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
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
Azene E et al: Foamed bleomycin sclerosis of airway venous malformations: the role of interspecialty collaboration. Laryngoscope. 126(12):2726-2732, 2016
International Society for the Study of Vascular Anomalies: ISSVA classification of vascular anomalies. www.issva.org/classification. Published 2014. Accessed April 6, 2017
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
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
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
Geschwind J et al: Abrams’ Angiography: Interventional Radiology. 3rd ed. In Geschwind J et al. Philadelphia: Lippincott, Williams, & Wilkins; 2013
Thawait SK et al: MR imaging characteristics of soft tissue vascular anomalies in children. Eur J Pediatr. 172(5):591-600, 2013
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
Do YS et al: Extremity arteriovenous malformations involving the bone: therapeutic outcomes of ethanol embolotherapy. J Vasc Interv Radiol. 21(6):807-16, 2010
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
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
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
Do YS et al: How do we treat arteriovenous malformations (tips and tricks)? Tech Vasc Interv Radiol. 10(4):291-8, 2007
Lee BB et al: Congenital vascular malformations: general diagnostic principles. Phlebology. 22(6):253-7, 2007
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
Do YS et al: Ethanol embolization of arteriovenous malformations: interim results. Radiology. 235(2):674-82, 2005
Shin BS et al: Multistage ethanol sclerotherapy of soft-tissue arteriovenous malformations: effect on pulmonary arterial pressure. Radiology. 235(3):1072-7, 2005
Herborn CU et al: Comprehensive time-resolved MRI of peripheral vascular malformations. AJR Am J Roentgenol. 181(3):729-35, 2003
Dubois J et al: Soft-tissue venous malformations in adult patients: imaging and therapeutic issues. Radiographics. 21(6):1519-31, 2001
Yakes WF: Yakes’ AVM classification system. Journal of Vascular and Interventional Radiology. 26(2):S224. 2014
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
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
Azene E et al: Foamed bleomycin sclerosis of airway venous malformations: the role of interspecialty collaboration. Laryngoscope. 126(12):2726-2732, 2016
International Society for the Study of Vascular Anomalies: ISSVA classification of vascular anomalies. www.issva.org/classification. Published 2014. Accessed April 6, 2017
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
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
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
Geschwind J et al: Abrams’ Angiography: Interventional Radiology. 3rd ed. In Geschwind J et al. Philadelphia: Lippincott, Williams, & Wilkins; 2013
Thawait SK et al: MR imaging characteristics of soft tissue vascular anomalies in children. Eur J Pediatr. 172(5):591-600, 2013
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
Do YS et al: Extremity arteriovenous malformations involving the bone: therapeutic outcomes of ethanol embolotherapy. J Vasc Interv Radiol. 21(6):807-16, 2010
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
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
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
Do YS et al: How do we treat arteriovenous malformations (tips and tricks)? Tech Vasc Interv Radiol. 10(4):291-8, 2007
Lee BB et al: Congenital vascular malformations: general diagnostic principles. Phlebology. 22(6):253-7, 2007
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
Do YS et al: Ethanol embolization of arteriovenous malformations: interim results. Radiology. 235(2):674-82, 2005
Shin BS et al: Multistage ethanol sclerotherapy of soft-tissue arteriovenous malformations: effect on pulmonary arterial pressure. Radiology. 235(3):1072-7, 2005
Herborn CU et al: Comprehensive time-resolved MRI of peripheral vascular malformations. AJR Am J Roentgenol. 181(3):729-35, 2003
Dubois J et al: Soft-tissue venous malformations in adult patients: imaging and therapeutic issues. Radiographics. 21(6):1519-31, 2001
Yakes WF: Yakes’ AVM classification system. Journal of Vascular and Interventional Radiology. 26(2):S224. 2014
Baum S et al: Abrams' Angiography: Interventional Radiology. 2nd ed. In: Baum S et al. Philadelphia: Lippincott, Williams, and Wilkins; 2006
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