Huston J, Torres V E, Wiebers D O, Schievink W I
Department of Diagnostic Radiology, Mayo Clinic, Rochester, MN 55905, USA.
J Am Soc Nephrol. 1996 Oct;7(10):2135-41. doi: 10.1681/ASN.V7102135.
The purpose of this study was to assess the value of magnetic resonance angiography (MRA) in the follow-up of patients with autosomal dominant polycystic kidney disease (ADPKD) and saccular intracranial aneurysms (ICA), the risk of MRA-defined growth of asymptomatic incidental ICA, and the rate of development of MRA-defined de novo ICA in these patients. Between 1989 and 1995, 15 asymptomatic incidental ICA measuring 1.5 to 6.5 mm in diameter, three symptomatic aneurysms, and one asymptomatic concurrent aneurysm were detected by MRA in this study in 18 patients from 15 families. Four-vessel cerebral angiography in the three patients with symptomatic ICA and autopsy in one patient with an asymptomatic incidental ICA did not reveal additional aneurysms undetected by MRA. Thirty MRA studies were obtained in 10 of the 15 patients with incidental ICA during a cumulative clinical follow-up of 500 months (mean, 33.3; range, 0 to 65 months). The cumulative interval between the initial and the last MRA was 306 months (mean, 30.6; range, 14 to 51 months). No change in aneurysmal size or development of de novo aneurysms was detected. Eight MRA studies were obtained in the three patients with symptomatic ICA during a cumulative clinical follow-up of 130 months (mean, 43.3; range, 23 to 64 months). The cumulative interval between the first and the last MRA was 95 months (mean, 31.7; range, 15 to 49 months). Development of de novo aneurysms was not detected. These results indicate that MRA is an appropriate technique to follow small asymptomatic incidental ICA in patients with ADPKD and that the risk for rapid growth of these aneurysms is low. Although the results of this study should be viewed as preliminary, they do not suggest a higher rate of development of de novo aneurysms or a higher frequency of multiple aneurysms in patients with ADPKD and ICA as compared with patients with sporadic ICA in the general population.
本研究的目的是评估磁共振血管造影(MRA)在常染色体显性遗传性多囊肾病(ADPKD)合并颅内囊状动脉瘤(ICA)患者随访中的价值、MRA所定义的无症状偶然发现的ICA生长风险,以及这些患者中MRA所定义的新发ICA的发生率。1989年至1995年间,本研究通过MRA在来自15个家庭的18例患者中检测到15个直径为1.5至6.5毫米的无症状偶然发现的ICA、3个有症状的动脉瘤和1个无症状的并发动脉瘤。3例有症状ICA患者的四血管脑血管造影以及1例无症状偶然发现ICA患者的尸检均未发现MRA未检测到的其他动脉瘤。在15例有偶然发现ICA的患者中的10例患者进行了30次MRA检查,累计临床随访500个月(平均33.3个月;范围0至65个月)。首次和最后一次MRA之间的累计间隔为306个月(平均30.6个月;范围14至51个月)。未检测到动脉瘤大小变化或新发动脉瘤。在3例有症状ICA的患者进行了8次MRA检查,累计临床随访130个月(平均43.3个月;范围23至64个月)。首次和最后一次MRA之间的累计间隔为95个月(平均31.7个月;范围15至49个月)。未检测到新发动脉瘤。这些结果表明,MRA是随访ADPKD患者中小的无症状偶然发现ICA的合适技术,并且这些动脉瘤快速生长的风险较低。尽管本研究结果应视为初步结果,但与普通人群中散发性ICA患者相比,它们并未提示ADPKD合并ICA患者中有更高的新发动脉瘤发生率或多发动脉瘤频率。