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Venous Malformation
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Venous malformation (VM) is preferred term
            • Synonyms

              • Cavernous malformation, cavernous hemangioma (both terms to be avoided)
            • Definitions

              • VM: Subtype of congenital slow-/low-flow VM due to error in vein formation
                • i.e., not neoplasm

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Other Modality 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. Mamlouk MD et al: Vascular anomaly imaging mimics and differential diagnoses. Pediatr Radiol. 49(8):1088-1103, 2019
                                                2. Alexander MD et al: Revisiting classic MRI findings of venous malformations: changes in protocols may lead to potential misdiagnosis. Neuroradiol J. 31(5):509-12, 2018
                                                3. ISSVA International Society for the Study of Vascular Anomalies. Published May 2018. Accessed May 3, 2021. https://www.issva.org/classification
                                                4. Merrow AC et al: 2014 Revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
                                                5. Olivieri B et al: Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation--Part 2, Venous Malformation Mimickers. AJR Am J Roentgenol. 206(5):952-62, 2016
                                                6. White CL et al: Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation--Part 1, Lymphatic Malformation Mimickers. AJR Am J Roentgenol. 206(5):940-51, 2016
                                                7. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                                8. Kollipara R et al: Current classification and terminology of pediatric vascular anomalies. AJR Am J Roentgenol. 201(5):1124-35, 2013
                                                9. Chen WL et al: Percutaneous sclerotherapy of massive venous malformations of the face and neck using fibrin glue combined with OK-432 and pingyangmycin. Head Neck. 32(4):467-72, 2010
                                                10. Eivazi B et al: Orbital and periorbital vascular anomalies - an approach to diagnosis and therapeutic concepts. Acta Otolaryngol. Epub ahead of print, 2010
                                                11. Glade RS et al: Diagnosis and management of pediatric cervicofacial venous malformations: retrospective review from a vascular anomalies center. Laryngoscope. 120(2):229-35, 2010
                                                12. Duyka LJ et al: Progesterone receptors identified in vascular malformations of the head and neck. Otolaryngol Head Neck Surg. 141(4):491-5, 2009
                                                13. Zheng JW et al: Intralesional injection of Pingyangmycin for vascular malformations in oral and maxillofacial regions: an evaluation of 297 consecutive patients. Oral Oncol. 45(10):872-6, 2009
                                                14. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                                15. Lee BB et al: Advanced management of venous malformation with ethanol sclerotherapy: mid-term results. J Vasc Surg. 37(3): 533-8, 2003
                                                16. Scolozzi P et al: Intraoral venous malformation presenting with multiple phleboliths. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 96(2): 197-200, 2003
                                                17. Curé JK: Imaging of vascular lesions of the head and neck. Facial Plast Surg Clin North Am. 9(4):525-49, 2001
                                                18. Holt PD et al: Interventional radiology in the treatment of vascular lesions. Facial Plast Surg Clin North Am. 9(4):585-99, 2001
                                                19. Marler JJ et al: Vascular anomalies: classification, diagnosis, and natural history. Facial Plast Surg Clin North Am. 9(4):495-504, 2001
                                                20. North PE et al: Histopathological diagnosis of infantile hemangiomas and vascular malformations. Facial Plast Surg Clin North Am. 9(4):505-24, 2001
                                                21. Boukobza M et al: Cerebral developmental venous anomalies associated with head and neck venous malformations. AJNR Am J Neuroradiol. 17(5): 987-94, 1996
                                                22. Baker LL et al: Hemangiomas and vascular malformations of the head and neck: MR characterization. AJNR Am J Neuroradiol. 14(2):307-14, 1993
                                                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

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Venous malformation (VM) is preferred term
                                                          • Synonyms

                                                            • Cavernous malformation, cavernous hemangioma (both terms to be avoided)
                                                          • Definitions

                                                            • VM: Subtype of congenital slow-/low-flow VM due to error in vein formation
                                                              • i.e., not neoplasm

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Other Modality 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. Mamlouk MD et al: Vascular anomaly imaging mimics and differential diagnoses. Pediatr Radiol. 49(8):1088-1103, 2019
                                                                                              2. Alexander MD et al: Revisiting classic MRI findings of venous malformations: changes in protocols may lead to potential misdiagnosis. Neuroradiol J. 31(5):509-12, 2018
                                                                                              3. ISSVA International Society for the Study of Vascular Anomalies. Published May 2018. Accessed May 3, 2021. https://www.issva.org/classification
                                                                                              4. Merrow AC et al: 2014 Revised classification of vascular lesions from the International Society for the Study of Vascular Anomalies: radiologic-pathologic update. Radiographics. 36(5):1494-516, 2016
                                                                                              5. Olivieri B et al: Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation--Part 2, Venous Malformation Mimickers. AJR Am J Roentgenol. 206(5):952-62, 2016
                                                                                              6. White CL et al: Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation--Part 1, Lymphatic Malformation Mimickers. AJR Am J Roentgenol. 206(5):940-51, 2016
                                                                                              7. Wassef M et al: Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 136(1):e203-14, 2015
                                                                                              8. Kollipara R et al: Current classification and terminology of pediatric vascular anomalies. AJR Am J Roentgenol. 201(5):1124-35, 2013
                                                                                              9. Chen WL et al: Percutaneous sclerotherapy of massive venous malformations of the face and neck using fibrin glue combined with OK-432 and pingyangmycin. Head Neck. 32(4):467-72, 2010
                                                                                              10. Eivazi B et al: Orbital and periorbital vascular anomalies - an approach to diagnosis and therapeutic concepts. Acta Otolaryngol. Epub ahead of print, 2010
                                                                                              11. Glade RS et al: Diagnosis and management of pediatric cervicofacial venous malformations: retrospective review from a vascular anomalies center. Laryngoscope. 120(2):229-35, 2010
                                                                                              12. Duyka LJ et al: Progesterone receptors identified in vascular malformations of the head and neck. Otolaryngol Head Neck Surg. 141(4):491-5, 2009
                                                                                              13. Zheng JW et al: Intralesional injection of Pingyangmycin for vascular malformations in oral and maxillofacial regions: an evaluation of 297 consecutive patients. Oral Oncol. 45(10):872-6, 2009
                                                                                              14. Bisdorff A et al: Intracranial vascular anomalies in patients with periorbital lymphatic and lymphaticovenous malformations. AJNR Am J Neuroradiol. 28(2):335-41, 2007
                                                                                              15. Lee BB et al: Advanced management of venous malformation with ethanol sclerotherapy: mid-term results. J Vasc Surg. 37(3): 533-8, 2003
                                                                                              16. Scolozzi P et al: Intraoral venous malformation presenting with multiple phleboliths. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 96(2): 197-200, 2003
                                                                                              17. Curé JK: Imaging of vascular lesions of the head and neck. Facial Plast Surg Clin North Am. 9(4):525-49, 2001
                                                                                              18. Holt PD et al: Interventional radiology in the treatment of vascular lesions. Facial Plast Surg Clin North Am. 9(4):585-99, 2001
                                                                                              19. Marler JJ et al: Vascular anomalies: classification, diagnosis, and natural history. Facial Plast Surg Clin North Am. 9(4):495-504, 2001
                                                                                              20. North PE et al: Histopathological diagnosis of infantile hemangiomas and vascular malformations. Facial Plast Surg Clin North Am. 9(4):505-24, 2001
                                                                                              21. Boukobza M et al: Cerebral developmental venous anomalies associated with head and neck venous malformations. AJNR Am J Neuroradiol. 17(5): 987-94, 1996
                                                                                              22. Baker LL et al: Hemangiomas and vascular malformations of the head and neck: MR characterization. AJNR Am J Neuroradiol. 14(2):307-14, 1993