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Evaluation and treatment of Vascular Malformations Douglas C. Rivard, DO Chairman-Department of Radiology Children's Mercy Hospital Kansas City, Missouri Associate Professor, University of Missouri-Kansas City Adjunct Assistant Professor-Kansas University School of Medicine Disclosures I do not have a financial interest or other relationship with a commercial organization that may have an interest in the content of the educational activity. Learning objectives 1. Review prevalence and etiology of venous malformations 2. Discuss appropriate workup and imaging 3. Review indications and basic techniques for treatment Venous Malformations • Historically many misnomers—hemangioma, birthmark, etc • Occur in about 1:10,000 births • Current classification schemes dating back to early 1980’s (ISSVA, Hamburg) Venous Malformations Almost 50% of referrals to vascular anomalies centers Studies show… • 70% of patients given the wrong initial dx • 20% patients receive improper initial therapy Hassanein AH, et al. Evaluation of terminology for vascular anomalies in current literature. Plast Reconstr Surg 2011;127(1):347-51 Venous Malformations • Abnormal collections of veins • Variable luminal diameter and wall thickness • Not “normal” veins • No elastic intima • Paucity or lack of smooth muscle Venous Malformations • Can occur anywhere • Deep, superficial, diffuse, localized, multiple • Associated with syndromes (KlippelTrenaunay, Parkes-Weber, Blue rubber bleb) Venous Malformations • Histologically • No elastic intima • paucity/absence of smooth muscle Clinical • Usually present with pain or swelling • Soft, compressible, variably blue tinged • Trans-spatial/compartmental Clinical • Dependent venous engorgement • Impinge on nerve/fascial tissues = pain • Bleeding/Hemarthrosis • Localized stasis in lesion = thrombosis/thrombophlebitis = pain (can form phleboliths) Imaging • US • First modality usually employed • Heterogenous but hypoechoic • Tubular anechoic structures/channels not always appreciated Imaging • Doppler • Monophasic flow most common • Biphasic or high velocity arterial flow are NOT typical (think AVM or AVF) Imaging Imaging • MRI • Define relationships to deeper critical structures • 3D reconstructions • Follow response to therapy • Consider time resolved MRA techniques Imaging • Radiographs occasionally to evaluate for bone overgrowth or remodeling (phleboliths seen about 16% of lesions) Imaging • Nuclear medicine not contemporarily used • Low spatial resolution • Lack of specificity Treatment Decisions • Conservative • Compression, ASA • Intervention • Sclerotherapy/embolization Treatment decisions • Bleeding • Lesions located at life or limb threatening region • Disabling pain • Limb length discrepancy/vascular bone syndrome Pre-treatment • Coag panel—make sure no consumptive coag issues • Define expectations--not a cure, multiple sessions is the norm • Back up from surgical/plastics/derm colleagues • Nerve block? Sclerotherapy Legiehn GM, et al Classification, diagnosis, and interventional radiologic management of vascular malformations. Orthop Clin North America 2006;37:435-74 Sclerotherapy • Choice of sclerosant • STS • EtOH • Polidocanol • n-CBA glue • Dwell time Sclerotherapy • US guided needle placement • Contrast injection to see confines of lesion and runoff • Many times more than one needle Sclerotherapy • Control of sclerosant • Compression of runoff if possible • Slow injection • Vent needle for larger lesions Sclerotherapy Future directions?? Society for Interventional Radiology Annual Meeting March 2016 Summary • Clinical/Imaging findings • Treatment options • Conservative/none • Compression • Sclerotherapy [email protected]