Johnson Craig M, Navarro Oscar M
Division of Interventional Radiology, Department of Radiology, Nemours Children's Hospital, Orlando, FL, USA.
Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada.
Pediatr Radiol. 2017 Aug;47(9):1196-1208. doi: 10.1007/s00247-017-3906-x. Epub 2017 Aug 4.
Vascular malformations are a heterogeneous group of entities, many of which present in the pediatric age group. Sonography plays a major role in the management of children with these vascular anomalies by providing information that helps in diagnosing them, in assessing lesion extent and complications, and in monitoring response to therapy. The interpretation of sonographic findings requires correlation with clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these patients into the appropriate management pathway. Some vascular malformations are part of combined vascular anomalies or are associated with syndromes that include other disorders, frequently limb overgrowth, and these are now being reclassified based on their underlying genetic mutation. Sonography has limitations in the evaluation of some vascular malformations and in these cases MR imaging might be considered the imaging modality of choice, particularly for lesions that are large, that involve multiple compartments or are associated with other soft-tissue and bone abnormalities. In this article, which is part 2 of a two-part series, the authors review the most relevant clinical and sonographic features of arteriovenous, capillary, venous and lymphatic malformations as well as vascular malformations that are part of more complex conditions or associated with syndromes, including Parkes-Weber syndrome, phosphatase and tensin homologue (PTEN) hamartoma tumor syndromes, Klippel-Trénaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies) syndrome, fibro-adipose vascular anomaly and Proteus syndrome.
血管畸形是一组异质性病变,其中许多在儿童期出现。超声检查在这些血管异常患儿的管理中发挥着重要作用,它能提供有助于诊断、评估病变范围及并发症以及监测治疗反应的信息。超声检查结果的解读需要与临床发现相关联,其中一些临床发现可在扫描时轻松获得。这必须与使用恰当的命名法和最新分类相结合,以便将这些患者归入合适的管理路径。一些血管畸形是复合血管异常的一部分,或与包括其他疾病(常见肢体过度生长)的综合征相关,目前正根据其潜在基因突变进行重新分类。超声检查在某些血管畸形的评估中存在局限性,在这些情况下,磁共振成像可能被视为首选的成像方式,特别是对于那些大的、累及多个腔隙或与其他软组织和骨骼异常相关的病变。在这篇文章(两部分系列的第2部分)中,作者回顾了动静脉畸形、毛细血管畸形、静脉畸形和淋巴管畸形以及属于更复杂情况或与综合征相关的血管畸形(包括帕克斯 - 韦伯综合征、磷酸酶和张力蛋白同源物(PTEN)错构瘤肿瘤综合征、克-特综合征、CLOVES(先天性脂肪瘤过度生长、血管畸形、表皮痣和骨骼异常)综合征、纤维脂肪血管异常和变形综合征)最相关的临床和超声特征。