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库拉里诺综合征:连续五例患者报告。

Currarino syndrome: report of five consecutive patients.

作者信息

Duru Soner, Karabagli Hakan, Turkoglu Erhan, Erşahin Yusuf

机构信息

Department of Neurosurgery, Düzce University School of Medicine, Düzce, 81320, Turkey,

出版信息

Childs Nerv Syst. 2014 Mar;30(3):547-52. doi: 10.1007/s00381-013-2274-6. Epub 2013 Sep 8.

Abstract

INTRODUCTION

The Currarino syndrome is regarded as a developmental disorder based on its recognized etiological heterogeneity. This syndrome is thought to result from abnormal separation of the neuroectoderm from the endoderm. Our aim was to report the neurosurgical management of Currarino syndrome in children and adults and to describe what clinician could do if the Currarino triad was suspected.

CASE REPORTS

We present five cases of Currarino triad who underwent surgical intervention. All patients had sacral bony deformity, anorectal malformations, and anterior sacral meningocele. A 40-year-old-male had chronic constipation. He was incidentally diagnosed with Currarino syndrome. A 19-year-old-female suffered from a slight weakness in lower extremities and urinary incontinence. Her past medical history was remarkable for anal atresia. The other three cases were children.

CONCLUSION

When an anterior sacral meningocele is encountered, Currarino syndrome should be taken into consideration. Although it is rarity, the Currarino syndrome might be one of the causes of chronic constipation. Endoscopic or endoscope-assisted surgery via a posterior sacral route can be feasible for treatment of some of the patients with anterior sacral meningocele. Anterior meningocele pouch associated with Currarino syndrome will regresses over time following transdural ligation of its neck.

摘要

引言

由于其公认的病因异质性,库拉里诺综合征被视为一种发育障碍。该综合征被认为是神经外胚层与内胚层异常分离所致。我们的目的是报告儿童和成人库拉里诺综合征的神经外科治疗方法,并描述如果怀疑出现库拉里诺三联征临床医生可以采取的措施。

病例报告

我们介绍了5例接受手术干预的库拉里诺三联征患者。所有患者均有骶骨骨畸形、肛门直肠畸形和骶前脑脊膜膨出。一名40岁男性患有慢性便秘。他被偶然诊断为库拉里诺综合征。一名19岁女性下肢轻度无力且有尿失禁。她过去有肛门闭锁病史。另外3例为儿童。

结论

当遇到骶前脑脊膜膨出时,应考虑库拉里诺综合征。尽管罕见,但库拉里诺综合征可能是慢性便秘的病因之一。经骶后入路的内镜或内镜辅助手术对于一些骶前脑脊膜膨出患者的治疗可能是可行的。与库拉里诺综合征相关的前脑脊膜膨出囊在其颈部经硬膜结扎后会随时间消退。

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