996
                                                                                                  32. Acciarri N et al: Orbital cavernous angiomas: surgical experience on a series of 13 cases. J Neurosurg Sci. 39(4):203-9, 1995
                                                                                                  33. Rootman J et al: Multidisciplinary approaches to complicated vascular lesions of the orbit. Ophthalmology. 99(9):1440-6, 1992
                                                                                                  34. Fries PD et al: MR imaging of orbital cavernous hemangioma. J Comput Assist Tomogr. 11(3):418-21, 1987
                                                                                                  35. Mulliken JB et al: Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 69(3):412-22, 1982
                                                                                                  36. Harris GJ et al: Cavernous hemangioma of the orbit. J Neurosurg. 51(2):219-28, 1979