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库拉里诺综合征患儿手术入路的选择

Selection of operative approach in children with Currarino syndrome.

作者信息

Pang Wenbo, Chen Yajun, Wang Kai, Zhang Dan, Qi Xiang, Wu Dongyang

机构信息

Department of General Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China.

Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.

出版信息

Pediatr Surg Int. 2023 Jan 6;39(1):72. doi: 10.1007/s00383-022-05345-2.

Abstract

PURPOSE

To summarize the experience of surgical treatment of children diagnosed with Currarino syndrome, with an emphasis on the selection of an optimal operative approach.

METHODS

The clinical materials of patients diagnosed with Currarino syndrome were recorded. Special attention was given to the operative management, particularly the different routes for operation. The type of ARM was the critical point. The Rintala score was used for the evaluation of bowel function.

RESULTS

The medical records of 26 patients were reviewed. Seven were male, and 19 were female, with a mean age of 19.38 ± 13.80 months. The standard posterior sagittal approach (SPS) group included three perineal fistulae, one anal stenosis, one retraction of the rectum after anoplasty for vestibular fistula, one ARM with no fistula, one rectourethral fistula, and one cloaca. In the limited posterior sagittal approach (LPS) group, there were 13 perineal fistulae, one displacement of the rectum, and one retraction of the rectum after anoplasty for the vestibular fistula. In addition, the transanal approach (TA) and anterior sagittal approach (AS) were also used. The mean follow-up time was 39.48 ± 26.84 m. The Rintala score was 16.74 ± 2.93.

CONCLUSION

For a perineal fistula, SPS or LPS should be used to reach anoplasty and remove the presacral mass. For a vestibular fistula, the AS or LPS should be chosen. For anal stenosis, SPS or LPS should be used.

摘要

目的

总结小儿库拉里诺综合征的手术治疗经验,重点在于选择最佳手术入路。

方法

记录诊断为库拉里诺综合征患者的临床资料。特别关注手术管理,尤其是不同的手术途径。肛门直肠畸形的类型是关键要点。采用林塔拉评分评估肠道功能。

结果

回顾了26例患者的病历。男性7例,女性19例,平均年龄19.38±13.80个月。标准后矢状入路(SPS)组包括3例会阴部瘘、1例肛门狭窄、1例前庭瘘肛门成形术后直肠回缩、1例无瘘的肛门直肠畸形、1例直肠尿道瘘和1例泄殖腔畸形。在有限后矢状入路(LPS)组,有13例会阴部瘘、1例直肠移位和1例前庭瘘肛门成形术后直肠回缩。此外,还采用了经肛门入路(TA)和前矢状入路(AS)。平均随访时间为39.48±26.84个月。林塔拉评分为16.74±2.93。

结论

对于会阴部瘘,应采用SPS或LPS进行肛门成形术并切除骶前肿物。对于前庭瘘,应选择AS或LPS。对于肛门狭窄,应采用SPS或LPS。

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