先天性囊性纤维化患儿胎粪性肠梗阻的早期治疗：一项前瞻性人群队列研究。

Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study.

机构信息

Department of Paediatric Surgery, Cambridge University Hospitals, Cambridge, United Kingdom; National Perinatal Epidemiology Unit, Nuffield Department of Population Health, University of Oxford, Oxford United Kingdom.

Department of Paediatric Surgery, Southampton Children's Hospital, Southampton, United Kingdom; University Surgery Unit, Faculty of Medicine, University of Southampton, Southampton, United Kingdom.

出版信息

J Pediatr Surg. 2021 Aug;56(8):1287-1292. doi: 10.1016/j.jpedsurg.2021.02.047. Epub 2021 Feb 24.

DOI:10.1016/j.jpedsurg.2021.02.047

PMID:33789802

Abstract

BACKGROUND

Contemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management.

METHODS

A prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012-September 2014. Live-born infants with bowel-obstruction from inspissated meconium in the terminal ileum and CF were reported. Data are described as median (interquartile range, IQR).

RESULTS

56 infants were identified. 14/56(25%) had primary laparotomy (13/23 complicated MI, 1/33 simple), the remainder underwent contrast enema. Twelve, (12/33 (36%) with simple MI) achieved decompression. 8/12 (67%) who decompressed had >1 enema vs 3/20 (15%) with simple MI who had laparotomy after enema. The number of enemas per infant (1-4), contrast agents and their concentration, were highly variable. Enterostomy was formed at 24/44(55%) of laparotomies. In infants with simple MI, time to full enteral feeds was 6 (2-10) days in those decompressing with enema vs 15 (9-19) days with laparotomy after enema. Case fatality was 4% (95% CI 0.4-12%). Two infants, both preterm died, both in the second month after birth.

CONCLUSIONS

Infants with simple MI achieving successful enema decompression were more likely to have had repeat enemas than those who proceeded to laparotomy. Successful non-operative management was associated with a shorter time to full feeds. The early management of infants with MI is highly variable and not standardised across the UK and Ireland.

摘要

背景

目前缺乏关于囊性纤维化（CF）中胎粪性肠梗阻（MI）的当代早期结局数据，本研究描述了这些数据并探讨了与成功非手术治疗相关的因素。

方法

采用已建立的监测系统（BAPS-CASS）进行前瞻性人群队列研究，研究时间为 2012 年 10 月至 2014 年 9 月。报道了回肠末端浓稠胎粪引起的肠阻塞和 CF 的活产婴儿。数据以中位数（四分位距，IQR）表示。

结果

共确定了 56 名婴儿。56 名婴儿中，14 名（56 例中的 25%）接受了剖腹手术（23 例复杂 MI 中 13 例，33 例单纯 MI 中 1 例），其余婴儿接受了对比灌肠。12 名（33 例单纯 MI 中的 12 名，36%）获得了减压。减压的 12 名中有 8 名（67%）接受了>1 次灌肠，而接受灌肠后行剖腹手术的 20 名单纯 MI 婴儿中只有 3 名（15%）。每个婴儿灌肠的次数（1-4 次）、造影剂及其浓度均存在较大差异。44 例剖腹手术中有 24 例（55%）形成肠造口。单纯 MI 患儿中，通过灌肠减压的患儿完全经口喂养的时间为 6（2-10）天，而灌肠后行剖腹手术的患儿为 15（9-19）天。病死率为 4%（95%CI 0.4-12%）。2 名婴儿均为早产儿，均在出生后第 2 个月死亡。