Localised intravascular coagulation complicating venous malformations in children: Associations and therapeutic options.
Venous malformations are slow-flow congenital vascular malformations that enlarge as the child ages and may be associated with localised intravascular coagulation, a consumptive coagulopathy characterised by elevated D-dimer and decreased fibrinogen levels. The authors review the known correlations between localised intravascular coagulation and venous malformation number, size and planes involved, and call attention to the concept of the progression of localised intravascular coagulopathy as the child ages and their venous malformations enlarge. The authors also discuss the identified therapeutic options for its investigation, management and treatment, including compression garments, anti-coagulation therapy, sclerotherapy, endovascular laser, surgical excision and sirolimus (rapamycin). Evidence for protocol improvements that may be instigated for the optimal physical and medical therapy of venous malformations complicated by localised intravascular coagulopathy is reviewed.
Department of Paediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia.|School of Women's and Children's Health, University of New South Wales, Sydney, New South Wales, Australia.
Kevin Y
KY
Zhuo
School of Women's and Children's Health, University of New South Wales, Sydney, New South Wales, Australia.|Kid's Cancer Centre, Sydney Children's Hospital, Sydney, New South Wales, Australia.
Susan
S
Russell
School of Women's and Children's Health, University of New South Wales, Sydney, New South Wales, Australia.|Department of Paediatric Dermatology, Sydney Children's Hospital, Sydney, New South Wales, Australia.
Orli
O
Wargon
Department of Paediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia.|School of Women's and Children's Health, University of New South Wales, Sydney, New South Wales, Australia.|Neuroscience Research Australia, Sydney, New South Wales, Australia.
Susan
S
Adams
D000925:Anticoagulants / Q000627:therapeutic use*; D001241:Aspirin / Q000627:therapeutic use; D002648:Child; D004211:Disseminated Intravascular Coagulation / Q000097:blood* / Q000188:drug therapy*; D005337:Fibrin / Q000627:therapeutic use; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D012307:Risk Factors; D054079:Vascular Malformations / Q000209:etiology*
D000925:Anticoagulants; D005337:Fibrin; D006493:Heparin; D001241:Aspirin
aspirin; biomarker/blood; fibrin degradation product; heparin; risk factor; sirolimus