Parkin Cameron James, Wilcox Chloe B, Chalasani Venu
Department of Urology, Royal North Shore Hospital, Northern Sydney Local Health District, NSW, Australia.
School of Medicine, The University of Notre Dame, Sydney, NSW, Australia.
Urol Case Rep. 2021 May 3;38:101703. doi: 10.1016/j.eucr.2021.101703. eCollection 2021 Sep.
Sacral Meningoceles, ductal ectasia and pseudomeningoceles are all rare spinal defects that occur due to errors in collagen biosynthesis in the setting of Marfan's Syndrome. Meningoceles, which are extradural collections of cerebrospinal fluid, can form large pelvic collections which can compress local structures. In rare cases, this can lead to extrinsic ureteric obstruction, which can result in acute renal failure and urosepsis. We present a case of a 35-year old female with Marfan's syndrome, with one of the largest sacral meningoceles reported in the literature, causing acute ureteric obstruction, requiring urgent surgical intervention.
骶部脑脊膜膨出、导管扩张和假性脑脊膜膨出都是罕见的脊柱缺陷，它们是在马凡综合征背景下胶原蛋白生物合成错误所导致的。脑脊膜膨出是脑脊液的硬膜外积聚，可形成巨大的盆腔积聚，从而压迫局部结构。在罕见情况下，这可导致外在性输尿管梗阻，进而引发急性肾衰竭和尿脓毒症。我们报告一例35岁患有马凡综合征的女性病例，其骶部脑脊膜膨出是文献报道中最大的之一，导致急性输尿管梗阻，需要紧急手术干预。
