Section of Radiology, Di.M.I.M.P., HHT Interdepartmental Centre, University Hospital, Policlinico of Bari, Piazza Giulio Cesare 11, 70124, Bari, Italy.
Radiol Med. 2012 Feb;117(1):29-45. doi: 10.1007/s11547-011-0688-3. Epub 2011 Jun 4.
Hereditary haemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a rare autosomal dominant disorder characterised by mucocutaneous or visceral vascular abnormalities that may be widely distributed throughout the cardiovascular system. The purpose of this study was to compare multislice computed tomography angiography (MSCTA) and 4D dynamic contrast-enhanced magnetic resonance angiography (D-MRA) for evaluating vascular hepatic involvement in patients with HHT.
Fifty-two consecutive HHT patients underwent MSCTA and D-MRA examinations for systematic analysis of vascular visceral involvement. The images from the two techniques were reviewed independently by two expert radiologists to identify the following vascular abnormalities: telangiectases or large vascular masses; perfusion disorders [transient hepatic attenuation differences (THADs)]; hepatic arteriovenous malformations (HAVMs). Data, as well as diameters of the common hepatic artery and portal vein, were compared with Cohen's kappa statistic, Student's t test and receiver operating characteristic (ROC) curve analysis, as appropriate.
Both MSCTA and D-MRA detected one or more of the following hepatic vascular abnormalities in 36/52 cases (telangiectases in 29/52, THADs in 23/52 and HAVMs in 25/52[CE1]). A good concordance was found between the two techniques when determining the type of hepatic shunt (κ=0.9). No statistically significant differences were found when comparing mean common hepatic artery and portal vein diameters (p=0.09 and 0.22, respectively) and their accuracy in predicting HAVMs.
D-MRA has the same diagnostic accuracy as MSCTA and has the advantage of being less invasive due to the absence of ionising radiation.
遗传性出血性毛细血管扩张症（HHT），也称为 Rendu-Osler-Weber 病，是一种罕见的常染色体显性遗传疾病，其特征为黏膜皮肤或内脏血管异常，这些异常可能广泛分布于心血管系统中。本研究的目的是比较多层螺旋 CT 血管造影（MSCTA）和 4D 动态对比增强磁共振血管造影（D-MRA）在评估 HHT 患者血管肝内受累方面的价值。
52 例连续 HHT 患者接受 MSCTA 和 D-MRA 检查，对血管内脏受累进行系统分析。两位专家放射科医生独立对两种技术的图像进行了回顾，以识别以下血管异常：毛细血管扩张或大血管肿块；灌注障碍[短暂性肝衰减差异（THADs）]；肝动静脉畸形（HAVM）。数据以及肝总动脉和门静脉的直径，均通过 Cohen's kappa 统计、Student's t 检验和受试者工作特征（ROC）曲线分析进行了比较。
MSCTA 和 D-MRA 在 36/52 例病例中均发现了一种或多种以下肝血管异常（29/52 例存在毛细血管扩张，23/52 例存在 THAD，25/52 例存在 HAVM[CE1]）。两种技术在确定肝分流类型时具有良好的一致性（κ=0.9）。比较肝总动脉和门静脉的平均直径及其预测 HAVM 的准确性时，两种技术之间没有统计学上的显著差异（p=0.09 和 0.22）。
D-MRA 与 MSCTA 具有相同的诊断准确性，并且由于没有电离辐射，因此具有侵入性更小的优势。
