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肛门直肠畸形与先天性巨结肠病:一项30年的回顾性外展研究

Anorectal Malformations and Hirschsprung Disease: A 30-Year Retrospective Outreach Review.

作者信息

Dewan Patrick A, Mill Patrick J

机构信息

Paediatric Surgery, Sunshine Private Hospital, Melbourne, AUS.

Paediatrics, Oceania University of Medicine, Apia, WSM.

出版信息

Cureus. 2025 Mar 3;17(3):e79987. doi: 10.7759/cureus.79987. eCollection 2025 Mar.

Abstract

Objective This study analyses the indications for and outcomes of primary and redo surgeries for anorectal malformations (ARMs) and Hirschsprung disease (HD) in developing, resource-limited countries. The study seeks to identify trends in primary surgery complication rates, evaluate the indications for reoperation and explore potential strategies to improve surgical management and long-term outcomes for children with ARM and HD in these settings. Methodology A retrospective cohort analysis was conducted on data collected by the Kind Cut for Kids (KCFK) surgical outreach program, a charitable initiative that provides paediatric surgical care in under-served regions. Data was collected over a 30-year period across 22 developing countries. The dataset contained 2,498 observations linked to ARM or HD, which was filtered to include those with primary surgeries, reoperations for prior complications and management of complications of any surgery done by the visiting team. Clinical data included demographics, pathology classification, surgical details, and postoperative outcomes. Results The final cohort included 496 ARM patients and 224 HD patients, with 65% and 41%, respectively, undergoing primary corrective surgeries. Among ARM cases, 25% required redo surgeries, with malposition (33%), strictures (24%), or prolapse (8%) being the most common indications. In HD, 45% of patients required redo procedures, primarily for strictures (19%), prolapse (9%), or acquired fistulas (4%). The most common redo procedures for ARMs were the posterior sagittal anorectoplasty (PSARP) (58%) or anorectal angle plication (10%). For HD patients, PSARP (11%) and the Swenson procedure (10%) were the most common corrective redo procedures. Due to the focus of KCFK visits, there are significant data gaps pertaining to primary surgical details, reoperation indications and follow-up data. This reflects the challenges of managing these conditions in resource-limited environments and with an outreach program. Conclusion This study highlights high rates of complications from primary surgeries in ARM and HD cases treated in resource-limited settings, which emphasises the need for enhanced surgical precision, structured postoperative care, and consistent follow-up protocols, as well as education of surgeons in the countries visited. Targeted interventions such as capacity-building initiatives, tailored consensus guidelines, and telemedicine integration are critical to addressing disparities in care. Future prospective studies with standardised data collection and outcome metrics are essential to validate these findings and improve care delivery for children with ARM and HD in underserved regions.

摘要

目的 本研究分析了发展中、资源有限国家肛门直肠畸形(ARM)和先天性巨结肠(HD)初次手术和再次手术的适应证及手术结果。该研究旨在确定初次手术并发症发生率的趋势,评估再次手术的适应证,并探索改善这些地区ARM和HD患儿手术管理及长期预后的潜在策略。方法 对儿童慈善手术外展项目“儿童慈善切口”(KCFK)收集的数据进行回顾性队列分析,该项目是一项在服务不足地区提供小儿外科护理的慈善倡议。数据收集时间跨度为30年,涉及22个发展中国家。数据集包含与ARM或HD相关的2498条观察记录,经过筛选,纳入了接受初次手术、因先前并发症进行再次手术以及由访问团队进行的任何手术并发症管理的记录。临床数据包括人口统计学、病理分类、手术细节和术后结果。结果 最终队列包括496例ARM患者和224例HD患者,分别有65%和41%接受了初次矫正手术。在ARM病例中,25%需要再次手术,位置异常(33%)、狭窄(24%)或脱垂(8%)是最常见的适应证。在HD中,45%的患者需要再次手术,主要原因是狭窄(19%)、脱垂(9%)或后天性瘘管(4%)。ARM最常见的再次手术是后矢状入路肛门直肠成形术(PSARP)(58%)或肛门直肠角折叠术(10%)。对于HD患者,PSARP(11%)和斯文森手术(10%)是最常见的矫正再次手术。由于KCFK访问的重点,在初次手术细节、再次手术适应证和随访数据方面存在重大数据缺口。这反映了在资源有限的环境中以及通过外展项目管理这些疾病的挑战。结论 本研究强调了在资源有限的环境中治疗的ARM和HD病例初次手术并发症发生率较高,这突出了提高手术精度、结构化术后护理、一致的随访方案以及对访问国家外科医生进行教育的必要性。有针对性的干预措施,如能力建设倡议、量身定制的共识指南和远程医疗整合,对于解决护理差距至关重要。未来采用标准化数据收集和结果指标的前瞻性研究对于验证这些发现并改善服务不足地区ARM和HD患儿的护理至关重要。

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本文引用的文献

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Nat Rev Dis Primers. 2024 Nov 21;10(1):88. doi: 10.1038/s41572-024-00574-2.
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Semin Pediatr Surg. 2022 Dec;31(6):151226. doi: 10.1016/j.sempedsurg.2022.151226. Epub 2022 Nov 16.
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Best Pract Res Clin Gastroenterol. 2022 Feb-Mar;56-57:101765. doi: 10.1016/j.bpg.2021.101765. Epub 2021 Sep 18.
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Pediatr Surg Int. 2020 Apr;36(4):431-445. doi: 10.1007/s00383-020-04629-9. Epub 2020 Feb 21.
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Diagnosis of Hirschsprung Disease.先天性巨结肠症的诊断
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