Experiment and clinical study on pancreaticobiliary maljunction as to the influence on the bile duct dilatation

1.本研究的目的是阐明胆管扩张的病理生理机制，胆管扩张是胆总管囊肿的特征.本研究分析胆总管囊肿患者的胆管壁，以了解壁内的神经纤维和神经细胞是否异常。本文对8例胆总管囊肿患者的胆管和3例无扩张的正常胆管进行了研究。将这些胆管固定在Zamboni溶液中。通过在体视显微镜下去除粘膜、粘膜下层、纤维肌层，制备显示在胆管外表面下运行的肌间神经丛的整体封片。将整体封片制备物浸入0.3% Triton X-磷酸盐缓冲液中2至3天。用抗蛋白基因产物（PGP）9.5抗体作为一抗孵育6- 7天后，使用链霉抗生物素蛋白-二羟色胺-过氧化物酶法进行免疫化学染色。在靠近外表面的纤维肌层中观察到发达的肌间神经丛网络。神经节分布清楚。患者的神经束与对照组相比无显著性差异，但神经节的数量减少至对照组的十分之一.胆总管囊肿内神经束和神经节的分布与神经节细胞减少症的肠壁相似，提示胆总管囊肿的胆管扩张机制与神经节细胞减少症的巨结肠相同。

1. The aim of this study is to elucidate the pathophysiological mechanism of bile duct dilatation which is characteristic of the choledochal cyst.2. This study analyzed the bile duct walls of patients with choledochal cyst as to whether the nerve fibers and nerve cells in the wall are abnormal or not. Bile ducts obtained from eight patients with choledochal cyst and three normal bile ducts without dilatation were studied. These bile ducts were fixed in Zamboni solution. Whole-mount preparations displaying the myenteric plexus running beneath the outer surface of the bile duct were made by removing the mucosa, submucosa, fibromuscular layers under a stereomicroscope. Whole-mount preparations were immersed in 0.3% Triton X-phosphate buffer solution for 2 to 3 days. They were immunohistochemically stained using the streptavidin-diotin-peroxidase method after 6- to 7-day incubation with anti-protein gene product (PGP) 9.5 antibody as the primary antibody.3. Developed networks of the myenteric plexus were observed in the fibromuscular layer near the outer surface. Distribution of the ganglia was clearly observed. Nerve strands of the patients were not significantly different from those of controls, but the number of ganglia of the patients decreased to one tenth of that of controls.4. This distribution of nerve strands and ganglia in the bite duct wall choledochal cyst is similar to that in the intestinal wall of hypoganglionosis, which implies the mechanism of dilatation of the bile duct in patients with choledochal cyst is the same as that of megacolon in patients with hypoganglionosis.