Tan C E, Driver M, Howard E R, Moscoso G J
Department of Surgery, King's College Hospital, London, England.
J Pediatr Surg. 1994 Jun;29(6):808-14. doi: 10.1016/0022-3468(94)90377-8.
Biliary atresia is an obliterative disorder of the bile ducts, causing obstructive jaundice in neonates. In this study, the developing biliary system of normal human embryos and fetuses was examined and compared with the resected extrahepatic biliary remnants from 205 cases of biliary atresia. At the porta hepatis level, it was found that the primary biliary ductal plate undergoes a specific sequence of remodelling, resulting in the formation of large tubular bile ducts surrounded by thick mesenchyme, between 11 and 13 weeks postfertilisation. These developing ducts are in luminal continuity with the extrahepatic biliary tree throughout gestation. Contrary to long-held belief, no "solid phase" was observed in the development of the extrahepatic bile duct. Examination of the biliary remnants in biliary atresia showed that the porta hepatis is encased in fibrous tissue, and a variable pattern of obliteration of the common hepatic and common bile ducts was observed. Anticytokeratin immunostaining showed similarities between the abnormal ductules within the porta hepatis in biliary atresia, and the developing bile ducts in the first trimester. Biliary atresia may be caused by failure of the remodelling process at the hepatic hilum, with persistence of fetal bile ducts poorly supported by mesenchyme. As bile flow increases perinatally, bile leakage from these abnormal ducts may trigger an intense inflammatory reaction, with subsequent obliteration of the biliary tree.
胆道闭锁是一种胆管闭锁性疾病，可导致新生儿出现梗阻性黄疸。在本研究中，对正常人类胚胎和胎儿发育中的胆道系统进行了检查，并与205例胆道闭锁病例切除的肝外胆道残余组织进行了比较。在肝门水平发现，初级胆管板经历了特定的重塑过程，在受精后11至13周形成了由厚间质包围的大管状胆管。在整个妊娠期，这些发育中的胆管与肝外胆道树在管腔上是连续的。与长期以来的观点相反，在肝外胆管发育过程中未观察到“实性阶段”。对胆道闭锁中胆道残余组织的检查表明，肝门被纤维组织包裹，观察到肝总管和胆总管的闭锁模式各异。抗细胞角蛋白免疫染色显示，胆道闭锁时肝门内异常小胆管与孕早期发育中的胆管之间存在相似性。胆道闭锁可能是由于肝门处重塑过程失败，胎儿胆管持续存在且间质支持不足所致。随着围产期胆汁流量增加，这些异常胆管的胆汁渗漏可能引发强烈的炎症反应，随后导致胆道树闭锁。
