Management of Combined Vascular Malformations.

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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Journal
PubMed
Published
2026-10-01
DOI
https://doi.org/10.1016/j.cps.2026.05.011
Primary Topic
Vascular Malformations and Hemangiomas
Type
article
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article

Management of Combined Vascular Malformations.

Ann Marie Kulungowski, Samantha A. Spencer
PubMed
Vascular Malformations and Hemangiomas
article

Management of Combined Vascular Malformations.

Ann Marie Kulungowski, Samantha A. Spencer
article en

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.

PubMedVol. 53(4)
Boston Children's Hospital (US), Harvard University (US), Children's Hospital Colorado (US), University of Colorado Anschutz Medical Campus (US)
Openalex Percentile: Top 40%
Vascular Malformations and Hemangiomas
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Management of Combined Vascular Malformations. — Ann Marie Kulungowski, Samantha A. Spencer · PubMed (2026) | TGRS Research Map | TGRS