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小儿短肠综合征的当前挑战与新策略:聚焦手术方面及并发症预防

Current Challenges and New Strategies in Pediatric Short Bowel Syndrome: Focus on Surgical Aspects and Prevention of Complications.

作者信息

Sukhotnik Igor, Kammar Haguy

机构信息

Department of Pediatric Surgery, Dana Dwek Children's Hospital Tel Aviv Sourasky Medical Center, 6 Weizmann St., Tel Aviv 6423906, Israel.

出版信息

Children (Basel). 2025 May 12;12(5):621. doi: 10.3390/children12050621.

DOI:10.3390/children12050621
PMID:40426800
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12110667/
Abstract

The medical management and non-transplant surgical options for children with short bowel syndrome (SBS)are maximized as first-line treatments. The purpose of this review is to summarize the currently available evidence and new management strategies in children with SBS. A systematic review of the literature was conducted on data from the last four years, focusing on both the effectiveness and safety of intestinal lengthening procedures, as well as frameworks for the prevention of complications and the achievement of enteral autonomy. Of 546 abstracts that were screened, a total of 27 relevant full-text articles published between 2021 and 2025 were reviewed. The literature that was review showed that, over the past four years, the most commonly used lengthening procedure was serial transverse enteroplasty (STEP), which resulted in a 50-70% increase in bowel length, a decrease in PN dependency in most cases, and weaning off PN in 42-73% of patients. The longitudinal intestinal lengthening technique (LILT) has been used less frequently, allowing a similar 70% increase in small bowel length and 32-52% of patients to wean off PN, but with a higher mortality rate. The main reasons for surgery in patients with SBS patients were the inability to wean off PN, intestinal dysmotility, and bacterial overgrowth. Over the last decade, several new techniques-such as induced intestinal lengthening, distraction enterogenesis, ileal lengthening through internal distraction, and double-barrel enteroplasty-have been described as options for the treatment of a limited bowel length and less invasive modalities. : Autologous gastrointestinal reconstructive surgery, as a part of multidisciplinary management, remains vital for managing children with SBS.

摘要

短肠综合征(SBS)患儿的药物治疗和非移植手术选择作为一线治疗方法已得到最大化应用。本综述的目的是总结SBS患儿目前可用的证据和新的管理策略。对过去四年的数据进行了文献系统综述,重点关注肠延长手术的有效性和安全性,以及预防并发症和实现肠内营养自主的框架。在筛选的546篇摘要中,共审查了2021年至2025年发表的27篇相关全文文章。综述的文献表明,在过去四年中,最常用的延长手术是系列横断肠成形术(STEP),其使肠长度增加了50 - 70%,大多数情况下肠外营养(PN)依赖减少,42 - 73%的患者停用了PN。纵向肠延长技术(LILT)的使用频率较低,小肠长度增加了约70%,32 - 52%的患者停用了PN,但死亡率较高。SBS患者进行手术的主要原因是无法停用PN、肠道运动障碍和细菌过度生长。在过去十年中,一些新技术,如诱导肠延长、牵张肠再生、通过内牵张进行回肠延长和双腔肠成形术,已被描述为治疗肠长度受限和侵入性较小的治疗选择。自体胃肠重建手术作为多学科管理的一部分,对于管理SBS患儿仍然至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/d9ca0cdc0a79/children-12-00621-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/8ad6661de21d/children-12-00621-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/5677e0e14ba0/children-12-00621-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/f75e86e71d69/children-12-00621-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/d9ca0cdc0a79/children-12-00621-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/8ad6661de21d/children-12-00621-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/5677e0e14ba0/children-12-00621-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/f75e86e71d69/children-12-00621-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c51b/12110667/d9ca0cdc0a79/children-12-00621-g004.jpg

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本文引用的文献

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2
Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes.儿童短肠综合征的系列横向肠成形术(STEP):一项关于长期结局的多中心研究
J Pediatr Surg. 2025 Jan;60(1):161909. doi: 10.1016/j.jpedsurg.2024.161909. Epub 2024 Sep 26.
3
Congenital short bowel syndrome: Cases series in the same family and review of literature.
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Int J Surg Case Rep. 2024 Oct;123:110135. doi: 10.1016/j.ijscr.2024.110135. Epub 2024 Aug 22.
4
Outcomes of Children With Short Bowel Syndrome: Experiences in a Multidisciplinary Intestinal Rehabilitation Unit Over Two Decades.短肠综合征患儿的治疗结果:一个多学科肠道康复单元二十年的经验
J Pediatr Surg. 2025 Feb;60(2):161646. doi: 10.1016/j.jpedsurg.2024.07.031. Epub 2024 Jul 24.
5
Achieving Digestive Autonomy and Gastrointestinal Continuity in a Patient with Short Bowel Syndrome Secondary to Concomitant Jejunal Atresia and Small Intestinal Hirschsprung's Disease.在一名因合并空肠闭锁和小肠先天性巨结肠症继发短肠综合征的患者中实现消化自主和胃肠道连续性。
European J Pediatr Surg Rep. 2024 Jul 16;12(1):e45-e49. doi: 10.1055/a-2351-9413. eCollection 2024 Jan.
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