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Vascular Malformations
Christopher Bailey, MD; Scott R. Shuldiner, BS; Clifford R. Weiss, MD, FSIR
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KEY FACTS

  • Terminology

    • Preprocedure

      • Procedure

        • Outcomes

          TERMINOLOGY

          • Definitions

            • Vascular malformation (VMF): Most common vascular anomaly consisting of abnormal clusters of blood vessels without endothelial proliferation
              • Low flow: Capillary, venous, lymphatic or "mixed" malformations
                • Venous malformations (VM)
                  • Most common type of VMF consisting of dilated venous vessels with abnormal smooth muscle, which allows for expansion of VM over time
                  • May or may not communicate with systemic vasculature
                  • Usually sporadic but can occur as part of genetic syndrome (e.g., Klippel-Trénaunay)
                  • Diverse range of size, location, and appearance: From small, solitary, and localized to skin and subcutaneous tissues to diffuse masses extending across multiple tissue planes and surrounding vascular, nervous, and visceral bodies
                • Lymphatic malformation (LM)
                  • 2nd most common VMF consisting of benign cystic lesions made up of lymphatic vessels filled with lymphatic fluid
                  • Most commonly sporadic; however, some associated with genetic syndromes (e.g., Turner, Noonan)
                  • Classified based on cyst size as macrocystic (large), microcystic (small), or mixed
                  • Previously used terms such as lymphangiomas and cystic hygromas should be avoided
                • Capillary malformation (CM)
                  • Limited to superficial skin layers, formerly called port-wine stains
                  • Can darken, thicken, and become nodular if left untreated
                  • Often associated with congenital syndrome (e.g., Sturge-Weber, Parkes Weber, Klippel-Trénaunay)
              • High flow: Direct communication between arteries and veins
                • Arteriovenous malformations (AVM)
                  • Complex communicating network of arterial and venous vessels
                  • Typically more invasive than other VMFs
                  • Arterial inflow diverted via nidus into draining veins, causing tissue ischemia and venous hypertension
                  • High recurrence rate after treatment with total nidus eradication considered "curative"
                • Arteriovenous fistula (AVF)
                  • Direct abnormal communication between artery and vein without interposed nidus
                  • Can be acquired (most common) or congenital
                  • Limb ischemia can occur distal to AVF due to arterial blood shunted via fistula (steal phenomenon)
              • Syndromes associated with VMFs
                • Klippel-Trénaunay syndrome: VM combined with LM and CM
                • Parkes Weber syndrome: VM combined with LM, CM, and AVM
                • Sturge-Weber syndrome: Cutaneous CM along distribution of facial and ocular nerves; leptomeningeal vascular anomalies (CM, VM)
          • Classification

            • International Society for the Study of Vascular Anomalies (ISSVA) classification (revised 2014)
              • Most widely recognized classification system based on cellular features, flow characteristics, associated clinical features and genetics
              • Defines common language used by all members of interdisciplinary teams involved in care of vascular anomalies
              • Broadly divides vascular anomalies into nonneoplastic lesions (VMFs) and neoplastic lesions (vascular tumors)
            • SE Mitchell Vascular Anomalies Flow Chart (SEMVAFC)
              • Combines clinical and imaging findings for accurate VMF diagnosis
              • Utilizes classification schema similar to ISSVA to preserve common language
              • Potential to identify syndromes associated with VMF
            • Yakes classification
              • Organizes AVMs by angioarchitecture and ascribes specific endovascular treatment options for each subtype
          • Low-Flow Malformations: Clinical Features

            • Presentation & physical exam
              • VM
                • Present as soft, compressible, nonpulsatile, bluish lesions
                • Often present with pain, swelling, and depending on location, mechanical dysfunction or cosmetic disfigurement
                • Vary in size and distribution; potentially involves multiple anatomic locations
                • Majority are solitary but can be associated with genetic syndrome (e.g., blue rubber bleb nevus syndrome)
                • Can occur in any anatomic depth (i.e., skin, subcutaneous tissue, muscle, etc.) and are often infiltrative and extend to underlying or nearby structures
                • Placement of affected area in dependent position or Valsalva maneuver may elicit blood pooling and subsequent enlargement of lesion
                • Most commonly occur in head, neck, and extremities but can present anywhere on body
              • LM
                • Present as soft, nonpulsatile, uniocular or multilocular cystic lesions
                • Often present with swelling, and depending on location, mechanical dysfunction or cosmetic disfigurement
                • Can become swollen and painful when locally infected or with systemic infection
                • Vary in size from microcystic to macrocystic (> 1-2 cm) or mixed type
                • Can be isolated or associated with genetic syndrome (e.g., Turner, Noonan)
                • Most often present in head and neck, but chest, axilla, and perineum also common sites
              • CM
                • Present as pink-red patches but may darken, thicken, and become more nodular with time
                • Can be isolated or coexist with other VMFs, or associated with genetic syndrome (e.g., Kippel-Trénaunay, Sturge-Weber, Parkes Weber)
                • Physical exam and, in some cases, imaging should exclude underlying malformations
          • High-Flow Malformations: Clinical Features, Noncerebrovascular

            • Presentation and physical exam
              • AVM
                • Present as warm, palpable mass with ill-defined border and reddish discoloration of overlying skin
                • Surrounding fat tissue most often present
                • Often accompanied by palpable thrill/bruit
                • Most commonly present in limbs, trunk, and viscera
                • Bleeding, ulceration of surrounding soft tissue and high-output heart failure in advanced stages
                • May cause "steal" from surrounding or downstream organs
                • May lead heart failure due to high cardiac output
              • AVF
                • Present as palpable, pulsatile mass often associated with bruit and perivascular thrill
                • Only congenital AVFs classified as VMFs: Most are acquired (e.g., traumatic or iatrogenic)
                • Congenital AVFs are rare, can occur anywhere in body and commonly manifest in infancy as high-output heart failure
                • Claudication, deep vein thrombosis, rest pain, and varicose veins may develop in extremities distal to lesion

          PREPROCEDURE

          • Preprocedure Imaging

            • Getting Started

              • Treatments: Low-Flow Malformations

                • Treatments: High-Flow Malformations

                  PROCEDURE

                  • Patient Position/Location

                    • Equipment Preparation

                      • Procedure Steps

                        • Alternative Procedures/Therapies

                          POST PROCEDURE

                          • Things to Do

                            OUTCOMES

                            • Complications

                              • Expected Outcomes

                                Selected References

                                1. Azene E et al: Foamed bleomycin sclerosis of airway venous malformations: the role of interspecialty collaboration. Laryngoscope. 126(12):2726-2732, 2016
                                2. International Society for the Study of Vascular Anomalies: ISSVA classification of vascular anomalies. www.issva.org/classification. Published 2014. Accessed April 6, 2017
                                3. Higgins LJ et al: Time-resolved contrast-enhanced MRA (TWIST) with gadofosveset trisodium in the classification of soft-tissue vascular anomalies in the head and neck in children following updated 2014 ISSVA classification: first report on systematic evaluation of MRI and TWIST in a cohort of 47 children. Clin Radiol. 71(1):32-9, 2016
                                4. Ul Haq F et al: Bleomycin foam treatment of venous malformations: a promising agent for effective treatment with minimal swelling. J Vasc Interv Radiol. 26(10):1484-93, 2015
                                5. Tekes A et al: S.E. Mitchell Vascular Anomalies Flow Chart (SEMVAFC): a visual pathway combining clinical and imaging findings for classification of soft-tissue vascular anomalies. Clin Radiol. 69(5):443-57, 2014
                                6. Geschwind J et al: Abrams’ Angiography: Interventional Radiology. 3rd ed. In Geschwind J et al. Philadelphia: Lippincott, Williams, & Wilkins; 2013
                                7. Burrows PE: Endovascular treatment of slow-flow vascular malformations. Tech Vasc Interv Radiol. 16(1):12-21, 2013
                                8. Thawait SK et al: MR imaging characteristics of soft tissue vascular anomalies in children. Eur J Pediatr. 172(5):591-600, 2013
                                9. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                10. Do YS et al: Extremity arteriovenous malformations involving the bone: therapeutic outcomes of ethanol embolotherapy. J Vasc Interv Radiol. 21(6):807-16, 2010
                                11. Shin BS et al: Effects of repeat bolus ethanol injections on cardiopulmonary hemodynamic changes during embolotherapy of arteriovenous malformations of the extremities. J Vasc Interv Radiol. 21(1):81-9, 2010
                                12. Li L et al: Fluoroscopy-guided foam sclerotherapy with sodium morrhuate for peripheral venous malformations: Preliminary experience. J Vasc Surg. 49(4):961-7, 2009
                                13. Lee KB et al: Incidence of soft tissue injury and neuropathy after embolo/sclerotherapy for congenital vascular malformation. J Vasc Surg. 48(5):1286-91, 2008
                                14. Do YS et al: How do we treat arteriovenous malformations (tips and tricks)? Tech Vasc Interv Radiol. 10(4):291-8, 2007
                                15. Lee BB et al: Congenital vascular malformations: general diagnostic principles. Phlebology. 22(6):253-7, 2007
                                16. Cho SK et al: Arteriovenous malformations of the body and extremities: analysis of therapeutic outcomes and approaches according to a modified angiographic classification. J Endovasc Ther. 13(4):527-38, 2006
                                17. Do YS et al: Ethanol embolization of arteriovenous malformations: interim results. Radiology. 235(2):674-82, 2005
                                18. Shin BS et al: Multistage ethanol sclerotherapy of soft-tissue arteriovenous malformations: effect on pulmonary arterial pressure. Radiology. 235(3):1072-7, 2005
                                19. Herborn CU et al: Comprehensive time-resolved MRI of peripheral vascular malformations. AJR Am J Roentgenol. 181(3):729-35, 2003
                                20. Dubois J et al: Soft-tissue venous malformations in adult patients: imaging and therapeutic issues. Radiographics. 21(6):1519-31, 2001
                                21. Yakes WF: Yakes’ AVM classification system. Journal of Vascular and Interventional Radiology. 26(2):S224. 2014
                                22. Baum S et al: Abrams' Angiography: Interventional Radiology. 2nd ed. In: Baum S et al. Philadelphia: Lippincott, Williams, and Wilkins; 2006
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Preprocedure

                                    • Procedure

                                      • Outcomes

                                        TERMINOLOGY

                                        • Definitions

                                          • Vascular malformation (VMF): Most common vascular anomaly consisting of abnormal clusters of blood vessels without endothelial proliferation
                                            • Low flow: Capillary, venous, lymphatic or "mixed" malformations
                                              • Venous malformations (VM)
                                                • Most common type of VMF consisting of dilated venous vessels with abnormal smooth muscle, which allows for expansion of VM over time
                                                • May or may not communicate with systemic vasculature
                                                • Usually sporadic but can occur as part of genetic syndrome (e.g., Klippel-Trénaunay)
                                                • Diverse range of size, location, and appearance: From small, solitary, and localized to skin and subcutaneous tissues to diffuse masses extending across multiple tissue planes and surrounding vascular, nervous, and visceral bodies
                                              • Lymphatic malformation (LM)
                                                • 2nd most common VMF consisting of benign cystic lesions made up of lymphatic vessels filled with lymphatic fluid
                                                • Most commonly sporadic; however, some associated with genetic syndromes (e.g., Turner, Noonan)
                                                • Classified based on cyst size as macrocystic (large), microcystic (small), or mixed
                                                • Previously used terms such as lymphangiomas and cystic hygromas should be avoided
                                              • Capillary malformation (CM)
                                                • Limited to superficial skin layers, formerly called port-wine stains
                                                • Can darken, thicken, and become nodular if left untreated
                                                • Often associated with congenital syndrome (e.g., Sturge-Weber, Parkes Weber, Klippel-Trénaunay)
                                            • High flow: Direct communication between arteries and veins
                                              • Arteriovenous malformations (AVM)
                                                • Complex communicating network of arterial and venous vessels
                                                • Typically more invasive than other VMFs
                                                • Arterial inflow diverted via nidus into draining veins, causing tissue ischemia and venous hypertension
                                                • High recurrence rate after treatment with total nidus eradication considered "curative"
                                              • Arteriovenous fistula (AVF)
                                                • Direct abnormal communication between artery and vein without interposed nidus
                                                • Can be acquired (most common) or congenital
                                                • Limb ischemia can occur distal to AVF due to arterial blood shunted via fistula (steal phenomenon)
                                            • Syndromes associated with VMFs
                                              • Klippel-Trénaunay syndrome: VM combined with LM and CM
                                              • Parkes Weber syndrome: VM combined with LM, CM, and AVM
                                              • Sturge-Weber syndrome: Cutaneous CM along distribution of facial and ocular nerves; leptomeningeal vascular anomalies (CM, VM)
                                        • Classification

                                          • International Society for the Study of Vascular Anomalies (ISSVA) classification (revised 2014)
                                            • Most widely recognized classification system based on cellular features, flow characteristics, associated clinical features and genetics
                                            • Defines common language used by all members of interdisciplinary teams involved in care of vascular anomalies
                                            • Broadly divides vascular anomalies into nonneoplastic lesions (VMFs) and neoplastic lesions (vascular tumors)
                                          • SE Mitchell Vascular Anomalies Flow Chart (SEMVAFC)
                                            • Combines clinical and imaging findings for accurate VMF diagnosis
                                            • Utilizes classification schema similar to ISSVA to preserve common language
                                            • Potential to identify syndromes associated with VMF
                                          • Yakes classification
                                            • Organizes AVMs by angioarchitecture and ascribes specific endovascular treatment options for each subtype
                                        • Low-Flow Malformations: Clinical Features

                                          • Presentation & physical exam
                                            • VM
                                              • Present as soft, compressible, nonpulsatile, bluish lesions
                                              • Often present with pain, swelling, and depending on location, mechanical dysfunction or cosmetic disfigurement
                                              • Vary in size and distribution; potentially involves multiple anatomic locations
                                              • Majority are solitary but can be associated with genetic syndrome (e.g., blue rubber bleb nevus syndrome)
                                              • Can occur in any anatomic depth (i.e., skin, subcutaneous tissue, muscle, etc.) and are often infiltrative and extend to underlying or nearby structures
                                              • Placement of affected area in dependent position or Valsalva maneuver may elicit blood pooling and subsequent enlargement of lesion
                                              • Most commonly occur in head, neck, and extremities but can present anywhere on body
                                            • LM
                                              • Present as soft, nonpulsatile, uniocular or multilocular cystic lesions
                                              • Often present with swelling, and depending on location, mechanical dysfunction or cosmetic disfigurement
                                              • Can become swollen and painful when locally infected or with systemic infection
                                              • Vary in size from microcystic to macrocystic (> 1-2 cm) or mixed type
                                              • Can be isolated or associated with genetic syndrome (e.g., Turner, Noonan)
                                              • Most often present in head and neck, but chest, axilla, and perineum also common sites
                                            • CM
                                              • Present as pink-red patches but may darken, thicken, and become more nodular with time
                                              • Can be isolated or coexist with other VMFs, or associated with genetic syndrome (e.g., Kippel-Trénaunay, Sturge-Weber, Parkes Weber)
                                              • Physical exam and, in some cases, imaging should exclude underlying malformations
                                        • High-Flow Malformations: Clinical Features, Noncerebrovascular

                                          • Presentation and physical exam
                                            • AVM
                                              • Present as warm, palpable mass with ill-defined border and reddish discoloration of overlying skin
                                              • Surrounding fat tissue most often present
                                              • Often accompanied by palpable thrill/bruit
                                              • Most commonly present in limbs, trunk, and viscera
                                              • Bleeding, ulceration of surrounding soft tissue and high-output heart failure in advanced stages
                                              • May cause "steal" from surrounding or downstream organs
                                              • May lead heart failure due to high cardiac output
                                            • AVF
                                              • Present as palpable, pulsatile mass often associated with bruit and perivascular thrill
                                              • Only congenital AVFs classified as VMFs: Most are acquired (e.g., traumatic or iatrogenic)
                                              • Congenital AVFs are rare, can occur anywhere in body and commonly manifest in infancy as high-output heart failure
                                              • Claudication, deep vein thrombosis, rest pain, and varicose veins may develop in extremities distal to lesion

                                        PREPROCEDURE

                                        • Preprocedure Imaging

                                          • Getting Started

                                            • Treatments: Low-Flow Malformations

                                              • Treatments: High-Flow Malformations

                                                PROCEDURE

                                                • Patient Position/Location

                                                  • Equipment Preparation

                                                    • Procedure Steps

                                                      • Alternative Procedures/Therapies

                                                        POST PROCEDURE

                                                        • Things to Do

                                                          OUTCOMES

                                                          • Complications

                                                            • Expected Outcomes

                                                              Selected References

                                                              1. Azene E et al: Foamed bleomycin sclerosis of airway venous malformations: the role of interspecialty collaboration. Laryngoscope. 126(12):2726-2732, 2016
                                                              2. International Society for the Study of Vascular Anomalies: ISSVA classification of vascular anomalies. www.issva.org/classification. Published 2014. Accessed April 6, 2017
                                                              3. Higgins LJ et al: Time-resolved contrast-enhanced MRA (TWIST) with gadofosveset trisodium in the classification of soft-tissue vascular anomalies in the head and neck in children following updated 2014 ISSVA classification: first report on systematic evaluation of MRI and TWIST in a cohort of 47 children. Clin Radiol. 71(1):32-9, 2016
                                                              4. Ul Haq F et al: Bleomycin foam treatment of venous malformations: a promising agent for effective treatment with minimal swelling. J Vasc Interv Radiol. 26(10):1484-93, 2015
                                                              5. Tekes A et al: S.E. Mitchell Vascular Anomalies Flow Chart (SEMVAFC): a visual pathway combining clinical and imaging findings for classification of soft-tissue vascular anomalies. Clin Radiol. 69(5):443-57, 2014
                                                              6. Geschwind J et al: Abrams’ Angiography: Interventional Radiology. 3rd ed. In Geschwind J et al. Philadelphia: Lippincott, Williams, & Wilkins; 2013
                                                              7. Burrows PE: Endovascular treatment of slow-flow vascular malformations. Tech Vasc Interv Radiol. 16(1):12-21, 2013
                                                              8. Thawait SK et al: MR imaging characteristics of soft tissue vascular anomalies in children. Eur J Pediatr. 172(5):591-600, 2013
                                                              9. Flors L et al: MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up. Radiographics. 31(5):1321-40; discussion 1340-1, 2011
                                                              10. Do YS et al: Extremity arteriovenous malformations involving the bone: therapeutic outcomes of ethanol embolotherapy. J Vasc Interv Radiol. 21(6):807-16, 2010
                                                              11. Shin BS et al: Effects of repeat bolus ethanol injections on cardiopulmonary hemodynamic changes during embolotherapy of arteriovenous malformations of the extremities. J Vasc Interv Radiol. 21(1):81-9, 2010
                                                              12. Li L et al: Fluoroscopy-guided foam sclerotherapy with sodium morrhuate for peripheral venous malformations: Preliminary experience. J Vasc Surg. 49(4):961-7, 2009
                                                              13. Lee KB et al: Incidence of soft tissue injury and neuropathy after embolo/sclerotherapy for congenital vascular malformation. J Vasc Surg. 48(5):1286-91, 2008
                                                              14. Do YS et al: How do we treat arteriovenous malformations (tips and tricks)? Tech Vasc Interv Radiol. 10(4):291-8, 2007
                                                              15. Lee BB et al: Congenital vascular malformations: general diagnostic principles. Phlebology. 22(6):253-7, 2007
                                                              16. Cho SK et al: Arteriovenous malformations of the body and extremities: analysis of therapeutic outcomes and approaches according to a modified angiographic classification. J Endovasc Ther. 13(4):527-38, 2006
                                                              17. Do YS et al: Ethanol embolization of arteriovenous malformations: interim results. Radiology. 235(2):674-82, 2005
                                                              18. Shin BS et al: Multistage ethanol sclerotherapy of soft-tissue arteriovenous malformations: effect on pulmonary arterial pressure. Radiology. 235(3):1072-7, 2005
                                                              19. Herborn CU et al: Comprehensive time-resolved MRI of peripheral vascular malformations. AJR Am J Roentgenol. 181(3):729-35, 2003
                                                              20. Dubois J et al: Soft-tissue venous malformations in adult patients: imaging and therapeutic issues. Radiographics. 21(6):1519-31, 2001
                                                              21. Yakes WF: Yakes’ AVM classification system. Journal of Vascular and Interventional Radiology. 26(2):S224. 2014
                                                              22. Baum S et al: Abrams' Angiography: Interventional Radiology. 2nd ed. In: Baum S et al. Philadelphia: Lippincott, Williams, and Wilkins; 2006