Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Pediatr Radiol. 2011 Mar;41(3):308-16. doi: 10.1007/s00247-010-1854-9. Epub 2010 Oct 16.
Fibromuscular dysplasia (FMD) and neurofibromatosis type 1 (NF1) are the most common causes of pediatric renal artery stenosis (RAS) in western countries, and characterization of their angiographic features could aid in an accurate diagnosis and in treatment.
This study characterizes renal angiographic findings in pediatric fibromuscular dysplasia (FMD) and neurofibromatosis type 1(NF1).
We reviewed 68 angiograms performed over 11 years on 43 children with renovascular hypertension (20 male, 23 females; ages 1 month to -19 years; median/average 9.8 years). Ten patients were diagnosed with NF1, and 33 had presumed FMD. The frequency, extent and distribution of lesions were determined and analyzed.
Stenosis was found in 91% of patients (n=39/43), with 86% showing stenosis of 1st or 2nd order arteries, and 12% distal to 2nd order. Stenoses in multiple 1st/2nd order arteries were found in 32% of patients, and 36/43 patients had 1-2 stenoses. The most common lesion was a ≤ 5 mm stenosis in a 1st/2nd order artery, in 42%. Mean percentage of stenosis in a 1st/2nd order vessel was 62%; ≥ 70% stenosis was found in 53%, and ≥ 90% stenosis in 29%. Bilateral disease was noted in 30% of patients. Intraparenchymal disease, distal to 2nd order branches, was seen in 30%. Aneurysms were seen in 28%, beading in 19% and collaterals in 51% (associated with ≥ 70% stenosis). Mid-aortic narrowing was seen in 16%, more often in patients with NF1.
We provide a descriptive characterization of renal angiographic findings in pediatric FMD and NF1.
纤维肌性发育不良（FMD）和 1 型神经纤维瘤病（NF1）是西方国家儿童肾动脉狭窄（RAS）的最常见原因，其血管造影特征的描述有助于准确诊断和治疗。
本研究描述了儿童纤维肌性发育不良（FMD）和 1 型神经纤维瘤病（NF1）的肾血管造影表现。
我们回顾了 11 年间对 43 例肾血管性高血压儿童（20 名男性，23 名女性；年龄 1 个月至 19 岁；中位数/平均年龄 9.8 岁）进行的 68 次血管造影。10 例患者诊断为 NF1，33 例患者诊断为 FMD。确定并分析了病变的频率、程度和分布。
91%的患者（n=39/43）存在狭窄，86%的狭窄发生在 1 级或 2 级动脉，12%的狭窄发生在 2 级动脉远端。32%的患者存在多处 1 级/2 级动脉狭窄，43 例患者中有 36 例存在 1-2 处狭窄。最常见的病变是 1 级/2 级动脉的≤5mm狭窄，占 42%。1 级/2 级血管的平均狭窄程度为 62%；53%的狭窄程度≥70%，29%的狭窄程度≥90%。30%的患者存在双侧病变。30%的患者存在 2 级分支远端的实质内病变。28%的患者存在动脉瘤，19%的患者存在串珠状表现，51%的患者存在侧支循环（与≥70%的狭窄相关）。16%的患者存在中主动脉狭窄，这种情况更常见于 NF1 患者。
我们提供了儿童 FMD 和 NF1 肾血管造影表现的描述性特征。
