常染色体显性多囊肾病中脊髓脑脊膜憩室的患病率
Prevalence of Spinal Meningeal Diverticula in Autosomal Dominant Polycystic Kidney Disease.
作者信息
Sattar Usama, Yin Xiaorui, Luo Xianfu, Zhu Chenglin, Hu Zhongxiu, Blumenfeld Jon D, Rennert Hanna, Wu Alan, RoyChoudhury Arindam, Salama Gayle, Prince Martin R
机构信息
From the Department of Radiology, Weill Cornell Medicine (U.S., X.Y., X.L., C.Z., Z.H., G.S.), New York, New York.
Department of Medicine (J.D.B.), Weill Cornell Medicine, New York, New York.
出版信息
AJNR Am J Neuroradiol. 2025 Jan 8;46(1):200-206. doi: 10.3174/ajnr.A8407.
BACKGROUND AND PURPOSE
Patients with autosomal dominant polycystic kidney disease (ADPKD) develop cysts in the kidneys, liver, spleen, pancreas, prostate, and arachnoid spaces. In addition, spinal meningeal diverticula have been reported. To determine whether spinal meningeal diverticula are associated with ADPKD, we compared their prevalence in subjects with ADPKD with a control cohort without ADPKD.
MATERIALS AND METHODS
Subjects with ADPKD and age- and sex-matched controls without ADPKD undergoing abdominal MRI from the midthorax to the pelvis from 2003 to 2023 were retrospectively evaluated for spinal meningeal diverticula by 4 blinded observers. The prevalence of spinal meningeal diverticula in ADPKD was compared with that in control subjects, using tests and correlated with clinical and laboratory data and MR imaging features, including cyst volumes and cyst counts.
RESULTS
Identification of spinal meningeal diverticula in ADPKD ( = 285, median age, 47; interquartile range [IQR], 37-56 years; 54% female) and control ( = 285, median age, 47; IQR, 37-57 years; 54% female) subjects had high interobserver agreement (pairwise Cohen κ = 0.74). Spinal meningeal diverticula were observed in 145 of 285 (51%) subjects with ADPKD compared with 66 of 285 (23%) control subjects without ADPKD ( < .001). Spinal meningeal diverticula in ADPKD were more prevalent in women (98 of 153 [64%]) than men (47 of 132 [36%], < .001). The mean number of spinal meningeal diverticula per affected subject with ADPKD was 3.6 ± 2.9 compared with 2.4 ± 1.9 in controls with cysts ( < .001). The median volume (IQR, 25%-75%) of spinal meningeal diverticula was 400 (IQR, 210-740) mm in those with ADPKD compared with 250 (IQR, 180-440) mm in controls ( < .001). The mean spinal meningeal diverticulum diameter was greater in the sacrum (7.3 [SD, 4.1] mm) compared with thoracic (5.4 [SD, 1.8] mm) and lumbar spine (5.8 [SD, 2.0] mm), ( < .001), suggesting that hydrostatic pressure contributed to enlargement.
CONCLUSIONS
ADPKD has a high prevalence of spinal meningeal diverticula, particularly in women.
背景与目的
常染色体显性遗传性多囊肾病（ADPKD）患者的肾脏、肝脏、脾脏、胰腺、前列腺及蛛网膜下腔会出现囊肿。此外，已有报道称存在脊髓脊膜憩室。为确定脊髓脊膜憩室是否与ADPKD相关，我们比较了ADPKD患者与无ADPKD的对照队列中脊髓脊膜憩室的患病率。
材料与方法
回顾性评估了2003年至2023年期间接受从胸中部至骨盆的腹部MRI检查的ADPKD患者以及年龄和性别匹配的无ADPKD对照者，由4名盲法观察者对脊髓脊膜憩室进行评估。使用检验比较ADPKD患者与对照者中脊髓脊膜憩室的患病率，并将其与临床和实验室数据以及MR成像特征（包括囊肿体积和囊肿数量）相关联。
结果
在ADPKD患者（n = 285，中位年龄47岁；四分位间距[IQR]，37 - 56岁；54%为女性）和对照者（n = 285，中位年龄47岁；IQR，37 - 57岁；54%为女性）中识别脊髓脊膜憩室的观察者间一致性较高（两两Cohen κ = 0.74）。285例ADPKD患者中有145例（51%）观察到脊髓脊膜憩室，而285例无ADPKD的对照者中有66例（23%）观察到（P <.001）。ADPKD患者中的脊髓脊膜憩室在女性中（153例中的98例[64%]）比男性中（132例中的47例[36%]）更常见（P <.001）。每例受影响的ADPKD患者脊髓脊膜憩室的平均数量为3.6 ± 2.9个，而有囊肿的对照者为2.4 ± 1.9个（P <.001）。ADPKD患者中脊髓脊膜憩室的中位体积（IQR，25% - 75%）为400（IQR，210 - 740）mm³，对照者为250（IQR，180 - 440）mm³（P <.001）。骶部脊髓脊膜憩室的平均直径（7.3[标准差，4.1]mm）大于胸部（5.4[标准差，1.8]mm）和腰部脊柱（5.8[标准差，2.0]mm）（P <.001），提示静水压导致其增大。
结论
ADPKD患者中脊髓脊膜憩室的患病率较高，尤其是在女性中。
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