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纤维脂肪血管异常(FAVA)的临床和影像学特征。

Clinical and imaging features in fibro-adipose vascular anomaly (FAVA).

机构信息

Division of Vascular and Interventional Radiology, Department of Radiology, University of Iowa Hospitals and Clinics, Carver College of Medicine, Iowa City, IA, USA.

Division of Interventional Radiology, Boston Children's Hospital, 333 Longwood Ave., Boston, MA, 02115, USA.

出版信息

Pediatr Radiol. 2020 Mar;50(3):380-387. doi: 10.1007/s00247-019-04571-6. Epub 2019 Dec 13.

Abstract

BACKGROUND

Fibro-adipose vascular anomaly (FAVA) is a rare and recently described complex vascular malformation. The clinical and imaging features and morphology can be confusing and often overlap with features of other vascular malformations and tumors.

OBJECTIVE

To present the imaging characterization of FAVA in association with clinical features.

MATERIALS AND METHODS

We retrospectively evaluated clinical and imaging (MRI, sonography and venography) findings in FAVA. We documented the clinical presentation, lesion morphology, imaging characteristics, tissue distribution, pattern of contrast enhancement and vascular characteristics.

RESULTS

Thirty-eight people (31 female, 7 male) ages 1-30 years (mean 12 years) were diagnosed with FAVA based on clinical findings combined with imaging or histopathological findings (n=17). Most lesions were in the lower extremity (n=36). Three patterns were noticed: focal, focal infiltrative and diffuse.

CONCLUSION

Fibro-adipose vascular anomaly, a rare and complex vascular malformation, has distinct clinical and imaging features that a radiologist should be familiar with to avoid delay in diagnosis and to direct appropriate management.

摘要

背景

纤维脂肪血管异常(FAVA)是一种罕见且最近描述的复杂血管畸形。其临床和影像学特征及形态可能具有混淆性,且常与其他血管畸形和肿瘤的特征重叠。

目的

呈现 FAVA 的影像学特征,并结合临床特征进行描述。

材料与方法

我们回顾性评估了 FAVA 的临床和影像学(MRI、超声和静脉造影)表现。我们记录了临床表现、病变形态、影像学特征、组织分布、对比增强模式和血管特征。

结果

根据临床发现结合影像学或组织病理学发现,我们诊断了 38 例(31 名女性,7 名男性)年龄为 1-30 岁(平均 12 岁)的 FAVA 患者(n=17)。大多数病变位于下肢(n=36)。注意到三种模式:局灶性、局灶性浸润性和弥漫性。

结论

纤维脂肪血管异常是一种罕见且复杂的血管畸形,具有独特的临床和影像学特征,放射科医生应该熟悉这些特征,以避免延误诊断,并指导进行适当的治疗。

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