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有或无胎儿期诊断的先天性胆管扩张症的当前特征及治疗比较:日本九州小儿外科学术研究组过去十年连续231例病例报告

Comparison of the current characteristics and management of congenital biliary dilatation with and without a fetal diagnosis: report of 231 consecutive cases from the Kyushu Pediatric Surgery Study Group in Japan over the past decade.

作者信息

Murakami Masakazu, Yano Keisuke, Maeda Shohei, Shirai Takeshi, Yamane Yusuke, Kosaka Taichiro, Kawano Takafumi, Hayashida Makoto, Matsuura Toshiharu, Eto Hiroto, Torikai Motofumi, Tatekawa Yukihiro, Yamanouchi Takeshi, Hibi Taizo, Tajiri Tatsuro, Ieiri Satoshi

机构信息

Department of Pediatric Surgery, Medical and Dental Area, Research and Education Assembly, Research Field in Medical and Health Sciences, Kagoshima University, 8-35-1, Sakuragaoka, Kagoshima City, 890-8520, Japan.

Department of Pediatric Surgery, Hokkaido Medical Center for Child Health and Rehabilitation, Sapporo, Japan.

出版信息

Pediatr Surg Int. 2025 Jul 22;41(1):224. doi: 10.1007/s00383-025-06136-1.

Abstract

PURPOSE

The aim of this study is to investigate and to compare the current characteristics and management of congenital biliary dilatation (CBD) with and without fetal diagnosis (FD) based on a multicenter study at all institutions in Kyushu, Japan.

METHODS

A regional multicenter study was conducted among the institutions affiliated with the Kyushu Pediatric Surgery Study Group. Patients with CBD who underwent definitive surgery between 2014 and 2023 were enrolled.

RESULTS

All 28 affiliated institutions joined this multicenter study. There comprised 231 cases (Date collection rate: 100%), including 35 cases (15.2%) with an FD. Compared to cases without an FD, cases with an FD were significantly more frequently recognized in type Ia (p < 0.01), significantly less frequently recognized in type Ic (p = 0.04), and IV-A (p < 0.01), and showed a significantly lower frequency of preoperative cholangitis (p < 0.01) and pancreatitis (p < 0.01). Cases with an FD had significantly shorter operative times (p < 0.01). There were no significant differences in the frequency of postoperative and late postoperative complications between the patients with and without an FD.

CONCLUSION

CBD cases with an FD were more likely to be type Ia and less likely to have preoperative symptoms than those without an FD, with a comparable risk of postoperative complications.

摘要

目的

本研究旨在基于日本九州所有机构的多中心研究,调查和比较有和没有胎儿诊断(FD)的先天性胆管扩张症(CBD)的当前特征及管理情况。

方法

在九州小儿外科研究组所属机构中开展了一项区域多中心研究。纳入2014年至2023年间接受确定性手术的CBD患者。

结果

所有28个附属机构均参与了这项多中心研究。共纳入231例病例(数据收集率:100%),其中35例(15.2%)有胎儿诊断。与无胎儿诊断的病例相比,有胎儿诊断的病例在Ia型中显著更常被识别(p<0.01),在Ic型(p=0.04)和IV - A型中显著更少被识别(p<0.01),且术前胆管炎(p<0.01)和胰腺炎(p<0.01)的发生率显著更低。有胎儿诊断的病例手术时间显著更短(p<0.01)。有和无胎儿诊断的患者术后及术后晚期并发症发生率无显著差异。

结论

与无胎儿诊断的CBD病例相比,有胎儿诊断的CBD病例更可能为Ia型,术前症状更少,术后并发症风险相当。

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