Department of Paediatric Dermatology, Sydney Children's Hospital, Sydney, New South Wales, Australia.
Australas J Dermatol. 2009 Nov;50(4):276-80. doi: 10.1111/j.1440-0960.2009.00558.x.
We present two female children aged 7 years with extensive multifocal venous malformations complicated by chronic localized intravascular coagulation. In both cases ultrasonography and magnetic resonance imaging revealed extensive venous malformations involving the skin, mucous membranes and pharynx, which were not apparent on clinical examination. Haematological investigations demonstrated persistent elevation of the D-dimer, consistent with chronic localized intravascular coagulation. The course of one patient was complicated by the development of multiple painful thromboses at distant sites following percutaneous sclerotherapy. Persistent elevation of the D-dimer occurring in association with large venous and veno-lymphatic malformations has been termed chronic localized intravascular coagulation, and is thought to occur due to thrombosis at sites of stagnant blood flow within venous malformations. It is of clinical concern due to the potential for transformation into serious thrombohaemorrhagic coagulation disorders, including disseminated intravascular coagulation. While previously described in association with large segmental venous malformations, these cases demonstrate the occurrence of chronic localized intravascular coagulation as a complication of disseminated multifocal venous malformations.
我们报告了两例 7 岁女性儿童，患有广泛的多灶性静脉畸形，并伴有慢性局部血管内凝血。在这两种情况下，超声检查和磁共振成像均显示广泛的静脉畸形累及皮肤、粘膜和咽部，而临床检查则不明显。血液学检查显示 D-二聚体持续升高，符合慢性局部血管内凝血。其中一例患者在经皮硬化治疗后，远处多处疼痛性血栓形成，病程复杂。持续性 D-二聚体升高与大静脉和静脉淋巴管畸形相关，被称为慢性局部血管内凝血，据认为是由于静脉畸形内血流停滞部位的血栓形成所致。由于有转化为严重血栓出血性凝血障碍的潜在风险，包括弥散性血管内凝血，因此具有临床意义。虽然之前曾描述过大段静脉畸形相关，但这些病例表明，慢性局部血管内凝血是弥散性多灶性静脉畸形的并发症。
