A patient with a large venous malformation of the trunk or limb may experience intralesional thrombosis which can result in a consumptive coagulopathy.


Thrombosis in the malformation is associated with acute pain.


The risk of thrombosis may be increased by blood stasis in large malformations, hypercoagulable state and/or trauma. Women are more often affected than men.


Features of ongoing thrombosis:

(1) elevated D-dimer

(2) decrease in platelet count

(3) decrease in fibrinogen

(4) decrease in von Willebrand factor


Extensive thrombosis can result in disseminated intravascular coagulopathy (DIC) comparable to the Kasabach-Merritt phenomenon (see above). These patients may experience a clinical bleeding tendency.


Therapy with low molecular weight heparin can reduce pain and prevent the thrombosis causing the consumptive process..


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