Clinical analysis of congenital bile duct dilatation: Experience with 160 patients over a 7-year period

From July 1989, to June 1996, 160 patients with congenital bile duct dilatation (CBD) were treated at our institution. The incidence of CBD at our institution has increased annually. In approximately 90% of patients with CBD there was associated anomalous arrangement of the pancreaticobiliary ductal system, and we concluded that this was one of the main causes of CBD. Pediatric endoscopic retrograde cholangiopancreatography was first introduced to China in 1989, and has been successfully performed in 90.2% of 92 patients at our institution. We found that age at diagnosis was closely related to the subtype, complications, and mortality of CBD. The subtype also correlated with the presenting symptoms. One hundred and forty-seven of the 160 patients underwent surgery. Of these 147 patients, 136 (92.5%) were cured with normal liver function and 9 (6.1%) improved with liver impairment. Two patients died, one of postoperative pneumonia and one of liver failure. We conclude that early radical surgery and careful postoperative follow-up are essential in the prevention of CBD complications such as cholangitis, pancreatitis, biliary stones, and development of carcinoma. This article is based on a special lecture delivered before the 19th meeting of the Japanese Society of Pancreatico-biliary Maljunction, on September 14, 1996, in Tokyo     

胰胆管连接异常患儿血清淀粉酶同功酶变化

为观察胰胆管连接异常(PBM)患儿血清淀粉酶同功酶谱变化并探讨其临床意义，采集30例先天性胆管。扩张症(CBD)患儿(囊形24例，梭形6例，均伴PBM)手术前、后血清及胆汁；另取30例正常儿童和12例急性胰腺炎成人血清作为对照，应用不连续缓冲系统薄膜电泳法进行酶谱分析。结果显示，正常对照组血清胰型淀粉酶同功酶仅存在P1和P2波峰；PBM患儿术前血清和胆汁的胰型淀粉酶同功酶除有Pl和P2外，均出现P3、P4、P5等异常波峰，血清和胆汁各波峰的出现率具相似性；急性胰腺炎组除有P1、P2和P3外，P4较少见，无P5波峰；PBM患儿血清酶学的异常在胰胆分流术后基本消失。提示：PBM患儿血清淀粉酶同功酶谱存在异常，具一定特异性。该异常证实PBM畸形存在胆-血返流，对PBM，尤其是胆管扩张不明显者有一定的诊断价值。     

Clinicopathology of pancreaticobiliary maljunction: relationship between alterations in background biliary epithelium and neoplastic development

Background/Purpose

Between 1988 and 2003, 38 patients underwent biliary resection for pancreaticobiliary maljunction (PBM). We reviewed the histopathologic findings for the surgically resected specimens to compare the clinical and pathologic features and assess the relationship between changes in the background biliary epithelium and the development of neoplasms.

Methods

Papillary hyperplasia (PHP) seen in the biliary epithelium of patients with PBM, was classified into grades 0–III in the gallbladder and grades 0–II in the extrahepatic bile duct, according to the extent, and was assessed for links with tumors in the same specimens.

Results

The incidence of gallbladder carcinoma was 13/21 in grades I–II, versus 0/16 in grade III, while the incidence of bile duct carcinoma was 4/20 in grade I versus 0/5 in grade II. Furthermore, these incidences for patients below age 50 years and age 50 or older were 1/18 versus 12/20, and 0/14 versus 6/17, respectively.

Conclusions

PHP of the biliary epithelium in PBM patients is an important precursor lesion, especially for gallbladder cancer, and the risk becomes greater with age, regardless of the type of pancreatobiliary junction (PBJ) and its location in the biliary tract.     

End-to-side choledochoduodenostomy: A widely applicable procedure for biliary reconstruction

End-to-side choledochoduodenostomy was originally used for reconstruction between the duodenum and the biliary tree in iatrogenic bile duct stricture. However, we believe the procedure could be applied for various biliary disorders. We have recently shown the high carcinogenicity of biliary epithelium in patients with pancreaticobiliary maljunction, and consequently we recommend excision of the bile duct, along with appropriate reconstruction of the biliary system to divert the flow of pancreatic juice from bile fluid, to prevent carcinoma in biliary epithelium even in patients without dilatation of the bile duct. The conditions causing primary or recurrent bile duct stones must be removed. We employed this procedure for biliary reconstruction in 42 patients with pancreatico-biliary maljunction and in 30 patients with various benign biliary diseases, such as bile duct stones and benign biliary stenosis. We also used the procedure for palliation in 6 patients with malignant tumors around the head of the pancreas. Among these 78 patients over 20 years, we experienced 5 cases of reflux cholangitis with anastomotic stenosis, for which conservative dilatation was required. This procedure of end-to-side choledochoduodenostomy could be widely applicable for biliary reconstruction in terms of its being simplicity, minimal invasiveness and the establishment of a single physiological route for bile flow into the duodenum.     

Origin of the long common channel based on pancreatographic findings in pancreaticobiliary maljunction

BACKGROUND AND AIMS: The origin of a long common channel in pancreaticobiliary maljunction was suggested to be the ventral pancreatic duct. Pathogenesis of long common channels was investigated by anatomically analysing the arrangement of pancreatic ducts in pancreaticobiliary maljunction. MATERIALS AND METHODS: Cholangiopancreatography was performed for 66 cases of pancreaticobiliary maljunction and 200 controls. The accessory pancreatic duct was classified according to course and shape. In cases with long- or short-type accessory pancreatic duct, lengths of the main pancreatic duct from orifice to first inferior branch and junction with the accessory pancreatic duct, and the common channel were measured. RESULTS: Lengths of the main pancreatic duct from orifice to first inferior branch or junction with the accessory pancreatic duct were significantly longer in cases of pancreaticobiliary maljunction cases with the long- or short-type accessory pancreatic duct than in controls (p<0.01). Lengths of the main pancreatic duct from first inferior branch to junction with the accessory pancreatic duct were roughly equivalent in pancreaticobiliary maljunction and controls. CONCLUSIONS: Long common channels in pancreaticobiliary maljunction might be formed embryologically with adhesion of the right ventral pancreatic duct and the terminal portion of the bile duct.     

Diagnostic criteria for pancreaticobiliary maljunction 2013

Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. The diagnostic criteria for pancreaticobiliary maljunction were proposed in 1987. The committee of The Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) for diagnostic criteria for pancreaticobiliary maljunction began to revise the diagnostic criteria from 2011 taking recently advanced diagnostic imaging techniques into consideration, and the final revised version was approved in the 36^th Annual Meeting of JSPBM. For diagnosis of pancreaticobiliary maljunction, an abnormally long common channel and/or an abnormal union between the pancreatic and bile ducts must be evident on direct cholangiography, such as endoscopic retrograde cholangiopancreatography, percutaneous transpehatic cholangiography, or intraoperative cholangiography; magnetic resonance cholangiopancreatography; or three‐dimensional drip infusion cholangiography computed tomography. However, in cases with a relatively short common channel, it is necessary to confirm that the effect of the papillary sphincter does not extend to the junction by direct cholangiography. Pancreaticobiliary maljunction can be diagnosed also by endoscopic ultrasonography or multi‐planar reconstruction images provided by multi‐detector row computed tomography. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction.     

CA199联合ERCP对胰胆管恶性肿瘤的诊断价值研究

目的研究CA19—9对胆道和胰腺恶性肿瘤的诊断价值,以及胆总管梗阻对CA19—9值的影响。方法首先回顾性分析168例胆道和胰腺疾病的患者血清CA199对胆道和胰腺恶性肿瘤的诊断价值。然后前瞻性地对54例因胆总管梗阻而行ERCP＋胆总管支架植入术的患者进行研究。结果胆道和胰腺恶性肿瘤组CA199水平明显高于良性疾病组,差异具有统计学意义（P〈0.01）。血清CA199对胰腺和胆道恶性肿瘤诊断的敏感性分别为100%和84%,特异性分别为87.27%和53.16%。在ERCP术前CA199对胆总管恶性梗阻的诊断的特异性只有43.48%,而在ERCP术后上升到86.96%。结论 CA199对胰腺和胆道恶性肿瘤具有较高的诊断价值,尤其是解除胆道梗阻以后其诊断恶性肿瘤的特异性显著升高。     

胆胰内支架抗阻塞研究进展

胆胰内支架对解除胆胰良恶性狭窄尤其是肝门部肿瘤所致的恶性阻塞性黄疸效果显著,但阻塞依然是支架的主要并发症。肿瘤过度生长、胆汁成分、胆汁流动速度、细菌大量繁殖和支架的原料及形式参与了支架的阻塞。