Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55901, USA.
Clin J Am Soc Nephrol. 2011 Jun;6(6):1274-85. doi: 10.2215/CJN.09731110. Epub 2011 May 5.
Autosomal dominant polycystic kidney disease (ADPKD) patients have an increased risk for intracranial aneurysms (IAs). The importance of screening for unruptured IAs (UIAs) depends on their risks for growth and rupture.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: ADPKD patients with UIAs found by presymptomatic screening with magnetic resonance angiography (MRA) during 1989 to 2009 were followed initially at 6 months and annually, and less frequently after demonstration of stability.
Forty-five saccular aneurysms were detected in 38 patients from 36 families. Most were small (median diameter 3.5 mm) and in the anterior circulation (84%). Median age at diagnosis was 49 years. During cumulative imaging follow-up of 243 years, one de novo UIA was detected and increased in size from 2 to 4.4 mm over 144 months and two UIAs grew from 4.5 to 5.9 mm and 4.7 to 6.2 mm after 69 and 184 months, respectively. Seven patients did not have imaging follow-up. No change was detected in the remaining 28 patients. During cumulative clinical follow-up of 316 years, no aneurysm ruptured. Five patients died from unrelated causes and two were lost to follow-up after 8 and 120 months. Three patients underwent surgical clipping.
Most UIAs detected by presymptomatic screening in ADPKD patients are small and in the anterior circulation. Growth and rupture risks are not higher than those of UIAs in the general population. These data support very selective screening for UIAs in ADPKD patients, and widespread screening is not indicated.
常染色体显性多囊肾病（ADPKD）患者颅内动脉瘤（IAs）的风险增加。筛查未破裂颅内动脉瘤（UIAs）的重要性取决于其生长和破裂的风险。
设计、设置、参与者和测量方法：1989 年至 2009 年间，通过磁共振血管造影（MRA）进行症状前筛查发现有 UIAs 的 ADPKD 患者，最初每 6 个月和每年随访一次，在稳定后随访频率降低。
从 36 个家族中的 38 名患者中检测到 45 个囊状动脉瘤。大多数动脉瘤较小（直径中位数为 3.5 毫米），位于前循环（84%）。诊断时的中位年龄为 49 岁。在 243 年的累积影像学随访中，发现了一个新的 UIAs，其大小从 2 毫米增加到 4.4 毫米，历时 144 个月，两个 UIAs 分别从 4.5 毫米增长到 5.9 毫米，从 4.7 毫米增长到 6.2 毫米，历时分别为 69 个月和 184 个月。7 名患者没有影像学随访。其余 28 名患者未发现变化。在 316 年的累积临床随访中，没有动脉瘤破裂。5 名患者死于非相关原因，2 名患者在 8 个月和 120 个月后失访。3 名患者接受了手术夹闭。
在 ADPKD 患者中通过症状前筛查发现的大多数 UIAs 较小，位于前循环。生长和破裂的风险并不高于一般人群的 UIAs。这些数据支持对 ADPKD 患者进行非常选择性的 UIAs 筛查，广泛筛查是不必要的。
