Zhuo Kevin Y, Russell Susan, Wargon Orli, Adams Susan
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.
J Paediatr Child Health. 2017 Aug;53(8):737-741. doi: 10.1111/jpc.13461. Epub 2017 Feb 7.
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.
静脉畸形是一种缓慢流动的先天性血管畸形，随着儿童年龄增长而增大，可能与局部血管内凝血有关，这是一种消耗性凝血病，其特征是D - 二聚体升高和纤维蛋白原水平降低。作者回顾了局部血管内凝血与静脉畸形数量、大小及受累平面之间已知的相关性，并提醒注意随着儿童年龄增长及其静脉畸形增大，局部血管内凝血病进展的概念。作者还讨论了已确定的针对其检查、管理和治疗的治疗选择，包括压迫衣、抗凝治疗、硬化治疗、血管内激光、手术切除和西罗莫司（雷帕霉素）。本文回顾了可能为优化伴有局部血管内凝血的静脉畸形的物理和药物治疗而推动的方案改进的证据。
