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短段型先天性巨结肠的外科治疗

Surgical management of short-segment Hirschsprung disease.

作者信息

Etskovitz Haley, Kim Rosa S, Wang Sarah Ziqi, Nandivada Prathima

机构信息

Department of General Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.

出版信息

World J Pediatr Surg. 2024 Dec 31;7(4):e000916. doi: 10.1136/wjps-2024-000916. eCollection 2024.

DOI:10.1136/wjps-2024-000916
PMID:39845183
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11752025/
Abstract

Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect. This review synthesizes the current evidence surrounding surgical management of SS-HSCR, discussing technique-specific outcomes and areas for future research, with a focus on optimizing patient care and functional outcomes.

摘要

先天性巨结肠症（HSCR）是最常见的先天性肠道动力障碍性疾病，其特征是肌间神经丛和黏膜下神经丛中缺乏神经节细胞，导致功能性肠梗阻。短段先天性巨结肠症（SS-HSCR）占大多数病例，手术切除是治疗的基石。尽管手术技术有所进步，但在手术时机、技术选择以及切除无神经节直肠袖带的长度等实际操作方面仍存在很大差异。本综述综合了目前关于SS-HSCR手术治疗的证据，讨论了特定技术的结果和未来研究领域，重点是优化患者护理和功能结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/4eb0a1bf433c/wjps-7-4-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/d2db879d3dbb/wjps-7-4-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/79aeec2d1d29/wjps-7-4-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/4eb0a1bf433c/wjps-7-4-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/d2db879d3dbb/wjps-7-4-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/79aeec2d1d29/wjps-7-4-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/781a/11752025/4eb0a1bf433c/wjps-7-4-g003.jpg

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本文引用的文献

1
Robotic-assisted Proctosigmoidectomy Versus Laparoscopic-assisted Soave Pull-through for Hirschsprung Disease: Medium-term Outcomes From a Prospective Multicenter Study.机器人辅助乙状结肠直肠切除术与腹腔镜辅助Soave拖出术治疗先天性巨结肠症：一项前瞻性多中心研究的中期结果
Ann Surg. 2025 Apr 1;281(4):689-697. doi: 10.1097/SLA.0000000000006172. Epub 2023 Dec 11.
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Post-surgical Outcomes of Different Surgical Techniques in Hirschsprung's Disease: A Literature Review.先天性巨结肠症不同手术技术的术后结果：文献综述
Cureus. 2023 Oct 14;15(10):e47012. doi: 10.7759/cureus.47012. eCollection 2023 Oct.
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Transanal endorectal or transabdominal pull-through for Hirschsprung's disease; which is better? A systematic review and meta-analysis.
经肛门直肠内或经腹腔拖出术治疗先天性巨结肠：哪一种更好？系统评价和荟萃分析。
Pediatr Surg Int. 2023 Jan 24;39(1):89. doi: 10.1007/s00383-023-05378-1.
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Surgical approach to Hirschsprung disease.先天性巨结肠症的手术治疗方法。
Semin Pediatr Surg. 2022 Apr;31(2):151156. doi: 10.1016/j.sempedsurg.2022.151156. Epub 2022 Apr 8.
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Semin Pediatr Surg. 2022 Apr;31(2):151157. doi: 10.1016/j.sempedsurg.2022.151157. Epub 2022 Apr 18.
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Surgical history of Hirschsprung disease.先天性巨结肠手术史。
Semin Pediatr Surg. 2022 Apr;31(2):151174. doi: 10.1016/j.sempedsurg.2022.151174. Epub 2022 May 10.
7
Optimal timing of definitive surgery for Hirschsprung's disease to achieve better long-term bowel function.先天性巨结肠症最佳确定性手术时机以获得更好的长期肠道功能。
Surg Today. 2022 Jan;52(1):92-97. doi: 10.1007/s00595-021-02356-9. Epub 2021 Aug 12.
8
Optimal timing for Soave primary pull-through in short-segment Hirschsprung disease: A meta-analysis.短节段先天性巨结肠 Soave 经肛门根治术的最佳时机：一项荟萃分析。
J Pediatr Surg. 2022 Apr;57(4):719-725. doi: 10.1016/j.jpedsurg.2021.07.007. Epub 2021 Jul 21.
9
Perioperative and long-term functional outcomes of neonatal versus delayed primary endorectal pull-through for children with Hirschsprung disease: A pediatric colorectal and pelvic learning consortium study.新生儿与延迟一期经肛门直肠拖出术治疗先天性巨结肠患儿的围手术期和长期功能结局：小儿肛肠和骨盆学习联合会研究。
J Pediatr Surg. 2021 Aug;56(8):1465-1469. doi: 10.1016/j.jpedsurg.2021.04.024. Epub 2021 Apr 30.
10
Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.长段先天性巨结肠的治疗和结局：APSA 结果和循证实践委员会的系统评价。
J Pediatr Surg. 2021 Sep;56(9):1513-1523. doi: 10.1016/j.jpedsurg.2021.03.046. Epub 2021 Mar 28.