Abstract：
BACKGROUND: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients.
METHODS: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up.
RESULTS: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63).
CONCLUSIONS: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.
METHODS: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up.
RESULTS: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63).
CONCLUSIONS: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.
摘要：
背景：过渡区牵拉（TZPT）是Hirschsprung疾病（HD）患者的神经节肠/过渡区（TZ）的不完全去除。缺乏治疗产生最佳长期结果的证据。这项研究的目的是比较先天性巨结肠相关小肠结肠炎（HAEC）的长期发生率，干预的要求，保守治疗的TZPT患者与重做手术治疗的TZPT患者与非TZPT患者的功能结局和生活质量。
方法：我们回顾性研究了2000-2021年TZPT患者。TZPT患者与两名对照患者相匹配，完全切除了神经节/下神经节肠。使用Hirschsprung/肛门直肠畸形生活质量问卷和格罗宁根排便和连续性项目，以及Hirschsprung相关性小肠结肠炎（HAEC）的发生和干预措施的要求，评估了功能结局和生活质量。使用单向方差分析比较各组之间的得分。随访时间从手术时间持续到随访。
结果：15例TZPT患者（6例保守治疗，9例接受重做手术）与30例对照患者相匹配。中位随访时间为76个月（范围12-260）。两组之间在HAEC的发生率上没有发现显着差异（p=0.65），使用泻药（p=0.33），直肠冲洗使用（p=0.11），肉毒杆菌毒素注射（p=0.06），功能结局(p=0.67)和生活质量(p=0.63)。
结论：我们的研究结果表明，HAEC的长期发生率没有差异，干预的要求，TZPT保守治疗或重做手术患者与非TZPT患者的功能结局和生活质量.因此,我们建议在TZPT的情况下考虑保守治疗。
方法：我们回顾性研究了2000-2021年TZPT患者。TZPT患者与两名对照患者相匹配，完全切除了神经节/下神经节肠。使用Hirschsprung/肛门直肠畸形生活质量问卷和格罗宁根排便和连续性项目，以及Hirschsprung相关性小肠结肠炎（HAEC）的发生和干预措施的要求，评估了功能结局和生活质量。使用单向方差分析比较各组之间的得分。随访时间从手术时间持续到随访。
结果：15例TZPT患者（6例保守治疗，9例接受重做手术）与30例对照患者相匹配。中位随访时间为76个月（范围12-260）。两组之间在HAEC的发生率上没有发现显着差异（p=0.65），使用泻药（p=0.33），直肠冲洗使用（p=0.11），肉毒杆菌毒素注射（p=0.06），功能结局(p=0.67)和生活质量(p=0.63)。
结论：我们的研究结果表明，HAEC的长期发生率没有差异，干预的要求，TZPT保守治疗或重做手术患者与非TZPT患者的功能结局和生活质量.因此,我们建议在TZPT的情况下考虑保守治疗。
