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Venous Malformation
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Venous anomaly, common venous malformation (VM)
            • Not hemangioma (benign vascular neoplasm of capillaries) or arteriovenous malformation (high-flow lesion)
              • Distinct entities with different histologies & behaviors
          • Definitions

            • Subtype of congenital slow- or low-flow vascular malformation due to error in vein formation; not neoplastic

          IMAGING

          • General Features

            • Radiographic Findings

              • Fluoroscopic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  • Immunohistochemical Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                • Reporting Tips

                                                  Selected References

                                                  1. Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
                                                  2. Mattila KA et al: Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement. Pediatr Radiol. 50(4):509-15, 2020
                                                  3. Restrepo R et al: Three distinct vascular anomalies involving skeletal muscle: simplifying the approach for the general radiologist. Radiol Clin North Am. 58(3):603-18, 2020
                                                  4. Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
                                                  5. Mamlouk MD et al: Vascular anomaly imaging mimics and differential diagnoses. Pediatr Radiol. 49(8):1088-103, 2019
                                                  6. ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed April 27, 2020. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                                  7. 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
                                                  8. 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
                                                  9. Wassef M et al: Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics. 136(1):e203-14, 2015
                                                  10. Alomari AI et al: Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop. 34(1):109-17, 2014
                                                  11. Dasgupta R et al: Venous malformations. Semin Pediatr Surg. 23(4):198-202, 2014
                                                  12. Spencer SA et al: Orthopedic issues in vascular anomalies. Semin Pediatr Surg. 23(4):227-32, 2014
                                                  13. Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
                                                  14. Jans L et al: MRI demonstrates the extension of juxta-articular venous malformation of the knee and correlates with joint changes. Eur Radiol. 20(7):1792-8, 2010
                                                  15. Legiehn GM et al: A step-by-step practical approach to imaging diagnosis and interventional radiologic therapy in vascular malformations. Semin Intervent Radiol. 27(2):209-31, 2010
                                                  16. Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
                                                  17. 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
                                                  Related Anatomy
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                                                  Related Differential Diagnoses
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                                                  References
                                                  Tables

                                                  Tables

                                                  KEY FACTS

                                                  • Terminology

                                                    • Imaging

                                                      • Top Differential Diagnoses

                                                        • Clinical Issues

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Venous anomaly, common venous malformation (VM)
                                                            • Not hemangioma (benign vascular neoplasm of capillaries) or arteriovenous malformation (high-flow lesion)
                                                              • Distinct entities with different histologies & behaviors
                                                          • Definitions

                                                            • Subtype of congenital slow- or low-flow vascular malformation due to error in vein formation; not neoplastic

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • Fluoroscopic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  • Immunohistochemical Features

                                                                                    CLINICAL ISSUES

                                                                                    • Presentation

                                                                                      • Demographics

                                                                                        • Natural History & Prognosis

                                                                                          • Treatment

                                                                                            DIAGNOSTIC CHECKLIST

                                                                                            • Consider

                                                                                              • Image Interpretation Pearls

                                                                                                • Reporting Tips

                                                                                                  Selected References

                                                                                                  1. Abu Ata N et al: Imaging of vascular anomalies in the pediatric musculoskeletal system. Semin Roentgenol. 56(3):288-306, 2021
                                                                                                  2. Mattila KA et al: Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement. Pediatr Radiol. 50(4):509-15, 2020
                                                                                                  3. Restrepo R et al: Three distinct vascular anomalies involving skeletal muscle: simplifying the approach for the general radiologist. Radiol Clin North Am. 58(3):603-18, 2020
                                                                                                  4. Bertino F et al: Congenital limb overgrowth syndromes associated with vascular anomalies. Radiographics. 39(2):491-515, 2019
                                                                                                  5. Mamlouk MD et al: Vascular anomaly imaging mimics and differential diagnoses. Pediatr Radiol. 49(8):1088-103, 2019
                                                                                                  6. ISSVA Classification of Vascular Anomalies. Published April 2014. Updated May 2018. Accessed April 27, 2020. https://www.issva.org/UserFiles/file/ISSVA-Classification-2018.pdf
                                                                                                  7. 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
                                                                                                  8. 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
                                                                                                  9. Wassef M et al: Vascular anomalies classification: recommendations from the international society for the study of vascular anomalies. Pediatrics. 136(1):e203-14, 2015
                                                                                                  10. Alomari AI et al: Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity. J Pediatr Orthop. 34(1):109-17, 2014
                                                                                                  11. Dasgupta R et al: Venous malformations. Semin Pediatr Surg. 23(4):198-202, 2014
                                                                                                  12. Spencer SA et al: Orthopedic issues in vascular anomalies. Semin Pediatr Surg. 23(4):227-32, 2014
                                                                                                  13. Uller W et al: Overgrowth syndromes with complex vascular anomalies. Semin Pediatr Surg. 23(4):208-15, 2014
                                                                                                  14. Jans L et al: MRI demonstrates the extension of juxta-articular venous malformation of the knee and correlates with joint changes. Eur Radiol. 20(7):1792-8, 2010
                                                                                                  15. Legiehn GM et al: A step-by-step practical approach to imaging diagnosis and interventional radiologic therapy in vascular malformations. Semin Intervent Radiol. 27(2):209-31, 2010
                                                                                                  16. Lobo-Mueller E et al: Extremity vascular anomalies in children: introduction, classification, and imaging. Semin Musculoskelet Radiol. 13(3):210-35, 2009
                                                                                                  17. 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