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适当的切除手术可减少先天性胆管扩张症患儿的肝内胆管扩张。

Appropriate excision surgery reduces intrahepatic bile duct dilatation in pediatric patients with congenital biliary dilatation.

作者信息

Shirai Takeshi, Hamada Yoshinori, Hamada Hiroshi, Doi Takashi

机构信息

Department of Pediatric Surgery, Kansai Medical University, 2-3-1 Shinmachi, Hirakata, Osaka, Japan.

出版信息

Pediatr Surg Int. 2025 Apr 9;41(1):109. doi: 10.1007/s00383-025-06016-8.

DOI:10.1007/s00383-025-06016-8
PMID:40205222
Abstract

PURPOSE

This study aims to assess the long-term postoperative changes in the preoperative dilated intrahepatic bile duct (IHBD) in pediatric patients with congenital biliary dilatation (CBD).

METHODS

The postoperative serial changes in the preoperative dilated left IHBD in pediatric patients who underwent appropriate excision surgery were evaluated using magnetic resonance cholangiopancreatography (MRCP).

RESULTS

The IHBD dilation significantly decreased at a median of 1.5 years and was maintained up to 15 years postop in the 24 CBD patients, regardless of the methods of biliary reconstruction. In the 16 patients with apparent preoperative IHBD dilation (5 mm or more), a significant rapid decrease was also observed and maintained up to 15 years postop, regardless of age at surgery. However, a patient with failed rapid reduction at 1.5 years postop and sustained IHBD dilatation of more than 5 mm developed cholangitis 15 years postop with suspected residual congenital stenosis at the hepatic hilum.

CONCLUSIONS

Performing the appropriate excision surgery resulted in a rapid reduction of the preoperative IHBD dilation and maintained long-term reduced IHBD dilation. Patients with failed rapid reduction and sustained dilatation of the IHBD would have residual stenosis and should be carefully followed up for possible late cholangitis.

摘要

目的

本研究旨在评估先天性胆管扩张症(CBD)患儿术前扩张的肝内胆管(IHBD)术后的长期变化。

方法

使用磁共振胰胆管造影(MRCP)评估接受适当切除手术的患儿术前扩张的左肝内胆管术后的系列变化。

结果

24例CBD患儿的肝内胆管扩张在术后中位时间1.5年时显著减轻,并在术后长达15年保持稳定,无论胆管重建方法如何。16例术前肝内胆管明显扩张(5mm或以上)的患儿,无论手术时年龄大小,术后也观察到显著快速减轻,并在术后长达15年保持稳定。然而,1例术后1.5年肝内胆管扩张快速减轻失败且持续扩张超过5mm的患儿,术后15年发生胆管炎,怀疑肝门处存在先天性狭窄残留。

结论

进行适当的切除手术可使术前肝内胆管扩张快速减轻,并长期维持肝内胆管扩张减轻状态。肝内胆管扩张快速减轻失败且持续扩张的患儿可能存在残留狭窄,应密切随访以防可能发生的迟发性胆管炎。

相似文献

1
Appropriate excision surgery reduces intrahepatic bile duct dilatation in pediatric patients with congenital biliary dilatation.适当的切除手术可减少先天性胆管扩张症患儿的肝内胆管扩张。
Pediatr Surg Int. 2025 Apr 9;41(1):109. doi: 10.1007/s00383-025-06016-8.
2
[Surgical strategy for the treatment of congenital biliary dilatation based on the location of intrahepatic bile duct dilatation].
Nihon Geka Gakkai Zasshi. 1996 Aug;97(8):618-25.
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Laparoscopically assisted extrahepatic bile duct excision with ductoplasty and a widened hepaticojejunostomy for complicated hepatobiliary dilatation.腹腔镜辅助下肝外胆管切除、胆管成形术及扩大的肝管空肠吻合术治疗复杂性肝胆管扩张症。
Pediatr Surg Int. 2014 Jun;30(6):593-8. doi: 10.1007/s00383-014-3501-2. Epub 2014 Apr 10.
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Congenital intrahepatic bile duct dilatation is a potentially curable disease: long-term results of a multi-institutional study.先天性肝内胆管扩张症是一种潜在可治愈的疾病:一项多机构研究的长期结果
Ann Surg. 2007 Aug;246(2):236-45. doi: 10.1097/SLA.0b013e3180f61abf.
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Surgical treatment for congenital biliary dilatation, with or without intrahepatic bile duct dilatation.先天性胆管扩张症的手术治疗,无论有无肝内胆管扩张。
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Use of preoperative, 3-dimensional magnetic resonance cholangiopancreatography in pediatric choledochal cysts.术前三维磁共振胆胰管成像在小儿胆总管囊肿中的应用。
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Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst.术后先天性胆总管囊肿持续性胆管扩张和狭窄。
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[A surgical treatment for congenital bile duct dilatation with intrahepatic calculi].[先天性胆管扩张症合并肝内结石的外科治疗]
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本文引用的文献

1
Laparoscopic bile duct plasty for hilar bile duct stenosis (HBDS) in patients with congenital biliary dilatation: Diagnosis of HBDS by preoperative MRCP and laparoscopic strategy to relieve HBDS.先天性胆管扩张症患者肝门部胆管狭窄(HBDS)的腹腔镜胆管成形术:术前磁共振胰胆管造影(MRCP)诊断HBDS及缓解HBDS的腹腔镜策略
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Pancreaticobiliary maljunction and congenital biliary dilatation.胰胆管合流异常与先天性胆管扩张症。
Lancet Gastroenterol Hepatol. 2017 Aug;2(8):610-618. doi: 10.1016/S2468-1253(17)30002-X.
3
Duodenogastric regurgitation in hepaticoduodenostomy after excision of congenital biliary dilatation (choledochal cyst).
先天性胆管扩张症(胆总管囊肿)切除术后肝十二指肠吻合术中的十二指肠胃反流。
J Pediatr Surg. 2017 Oct;52(10):1621-1624. doi: 10.1016/j.jpedsurg.2017.03.063. Epub 2017 Apr 5.
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Diagnostic criteria for congenital biliary dilatation 2015.2015年先天性胆管扩张症诊断标准
J Hepatobiliary Pancreat Sci. 2016 Jun;23(6):342-6. doi: 10.1002/jhbp.346. Epub 2016 May 3.
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Intrahepatic duct dilatation in type 4 choledochal malformation: pressure-related, postoperative resolution.4 型胆总管畸形肝内胆管扩张:与压力相关,术后缓解。
J Pediatr Surg. 2011 Feb;46(2):299-303. doi: 10.1016/j.jpedsurg.2010.11.008.
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Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst.术后先天性胆总管囊肿持续性胆管扩张和狭窄。
J Hepatobiliary Pancreat Sci. 2011 Jan;18(1):47-52. doi: 10.1007/s00534-010-0294-0. Epub 2010 Jun 4.
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Congenital stenosis of the hepatic duct at the porta hepatis in children with choledochal cyst.先天性肝门部肝管狭窄合并胆总管囊肿患儿
J Pediatr Surg. 2009 Mar;44(3):512-6. doi: 10.1016/j.jpedsurg.2008.06.011.
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Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts.磁共振胰胆管造影在胆总管囊肿患儿术后复查中的应用
Pediatr Surg Int. 2004 Jan;20(1):43-6. doi: 10.1007/s00383-003-1079-1. Epub 2003 Dec 19.
9
Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture.先天性胆管囊性疾病的分类:特别提及伴有原发性胆管狭窄的Ic型和IVA型囊肿。
J Hepatobiliary Pancreat Surg. 2003;10(5):340-4. doi: 10.1007/s00534-002-0733-7.
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Operative treatment of congenital stenoses of the intrahepatic bile ducts in patients with choledochal cysts.胆总管囊肿患者肝内胆管先天性狭窄的手术治疗
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