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腹腔镜辅助下肛门直肠拖出术治疗库拉里诺综合征

Laparoscopic-Assisted Anorectal Pull-Through for Currarino Syndrome.

作者信息

Li Qi, Zhang Zhen, Jiang Qian, Yan Yuchun, Xiao Ping, Ma Ya, Li Long

机构信息

Department of General Surgery, Capital Institute of Pediatrics, Beijing, China.

Department of Medical Genetics, Capital Institute of Pediatrics, Beijing, China.

出版信息

J Laparoendosc Adv Surg Tech A. 2020 Jul;30(7):826-833. doi: 10.1089/lap.2019.0779. Epub 2020 Apr 17.

Abstract

Currarino syndrome (CS) is characterized by the triad of sacral anomalies, presacral tumor, and anorectal malformation (ARM). This study evaluates the feasibility and outcomes of laparoscopic-assisted anorectal pull-through (LAARP) for CS. Children admitted for primary or redo repair of CS through LAARP between 2016 and 2019 were reviewed. The indication of redo included constipation with megarectosigmoid, residual presacral mass, anastomosis leak, and secondary fistula. ARM was corrected by excision of rectal stenosis, fistula, and its associated megarectosigmoid. This was followed by a complete surgical resection of the presacral tumor, with subsequent pull-through and anocolic anastomosis. This was done with a combined laparoscopic and transanal approach. The detailed surgical techniques, early postoperative complications, and mid-term functional outcomes were summarized. Fourteen patients underwent LAARP for primary ( = 4) and redo ( = 10) repair of CS. Four of them had colostomy previously. Mean age at operation was 20.7 ± 13.9 months. Mean operative time was 120 ± 25 minutes. Median hospital stay was 8 days (range 7-9 days) postoperatively. None of the patients developed early postoperative complications such as anastomotic leaks, presacral abscesses, recurrent fistulas, and residual mass. Bowel function was assessed 1 year after LAARP in 10 patients. Mean follow-up time was 15.9 months. The frequency of bowel movements was 3.3 ± 1.5/day. Constipation occurred in 2 patients. Occasional soiling (<3 times/week) was reported in 4 patients and frequent soiling (>3 times/week) was in 1. LAARP for CS is safe and effective.

摘要

库拉里诺综合征(CS)的特征是骶骨异常、骶前肿瘤和肛门直肠畸形(ARM)三联征。本研究评估了腹腔镜辅助肛门直肠拖出术(LAARP)治疗CS的可行性和疗效。回顾了2016年至2019年间因原发性或再次手术通过LAARP治疗CS而入院的儿童。再次手术的指征包括伴有巨直肠乙状结肠的便秘、残留骶前肿块、吻合口漏和继发性瘘管。通过切除直肠狭窄、瘘管及其相关的巨直肠乙状结肠来纠正ARM。随后对骶前肿瘤进行完整的手术切除,接着进行拖出术和结肠肛管吻合术。这通过腹腔镜和经肛门联合入路完成。总结了详细的手术技术、术后早期并发症和中期功能结局。14例患者接受了LAARP进行CS的原发性(n = 4)和再次手术(n = 10)修复。其中4例先前有结肠造口术。手术时的平均年龄为20.7±13.9个月。平均手术时间为120±25分钟。术后中位住院时间为8天(范围7 - 9天)。所有患者均未出现术后早期并发症,如吻合口漏、骶前脓肿、复发性瘘管和残留肿块。10例患者在LAARP术后1年评估肠道功能。平均随访时间为15.9个月。排便频率为3.3±1.5次/天。2例患者出现便秘。4例患者报告偶尔弄脏(<3次/周),1例患者频繁弄脏(>3次/周)。LAARP治疗CS是安全有效的。

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