先天性胆总管囊肿胰管发育与十二指肠乳头异位的关系

目的十二指肠乳头的开口代表胚胎发育时期肝憩室的发生部位,而胰腺、胆总管和肝脏均起源于肝憩室。前续研究显示先天性胆总管囊肿、胰胆合流异常和十二指肠乳头异位密切相关。本文探讨胰管发育异常和十二指肠乳头异位之间的关系。方法先天性胆总管囊肿患儿118例,胰胆管造影了解其胰胆合流异常、十二指肠乳头异位及胰管发育异常情况,以11例年龄相仿球形红细胞增多症合并胆结石患儿的胆道造影结果为对照组。结果对照组中,11例患儿的十二指肠乳头均位于十二指肠降部。118例先天性胆总管囊肿患儿中,十二指肠乳头开口于降部38例,占32.2%,其余80例患儿,十二指肠乳头开口于降部以远,占67.8%。对于乳头开口于十二指肠降部以远的患儿,合并胰管发育异常者明显高于乳头开口位置正常者(十二指肠降部)(P<0.01)。乳头开口位置正常的先天性胆总管囊肿患儿,合并胰管发育异常仅占23.7%;乳头开口于十二指肠降部以远的患儿合并胰管发育异常仅占49.5%。乳头开口位置正常和开口于十二指肠降部以远的先天性胆总管囊肿患儿,合并胰管扩张的发生率分别为7.9%和28.7%,P<0.05。结论本研究结果表明胰管发育异常是先天性胆总管囊肿的重要病理改变,提示胚胎发育早期肝憩室的发育异常,可能是引起胆道扩张、胰管发育异常和乳头异位的原因。

Pancreatic duct anomalies associated with ectopic papilla of vater in choledochal cyst

Objective 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 among congenital biliary dilatation (CBD), certain type 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 were normal in 38 ((32.2%)), but in 80 ((67.8%)), the papilla were located distal to the descending portion of the duodenum. The incidences of PDA were significantly higher in ectopic papilla group than normal group (P<(0.01)). Pancreatic duct dilatation was also more frequent if papilla was located distally ((28.7%)) compared with CBD patients with a normal papilla ((7.9%)) or normal control (0%) (both P<(0.01)). PBMU was present in all CBD patients and absent in all controls. Conclusions Our study strongly suggests that abnormalities occurring during early embryonic diverticulum are responsible for the association between abnormal PDA and ectopic distal location of the papilla of Vater in CBD.