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合并库拉里诺综合征和尾部重复畸形患者巨大骶前脊膜膨出的神经外科治疗:病例说明

Neurosurgical management of a giant anterior sacral meningocele in a patient with combined Currarino syndrome and caudal duplication anomaly: illustrative case.

作者信息

Shah Khushi H, Tigchelaar Seth S, Khalafallah Adham M, Levi Allan D

机构信息

Department of Neurological Surgery, University of Miami Miller School of Medicine, Miami, Florida.

出版信息

J Neurosurg Case Lessons. 2025 Jun 9;9(23). doi: 10.3171/CASE2572.

Abstract

BACKGROUND

Anterior sacral meningoceles (ASMs) are rare spinal lesions that may be associated with Currarino syndrome, a condition defined by the triad of anorectal malformations, sacral anomalies, and a presacral mass. While Currarino syndrome and caudal duplication syndrome are distinct entities, their coexistence is exceptionally rare, with only 2 reported cases-none involving an ASM. The authors present a unique case of an ASM in a patient with Currarino syndrome and features of caudal duplication anomaly.

OBSERVATIONS

The patient presented with chronic low back pain, paresthesia in the lateral right thigh, and worsening renal function due to hydronephrosis of her solitary left kidney. Imaging revealed a large ASM compressing the left ureter, resulting in severe hydronephrosis and elevated creatinine levels. Surgical intervention included sacral laminectomy, spinal cord untethering, sectioning of the filum terminale, and disconnection of the fistulous connection between the spinal cord and the sacral meningocele. Postoperatively, her renal function improved, and her headache, paresthesia, and back pain significantly resolved.

LESSONS

This case underscores the rare coexistence of Currarino syndrome and caudal duplication anomaly, providing valuable insights into the management of an ASM in this setting. https://thejns.org/doi/10.3171/CASE2572.

摘要

背景

骶前脊膜膨出(ASMs)是一种罕见的脊柱病变,可能与库拉里诺综合征相关,该综合征由肛门直肠畸形、骶骨异常和骶前肿块三联征定义。虽然库拉里诺综合征和尾侧重复综合征是不同的疾病,但它们同时存在极为罕见,仅有2例报道,均未涉及骶前脊膜膨出。作者报告了1例患有库拉里诺综合征且具有尾侧重复畸形特征的骶前脊膜膨出患者的独特病例。

观察结果

该患者表现为慢性下腰痛、右大腿外侧感觉异常,以及因左侧孤立肾肾盂积水导致的肾功能恶化。影像学检查显示一个巨大的骶前脊膜膨出压迫左侧输尿管,导致严重肾盂积水和肌酐水平升高。手术干预包括骶椎板切除术、脊髓松解术、终丝切断术以及脊髓与骶部脊膜膨出之间瘘管连接的切断。术后,她的肾功能得到改善,头痛、感觉异常和背痛明显缓解。

经验教训

该病例强调了库拉里诺综合征和尾侧重复畸形罕见的共存情况,为这种情况下骶前脊膜膨出的管理提供了有价值的见解。https://thejns.org/doi/10.3171/CASE2572。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3909/12147661/e3fb25276788/CASE2572_figure_1.jpg

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