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一名患有库拉里诺三联征的成年人骶前脊膜膨出和畸胎瘤的多学科手术治疗

Multidisciplinary surgical treatment of presacral meningocele and teratoma in an adult with Currarino triad.

作者信息

Chakhalian Daniel, Gunasekaran Arunprasad, Gandhi Gautam, Bradley Lucas, Mizell Jason, Kazemi Noojan

机构信息

Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

Department of Surgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

出版信息

Surg Neurol Int. 2017 May 10;8:77. doi: 10.4103/sni.sni_439_16. eCollection 2017.

Abstract

BACKGROUND

Currarino syndrome (CS) is a rare genetic condition that presents with the defining triad of anorectal malformations, sacral bone deformations, and presacral masses, which may include teratoma. Neurosurgeons are involved in the surgical treatment of anterior meningoceles, which are often associated with this condition. The accepted surgical treatment is a staged anterior-posterior resection of the presacral mass and obliteration of the anterior meningocele.

CASE DESCRIPTION

This case involved a 36-year-old female who presented with late onset of symptoms attributed to CS (e.g., presacral mass, anterior sacral meningocele, and sacral agenesis). She successfully underwent multidisciplinary single-stage approach for treatment of the anterior sacral meningocele and resection of the presacral mass. This required obliteration of the meningocele and closure of the dural defect. One year later, her meningocele had fully resolved.

CONCLUSION

While late presentations with CS are rare, early detection and multidisciplinary treatment including single-state anterior may be successful for managing these patients.

摘要

背景

库拉里诺综合征(CS)是一种罕见的遗传性疾病,其典型三联征为肛门直肠畸形、骶骨畸形和骶前肿物,后者可能包括畸胎瘤。神经外科医生参与前位脑脊膜膨出的手术治疗,这种疾病常与此综合征相关。公认的手术治疗方法是分期进行骶前肿物的前后联合切除术以及闭塞前位脑脊膜膨出。

病例描述

该病例为一名36岁女性,因CS出现迟发性症状(如骶前肿物、骶前脑脊膜膨出和骶骨发育不全)。她成功接受了多学科单阶段手术,治疗骶前脑脊膜膨出并切除骶前肿物。这需要闭塞脑脊膜膨出并封闭硬脑膜缺损。一年后,她的脑脊膜膨出完全消退。

结论

虽然CS的迟发性表现罕见,但早期发现并采用包括单阶段前路手术在内的多学科治疗方法可能成功治疗这些患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/caef/5445655/96b530bb5b82/SNI-8-77-g001.jpg

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