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异常胰管解剖结构、十二指肠乳头异位远端位置与先天性胆管扩张:一种新的发育三联征?

Anomalous pancreatic duct anatomy, ectopic distal location of the papilla of Vater and congenital biliary dilatation: a new developmental triad?

作者信息

Li Long, Yamataka Atsuyuki, Wang Yian-Xia, Wang Da-Yong, Wang Kun, Li Zhi-Xin, Shimizu Toshiaki, Yamashiro Yuichiro, Zhang Jin-Zhe, Lane Geoffrey J, Miyano Takeshi

机构信息

Department of Pediatric Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, 113-8421 Tokyo, Japan.

出版信息

Pediatr Surg Int. 2003 May;19(3):180-5. doi: 10.1007/s00383-002-0914-0. Epub 2003 Apr 15.

DOI:10.1007/s00383-002-0914-0
PMID:12698266
Abstract

BACKGROUND

The opening of the papilla of Vater represents the orifice of the embryonic hepatic diverticulum from which the ventral pancreas, common bile duct, and liver are derived. Recently, we found a strong association between congenital biliary dilatation (CBD), certain types of pancreatic ductal anatomy (PDA), and ectopic distal location of the papilla of Vater which prompted us to study the relationship between the location of the papilla of Vater and abnormal PDA.

METHODS

A total of 118 patients with CBD were studied. Cholangiograms documented the presence of pancreaticobiliary malunion (PBMU), the location of the papilla of Vater, and the PDA. Eleven age-matched patients with intermittent jaundice were used as controls.

RESULTS

In the control group, the papilla of Vater was located normally in the descending portion of the duodenum in all cases. In the 118 CBD patients, the papilla of Vater was located normally in 38 (32.2%), but in 80 (67.8%), the papilla was located distal to the descending portion of the duodenum. When the papilla was located distally, the incidences of the specific types of PDA studied were significantly higher than when the papilla was located normally (p<0.01). Pancreatic duct dilatation was also more frequent if the papilla was located distally (28.7%) compared with CBD patients with a normal papilla (7.9%) or normal controls (0%) (both p<0.01). PBMU was present in all CBD patients and absent in all controls.

CONCLUSION

Our study strongly suggests that abnormalities occurring during early embryological development of the hepatic diverticulum are responsible for the association between abnormal PDA and ectopic distal location of the papilla of Vater in CBD.

摘要

背景

Vater壶腹的开口代表胚胎肝憩室的开口,腹侧胰腺、胆总管和肝脏均起源于此。最近,我们发现先天性胆管扩张(CBD)、某些类型的胰管解剖结构(PDA)与Vater壶腹异位远端位置之间存在密切关联,这促使我们研究Vater壶腹位置与异常PDA之间的关系。

方法

共研究了118例CBD患者。胆管造影记录了胰胆合流异常(PBMU)、Vater壶腹的位置和PDA。选取11例年龄匹配的间歇性黄疸患者作为对照。

结果

在对照组中,所有病例的Vater壶腹均正常位于十二指肠降部。在118例CBD患者中,38例(32.2%)的Vater壶腹位置正常,但80例(67.8%)的Vater壶腹位于十二指肠降部远端。当壶腹位于远端时,所研究的特定类型PDA的发生率显著高于壶腹位置正常时(p<0.01)。与壶腹位置正常的CBD患者(7.9%)或正常对照组(0%)相比,壶腹位于远端时胰管扩张也更常见(28.7%)(p均<0.01)。所有CBD患者均存在PBMU,而所有对照组均不存在。

结论

我们的研究强烈表明,肝憩室早期胚胎发育过程中出现的异常是导致CBD患者中异常PDA与Vater壶腹异位远端位置之间关联的原因。

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