Department of Paediatric Surgery, King's College Hospital, London, UK.
Br J Surg. 2011 Sep;98(9):1319-26. doi: 10.1002/bjs.7588. Epub 2011 Jul 4.
The aetiology of choledochal malformation is not known. Babbitt's hypothesis remains a popular concept, and assumes that activated pancreatic juice refluxes through the common pancreatobiliary channel causing mural damage and subsequent biliary dilatation. This hypothesis was tested clinically by evaluating the relationship between epithelial histology, choledochal pressure and degree of pancreatic reflux.
Children with choledochal malformation (cystic, type 1c; fusiform, type 1f; both intrahepatic and extrahepatic dilatation, type 4) operated on between January 1999 and October 2009 were identified. Where practical, choledochal pressure was measured on entry to the abdominal cavity, by puncture of the common bile duct, and bile was sampled for amylase content. Archival bile duct sections were scored using a semiquantitative epithelial lining/mural score (ELMS).
A total of 90 children with choledochal malformations were operated on during the study interval. Histology was available for 73 children (median age 2·9 (interquartile range 1·3-7·9) years), 29 with type 1c, 31 with type 1f and 13 with type 4 malformations. There was a significant stepwise increase in pressure with choledochal morphology (median pressure 13, 17 and 20 mmHg for types 1f, 1c and 4 respectively; P = 0·037). There was an inverse relationship between choledochal pressure and bile amylase activity (r(s) = - 0·45, P < 0·001). High ELMS values were associated with higher choledochal pressure (P = 0·057) and low bile amylase activity (P = 0·002).
High choledochal pressure (not bile amylase) was associated with more severe histopathological changes and choledochal morphology. These findings suggest that distal bile duct obstruction (and therefore high intraluminal pressure) contributes more to the key features of choledochal malformation than does pancreatic reflux.
胆总管畸形的病因尚不清楚。Babbitt 的假说仍然是一个流行的概念，它假设激活的胰液通过共同的胰胆管反流，导致壁损伤和随后的胆管扩张。这一假说通过评估上皮组织学、胆总管压力和胰液反流程度之间的关系在临床上进行了测试。
确定了 1999 年 1 月至 2009 年 10 月间接受手术治疗的胆总管畸形患儿（囊性，1c 型；梭形，1f 型；肝内外均扩张，4 型）。在实际可行的情况下，通过穿刺胆总管进入腹腔时测量胆总管压力，并抽取胆汁检测淀粉酶含量。使用半定量上皮衬里/壁评分（ELMS）对胆管切片进行评分。
在研究期间，共有 90 例胆总管畸形患儿接受了手术治疗。共有 73 例患儿（中位年龄 2.9 岁[四分位间距 1.3-7.9]）有组织学资料，其中 29 例为 1c 型，31 例为 1f 型，13 例为 4 型畸形。胆总管形态与压力呈显著的逐步递增关系（1f、1c 和 4 型的中位压力分别为 13、17 和 20mmHg；P = 0.037）。胆总管压力与胆汁淀粉酶活性呈负相关（r(s) = -0.45，P<0.001）。高 ELMS 值与较高的胆总管压力（P = 0.057）和低胆汁淀粉酶活性（P = 0.002）相关。
高胆总管压力（而非胆汁淀粉酶）与更严重的组织病理学变化和胆总管形态相关。这些发现表明，远端胆管梗阻（因此腔内压力较高）对胆总管畸形的关键特征的贡献大于胰液反流。
