回肠直肠Duhamel完全结肠无神经节病切除术后的长期功能结果和多学科管理——在三级外科中心的20年经验。

IF 1.5 3区 医学 Q2 PEDIATRICS European Journal of Pediatric Surgery Pub Date : 2024-10-01 Epub Date: 2023-09-25 DOI:10.1055/a-2181-2065
Kailas Bhandarkar, Paolo De Coppi, Kate Cross, Simon Blackburn, Joe Curry
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引用次数: 0

摘要

引言全结肠无胶质细胞病(TCA)患者的管理对儿科外科医生来说是一项挑战。本研究的目的是回顾我们机构20年来对这些患者在Ileoretal Duhamel pull-through手术后的长期结果和多学科团队管理的经验。材料与方法回顾性分析我院所有诊断为TCA的患者。收集了有关人口统计学、临床表现、并发症、需要额外手术以及对肠功能的长期影响的数据。结果202例先天性巨结肠(HSCR)患者中,13例诊断为TCA(6.4%)。临床表现多变。11例在新生儿期出现远端肠梗阻,2例在婴儿早期出现便秘。所有患者均进行了回肠直肠Duhamel牵引。中位随访时间为13年。11人受过如厕训练,其中5人是全大陆的。6人仍然有排便失禁或便秘的问题。1例复发性先天性巨结肠相关小肠结肠炎(HAEC)。2例患者吻合口重建。肠功能困难的患者由一个由外科医生、胃肠病学家、儿科心理学家和临床护士专家组成的多学科团队共同管理。结论TCA可能与显著的长期发病率有关。该系列中近一半的患者存在持续的排便问题,有些患者需要永久性造口。勤奋的随访加上多学科团队的投入,极大地帮助了我们机构管理这些复杂的患者。
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Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis-20-Year Experience in a Tertiary Surgical Center.

Introduction:  Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure.

Materials and methods:  Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function.

Results:  Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists.

Conclusion:  TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

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来源期刊
CiteScore
3.90
自引率
5.60%
发文量
66
审稿时长
6-12 weeks
期刊介绍: This broad-based international journal updates you on vital developments in pediatric surgery through original articles, abstracts of the literature, and meeting announcements. You will find state-of-the-art information on: abdominal and thoracic surgery neurosurgery urology gynecology oncology orthopaedics traumatology anesthesiology child pathology embryology morphology Written by surgeons, physicians, anesthesiologists, radiologists, and others involved in the surgical care of neonates, infants, and children, the EJPS is an indispensable resource for all specialists.
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