Anticoagulation and vascular anomalies

Abstract

A State of the Art lecture titled "Anticoagulation and Vascular Anomalies" was presented at the International Society on Thrombosis and Haemostasis (ISTH) Congress in 2023. Vascular anomalies have been classified by the International Society for the Study of Vascular Anomalies into vascular tumors and vascular malformations. Although some vascular tumors, such as tufted angioma and kaposiform hemangioendothelioma, and other vascular malformations can present with coagulation aberrancies, these are not generally managed with anticoagulation. A subclassification of vascular malformations includes slow-flow vascular malformations. It is this subgroup specifically that has a high risk of venous thromboembolism (VTE) and morbidity associated with coagulopathy that may be present. In these select cases, anticoagulation may be indicated to reduce the risk of VTE, treat VTE, or manage localized thrombosis in the malformation that causes significant pain and reduced quality of life. There are established risk factors for VTE in these patients that will be reviewed. Finally, we summarize relevant new data on this topic presented during the 2023 ISTH Congress.

Keywords: anticoagulant; coagulopathy; sclerotherapy; vascular malformation.

Figure 1

Patient with extensive multifocal venous malformation of his torso due to somatic TEK mutation. He has severe localized intravascular coagulopathy with hypofibrinogenemia, elevated D-dimer, and mild thrombocytopenia that has responded well to chronic anticoagulation with enoxaparin initially and, now, rivaroxaban.

Figure 2

Clinical photographs (black arrows) and magnetic resonance imaging (white arrows) of lateral marginal vein in a child with PIK3CA-related overgrowth syndrome. The patient has subsequently undergone several sclerotherapy procedures to ensure closure of the aberrant vein.

Figure 3

Proposed periprocedural management of localized intravascular coagulopathy to reduce risk of venous thromboembolism (VTE). aPTT, activated partial thromboplastin time; CBC, complete blood count; LMWH, low-molecular-weight heparin; PT, prothrombin time.