Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, No. 9 Jinsui Road, Guangzhou, Guangdong Province, China.
Department of Obstetrics and Gynecology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
BMC Gastroenterol. 2020 Mar 12;20(1):67. doi: 10.1186/s12876-020-01208-z.
Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However, the long-term outcomes of these patients (older than 10 years) have not been fully investigated. The aim of this systematic review is to clarify the prevalence of long-term outcomes and the quality of life of these patients.
PubMed, AMED, Cochrane Library, CINAHL and PsycINFO databases were searched from inception to October 2018, following the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guideline. Original studies reporting the outcomes of patients older than ten years with HD were selected and reviewed. The overall prevalence of fecal incontinence, constipation, bowel function score, bladder dysfunction symptoms, and patients' quality of life were extracted from the included studies and pooled through the random-effects meta-analysis model. The heterogeneity and variation in the pooled estimations were evaluated by Cochrane's Q test and the I test. The sensitivity analysis was conducted by the sequential omission of individual studies. Publication bias was evaluated by Egger's linear regression test. The whole procedure was conducted with Stata (version 14).
In total, 3406 articles were identified from the literature search, among which twelve studies, including 625 patients, were included for analysis. The pooled prevalences of fecal incontinence, constipation, and bladder dysfunction symptoms and good to excellent bowel function scores were 0.20 (95% CI 0.13-0.28), 0.14 (95% CI 0.06-0.25), 0.07 (95% CI 0.04-0.12), and 0.95 (95% CI: 0.91-0.97), respectively; the pooled mean score of gastrointestinal-related quality of life was 118 (95% CI: 112.56-123.44).
HD patients older than ten years old have an overall high prevalence of fecal incontinence and a low quality of life. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research that focuses on the attributes and predictors of the long-term prognosis of patients with HD are necessary.
手术技术和围手术期护理的进步改善了先天性巨结肠（HD）患者的短期和中期术后结果。然而，这些患者（年龄大于 10 岁）的长期结果尚未得到充分研究。本系统评价的目的是阐明这些患者的长期结果和生活质量的流行情况。
根据观察性研究的荟萃分析（MOOSE）指南，从成立到 2018 年 10 月，在 PubMed、AMED、Cochrane 图书馆、CINAHL 和 PsycINFO 数据库中进行了搜索。选择并回顾了报告年龄大于 10 岁的 HD 患者结果的原始研究。从纳入的研究中提取总体粪便失禁、便秘、肠道功能评分、膀胱功能障碍症状以及患者生活质量的患病率，并通过随机效应荟萃分析模型进行汇总。通过 Cochrane 的 Q 检验和 I 检验评估汇总估计值的异质性和变异性。通过逐个删除个别研究进行敏感性分析。通过 Egger 的线性回归检验评估发表偏倚。整个过程均在 Stata（版本 14）中进行。
共从文献检索中确定了 3406 篇文章，其中 12 项研究，包括 625 名患者，被纳入分析。粪便失禁、便秘和膀胱功能障碍症状以及良好至优秀肠道功能评分的汇总患病率分别为 0.20（95%CI 0.13-0.28）、0.14（95%CI 0.06-0.25）、0.07（95%CI 0.04-0.12）和 0.95（95%CI：0.91-0.97）；胃肠道相关生活质量的汇总平均得分为 118（95%CI：112.56-123.44）。
年龄大于 10 岁的 HD 患者总体上有较高的粪便失禁患病率和较低的生活质量。有针对性的循证随访程序和过渡性护理对于满足这些患者的长期护理需求至关重要。需要进行前瞻性和多中心研究，重点关注 HD 患者长期预后的属性和预测因素。
