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 Review prevalence and etiology of venous malformations Discuss appropriate workup and imaging 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 (Klippel- Trenaunay, 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 Not too much compression or extrav will occur. Vent needle path of least resistance some distance away from injecting needle

Sclerotherapy

Future directions?? Society for Interventional Radiology Annual Meeting March 2016

Summary Clinical/Imaging findings Treatment options Conservative/none Compression Sclerotherapy

dcrivard@cmh.edu