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Updated: Sep 3, 2026

Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Management of Combined Vascular Malformations
Ann M Kulungowski1, Samantha A Spencer2
1Division of Pediatric Surgery, Vascular Anomalies Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA.
Abstract:
Combined vascular malformations are complex, heterogeneous lesions increasingly classified by anatomic components and genetic drivers, specifically the PI3K/AKT/mTOR pathway (PROS) and RASA1 mutations. These syndromes, including Klippel-Trenaunay, CLOVES, and capillary malformation-arteriovenous malformation, present with soft-tissue hypertrophy, skeletal overgrowth, and varied vascular anomalies. Effective management requires a multidisciplinary approach to address complications such as thromboembolism, localized intravascular coagulopathy, and limb-length discrepancy. Diagnostic evaluation relies on ultrasound and MRI. Therapeutic interventions have evolved from conservative compression and sclerotherapy to targeted pharmacotherapy (sirolimus, alpelisib) and individualized surgical debulking, with the aim of improving functional outcomes and quality of life.
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