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先天性巨结肠症的预后:无神经节细胞症长度的意义及扩张段切除长度的参考值

Hirschsprung's disease prognosis: significance of the length of aganglionosis and reference value for the dilated segment resection length.

作者信息

Jia Yingyu, Li Bingliang, Xi Hongwei, Ren Hongxia

机构信息

Neonatal Surgery, Shanxi Children's Hospital, Taiyuan, China.

Department of Pediatrics, Shanxi Medical University, Taiyuan, China.

出版信息

Front Pediatr. 2025 Jun 16;13:1553317. doi: 10.3389/fped.2025.1553317. eCollection 2025.


DOI:10.3389/fped.2025.1553317
PMID:40590015
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12206712/
Abstract

BACKGROUND: The appropriate length of resection for the dilated segment in Hirschsprung's disease (HSCR) remains a subject of debate, and the correlation between postoperative clinical outcomes has yet to be elucidated. This study aimed to explore the relationship between the dilated segment resection length (DSRL) and the short-term clinical outcome of HSCR, as well as to determine the optimal DSRL value. METHODS: The clinical data of all children with HSCR who underwent a pull-through surgery at Shanxi Children's Hospital from May 2016 to September 2023 were analyzed retrospectively, the baseline characteristics such as sex, gestational age, family history, and complications such as soiling, perianal erosion, constipation were collected. The groups were stratified in recto-sigmoid aganglionosis (short-segment) and extended colonic (long-segment), and DSRL was divided into three groups: DSRL < 10 cm, 10 ≤ DSRL < 20 cm, and DSRL ≥ 20 cm. The Wingspread score system was used to evaluate anal function and analyze the short-term clinical outcome. RESULTS: A total of 223 children were included in the study, among which 104 cases had short-segment HSCR and 119 cases had long-segment HSCR. The median age at which pull-through surgery was performed was 4 months. In cases of short-segment HSCR, aside from preoperative anemia, baseline characteristics showed no statistically significant differences among the three groups. No statistically significant association was observed between DSRL, the total length of intestinal resection, the length of aganglionosis,and postoperative clinical outcomes.For short-segment HSCR, the best postoperative bowel function was observed when DSRL < 10 cm, with the optimal value being 7.25 cm. In cases of long-segment HSCR, no statistically significant differences in baseline characteristics were observed among the three groups. DSRL, the total length of intestinal resection and the length of aganglionosis all showed statistically significant differences in relation to soiling and perianal erosion. For long-segment HSCR, the best postoperative bowel function was observed when 10 ≤ DSRL < 20 cm, with the optimal value being 13.00 cm. CONCLUSIONS: Not only the dilated segment resection length matters for the outcome but also the length of aganglionosis. For short-segment HSCR, DSRL, the total length of intestinal resection and the length of aganglionosis showed no significant impact on short-term clinical outcomes. In contrast, these parameters in long-segment HSCR were significantly associated with soiling and perianal erosion, although overall patient quality of life remained satisfactory. Data from a single clinical center suggest that optimal clinical outcomes for children are achieved when the DSRL measurements are 7.25 cm for short-segment HSCR and 13.00 cm for long-segment HSCR.

摘要

背景:先天性巨结肠症(HSCR)扩张段的合适切除长度仍是一个有争议的话题,术后临床结果之间的相关性尚未阐明。本研究旨在探讨扩张段切除长度(DSRL)与HSCR短期临床结果之间的关系,并确定最佳DSRL值。 方法:回顾性分析2016年5月至2023年9月在山西省儿童医院接受拖出式手术的所有HSCR患儿的临床资料,收集性别、胎龄、家族史等基线特征以及污粪、肛周糜烂、便秘等并发症情况。将病例分为直肠乙状结肠无神经节细胞症(短段型)和结肠扩张型(长段型),并将DSRL分为三组:DSRL<10 cm、10≤DSRL<20 cm和DSRL≥20 cm。采用翼展评分系统评估肛门功能并分析短期临床结果。 结果:本研究共纳入223例患儿,其中短段型HSCR 104例,长段型HSCR 119例。拖出式手术的中位年龄为4个月。在短段型HSCR病例中,除术前贫血外,三组间基线特征无统计学显著差异。未观察到DSRL、肠切除总长度、无神经节细胞症长度与术后临床结果之间存在统计学显著关联。对于短段型HSCR,当DSRL<10 cm时术后肠道功能最佳,最佳值为7.25 cm。在长段型HSCR病例中,三组间基线特征无统计学显著差异。DSRL、肠切除总长度和无神经节细胞症长度在污粪和肛周糜烂方面均显示出统计学显著差异。对于长段型HSCR,当10≤DSRL<20 cm时术后肠道功能最佳,最佳值为13.00 cm。 结论:不仅扩张段切除长度对结果有影响,无神经节细胞症长度也有影响。对于短段型HSCR,DSRL、肠切除总长度和无神经节细胞症长度对短期临床结果无显著影响。相比之下,长段型HSCR中的这些参数与污粪和肛周糜烂显著相关,尽管总体患者生活质量仍令人满意。来自单一临床中心的数据表明,短段型HSCR的DSRL测量值为7.25 cm、长段型HSCR的DSRL测量值为13.00 cm时,患儿可获得最佳临床结果。

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本文引用的文献

[1]
Does Length of Extended Resection Beyond Transition Zone Change Clinical Outcome for Hirschsprung Pull-Through?

J Pediatr Surg. 2024-1

[2]
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Nat Rev Dis Primers. 2023-10-12

[3]
Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease.

Eur J Pediatr. 2023-3

[4]
Quality of life outcomes in children after surgery for Hirschsprung disease and anorectal malformations: a systematic review and meta-analysis.

World J Pediatr Surg. 2022-11-10

[5]
Surgical approach to Hirschsprung disease.

Semin Pediatr Surg. 2022-4

[6]
Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease.

J Pediatr Gastroenterol Nutr. 2022-3-1

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Orphanet J Rare Dis. 2020-6-25

[8]
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Tech Coloproctol. 2019-5

[9]
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J Pediatr Surg. 2019-4-26

[10]
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Gastroenterology. 2016-2-18

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