Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.     

Clinical problems in the surgical treatment of pancreaticobiliary maljunction

The results of surgical treatment of pancreaticobiliary maljunction at our department are described. The 67 patients who underwent surgery for this disease were divided by age into an adult group (45 patients, aged 16 years and over) and a pediatric group (22 patients, aged less than 16 years). The incidence of concomitant carcinoma before surgery and the incidence and severity of postoperative cholangitis were compared between these two groups. In addition, the cell proliferating activity of the biliary tract epithelium in cancer-free patients was compared between the two groups, using the proliferating cell nuclear antigen labeling index (PCNA LI). Ten patients (all adults) were diagnosed with cancer (gallbladder carcinoma in 7 and bile duct carcinoma in 3) before surgery. The surgical techniques used for reconstruction in the cancer-free patients were: in the adult group, hepaticoduodenostomy in 9 patients, Roux-en-Y hepaticojejunostomy in 17, jejunal interposition in 8, and another technique in 1. In the pediatric group, hepatico-duodenostomy was performed in 17 patients. Roux-en-Y hepaticojejunostomy in 3, and jejunal interposition in 2. Postoperative cholangitis occurred in 6 adults (including 2 with severe form) and 1 child (mild case). The PCNA LI of the biliary tract epithelium was high compared to control findings in the biliary tract epithelium of 10 adult patients without pancreaticobiliary maljunction. In the adult group with dilated extrahepatic bile ducts (n=10 examined) this index was 11.4% for the bile duct epithelium (control, 1.5%) and 12.7% for the gallbladder epithelium (control, 1.4%). In the adult group with non-dilated extrahepatic bile ducts (n=5 examined) it was 5.9% for the bile duct epithelium and 13.1% for the gallbladder epithelium. In the pediatric group (n=10 with extrahepatic bile duct dilatation) it was 7.5% for the bile duct and 9.7% for the gallbladder epithelium. (Differences from control values were all significant.) These results suggest that surgery for this disease should be performed as early as possible and that extrahepatic bile duct excision and biliary reconstruction should be performed whether or not extrahepatic bile ducts are dilated.     

Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst

Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years. Computed tomographic scan and percutaneous transhepatic cholangioscope revealed multiple stones and stenosis of the hepatic duct and the left intrahepatic bile duct. Histologic examination of a biopsy specimen obtained from the stenotic site showed adenocarcinoma of the intrahepatic bile duct. Left lobectomy with re-reconstruction by right hepaticojejunostomy was performed, and his postoperative course was uneventful. One year after the operation, however, he died of carcinomatous peritonitis with recurrence of cholangiocarcinoma. This report warns us that bile stasis owing to stenosis of the intrahepatic bile duct and repeated cholangitis with multiple stones are high-risk factors for carcinogenesis of the intrahepatic bile duct even after total resection of the infantile choledochal cyst.     

Double common bile duct： A case report

Double common bile duct （DCBD） is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct （ACBD） opens in different parts of upper gastrointestinal tract （stomach, duodenum, ductus pancreaticus or septum）. This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction （APBJ） and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APB3 with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography （ERCP） followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APB3 and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.     

胰胆管合流异常胰腺损伤的抗氧化治疗实验研究

目的：研究胰胆管合流异常（APBDU）胰腺损伤的抗氧化治疗。方法：采用13只健康杂种猫建立APBDU动物模型,随机分为A组（7只,不作抗氧化治疗）、B组（6只,抗氧化治疗）;并将13只的术前情况作为对照。饲养6个月,观察其胰腺的病理,胰腺过氧化产物丙二醛（MDA）变化。结果：13只猫均成活6个月;术前胰腺均呈粉红色。6个月后,A组胰腺颜色加重,充血、水肿,其中3只可见扩张的胰管;B组胰腺颜色稍加重,但无胰管扩张及腺体水肿。光镜下,A组有3只猫的胰腺可见炎性细胞浸润,4只见间质血管增生、白细胞附壁;而B组中未见炎性细胞浸润,仅2只间质血管内白细胞增多。电镜下,A组粗面内质网扩张;线粒体数量增多,体积增大,崩解;高尔基复合体发达,数量增多。而B组的胰酶分泌减少,较A组好转。对照组的MDA值为（1.234±0.716）μmol/gprot（微摩尔/克蛋白）,而A组为（2.900±1.888）μmol/gprot,B组为（0.927±0.880）μmol/gprot。ANOVA检验,P=0.0215,F=4.80,有统计学意义。结论：抗氧化治疗对APBDU有效。     

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct

Background/purpose

For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD.

Methods

The authors encountered 60 children with AAPBD in our institution between 1979 and 2002. Six of the 60 were classified as the nondilated type (CBD diameter; less than 8 mm), whereas the other 54 were classified as the dilated type (CBD diameter; more than 9 mm). Amylase levels in serum, CBD, and gallbladder were examined. Cellular activity of the resected gallbladder was examined for the incidence of hyperplasia and Ki-67 labeling index (Ki-67 LI).

Results

The amylase level in the nondilated type was elevated as in the dilated type. Epithelial hyperplasia of the gallbladder was present in 4 of the 6 with the nondilated type (67%). 10 of the 20 with the dilated type (50%), and none of the 6 controls (0%). The Ki-67 LI of the dilated type was significantly higher than that of control.

Conclusions

A free reflux of pancreatic juice into the biliary system was found regardless of dilatation, and cellular proliferative activity of the gallbladder mucosa was increased in both the nondilated and dilated type. Therefore, excision of the extrahepatic bile duct including cholecystectomy is recommended for AAPBD with nondilatation of the CBD.     

Choledochal cyst in adults: aetiological considerations to intrahepatic involvement

BACKGROUND: Choledochal cysts are rare anomalies of the biliary tree and their presentation in adults is infrequent. The high incidence of associated anomalous pancreaticobiliary duct junction (APBDJ) has been well documented. However, the prevalence of APBDJ in different anatomic distribution of cysts has yet to be clarified. METHODS: A total 39 adult patients with choledochal cysts were consecutively enrolled: 22 patients had Todani type I cysts, 16 patients had type IVa cysts, and one patient had a type IVb cyst. RESULTS: No statistical differences were found in age, sex or manifestations. A higher frequency of associated APBDJ was identified in patients with type IVa cyst (100%) than those with type I cyst (P < 0.05). With a mean follow-up of 7.2 years after cyst excision, three patients with type I choledochal cyst and four with type IVa cyst have had episodes of recurrent cholangitis, all accompanied with preoperatively diagnosed APBDJ. CONCLUSION: Anomalous pancreaticobiliary duct junction has a close relationship with adult choledochal cysts, particularly type IVa. We propose the hypothesis that intrahepatic involvement results from progressive enzymatic destruction. Adult choledochal cysts in association with APBDJ, with or without intrahepatic component, should be carefully monitored.