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Dong Qian, Shan Ruo-bing* Department of Pediatric Surgery, Affiliated Hospital of Medical College, Qing Dao University, Qing Dao Peoples' Republic of China *: Department of Pediatrics, Qing Dao Children's Hospital, Qing Dao Peoples' Republic of China Abstract
Introduction It is a well-known fact that almost all cases of congenital biliary dilatation (CBD) are associated with an anomalous arrangement of the pancreatico-biliary ducts (APBD) (1)-(3). It was generally accepted that there is a predominance in the Japanese, and in more than one-third of the cases are from Japanese literature (4),(5). However, Chinese people also belong to oriental race; in their oriental background Chinese people are the same as the Japanese. It is still unknown whether Chinese cases of CBD show a similar pattern including APBD association and a high incidence of biliary cancer, as those of the Japanese . In order to elucidate these points, 1482 cases with CBD or diagnosed as APBD were collected and analyzed from 25 medical universities and hospitals mainly in eastern and northern China . Materials and Methods Because of the well-known fact that almost all cases of congenital biliary dilatation (CBD) are associated with an anomalous arrangement of the pancreatico-biliary duct (APBD), this paper concerns patients with CBD and APBD, as well as APBD without CBD. Thus 1482 cases were collected from 25 medical universities and hospitals in China, and were diagnosed and treated during the past 30 years. Information concerning these patients contained several items, such as clinical history, patients' general information, hematological and biochemical data, as well as operative methods, complications and malignancy. ERCP findings or intraoperative cholangiography findings were also included. A total of 1482 cases were collected by means of case cards from cooperative hospitals and universities or from the published literature in Chinese journals. However, not all the items were filled out in every case. Therefore, the statistical findings were calculated from those cards that gave the particular data required. Because western China has been an undeveloped district, information on CBD and APBD was rare, so materials were mainly obtained from eastern, southern and northeastern China. RESULTS 1. Incidence by Age and Sex The number of cases with patients under the age of 14 (pediatric age group) was 1224. The number of cases above the age of 15 (adult cases) was 258. In the same university, for example, in Shanghai Medical University about 6.37 child cases per year were diagnosed and treated, but the adult cases were only 1.75 cases per year. Of the 1482 cases, 74.9% were female. The male to female ratio of 1 to 2.99 was observed. About 4.2 cases were diagnosed and treated in one hospital or university during one year in China. From these results, a high incidence of mortality of CBD and APBD was seen in China. 2. Incidence of APBD Associated with CBD Because of poor equipment and inadequate techniques, most hospitals, especially in undeveloped zones of China, can not do the examination of intraoperative cholangiography and ERCP. Therefore, only 191 cases of CBD in six hospitals from a total of 1482 cases in 25 institutes were investigated. ERCP, intraoperative total cholangiography, and cholangiography through external Drainage tube were used to determine whether the cases with CBD were associated with APBD (Table 1). In 143 out of 191 cases with CBD, satisfactory visualization of the biliary tract and pancreatic duct were seen. APBD was successfully visualized in 143 (74.9%) cases with CBD. None of the cases of the CBD had the absence of APBD as seen in both child and adult patients. Because of the unsuccessful cholangiography, the remaining 48 cases of CBD were undetermined concerning APBD . 3. Information about Biliary Duct Carcinoma (BCD) in the condition of CBD and APBD 475 cases of CBD and APBD were followed up for 5-25 years (a mean period of 12 years) to investigate the situations of BDC after initial operation. Of the 475 cases examined, 45 patients (9.6%) had carcinoma of the biliary tract. 35 out of 45 patients with CBD received several procedures in the initial operation. In 13 cases, choledochoduodenostomy was performed. In 20 cases, choledochojejunostomy Roux-en-Y, and in two cases, external drainage was done. The youngest child was diagnosed as adenocarcinoma arising in the biliary tract at the age of only 8 years old. The boy was diagnosed as CBD at the age of 10 months. For several reasons the boy had no surgical treatment until age seven, even though he had recurrent symptoms. The first operative procedure was choledochojejunostomy; one year later because of jaundice, he received the second operation and malignant changes were discovered. Only one case of biliary carcinoma developed in patients receiving the procedure of the resection of dilated common bile duct and hepaticojejunostomy. This was a young girl who had been misdiagnosed with hepatitis during the past ten years and had received the initial operation at 14 years. The initial operative procedure was the resection of dilated common bile duct and hepaticojejunostomy but two months later due to the obstructive biliary symptom, she underwent the second operation diagnosed as adenocarcinoma in the bile duct. It was thought that in the first operation the adenocarcinoma failed to be discovered and remained in the residual bile duct. Ten cases of CBD patients aged 14-52 were proved to have biliary carcinoma in the first operation. None of the cases had previous surgery, although almost all of them had a lot of pancreaticobiliary symptoms for several years. In these ten cases, the youngest one was a 13 year-old girl who had abdominal pain and intermittent jaundice for eight years. At the first surgical treatment, adenocarcinoma was detected. Even though the cyst wall was resected and hepaticojejunostomy was done, the girl died seven months after the operation. 4. Biliary Carcinoma from APBD Patients without CBD BDC was also found in APBD, which was not associated with CBD. Out of 1482 cases, 17 cases were diagnosed as APBD but without CBD. All of the 17 cases were examined by ERCP because of their pancreaticobiliary symptoms, such as choledocholithiasis (14/17), abdominal pain (16/17) and intelligent jaundice (6/17). 17 cases of APBD were diagnosed at the age of 6 to 79 years. In 17 cases of APBD, three (18%) cases had biliary carcinoma, two arising in the gallbladder and one in the common bile duct. 5. Surgical Treatment Of CBD and APBD Patients In 1978, as learned from Japanese colleagues, the first case of resection of dilated common bile duct and hepatico-jejunostomy was done in Harbin Medical University in China. Because of the very high incidence of biliary carcinoma arising in CBD, it was generally accepted that the surgical procedure of resection of dilated common bile duct and hepatico-jejunostomy must be the first choice for treating CBD. However, in certain districts of China, especially in undeveloped zones, internal enteric drainage procedure, i.e. choledocho-jejunostomy Roux-Y and choledocho-duodenostomy was still being performed through the 1990's. We know it is our duty to stop the old surgical procedure, which is still being done in parts of China. DISCUSSION Congenital biliary dilatation (CBD) associated with carcinoma was first reported in 1944 by Irwin and Morrison (6). In the past 55 years, as more attention has been directed to malignancy of the biliary tract of congenital biliary dilatation, the increased risk of bile duct carcinoma in CBD has been well characterized (7,8,10). The reported incidence of biliary tract carcinoma in CBD varies between 2.5% and 17.5%, which is significantly higher than that found in the general population, which is from 0.01 to 0.05% (9). Because of the scarcity of the literature in English about CBD and APBD from China published in the international medical journals, it was generally thought that there has been a predominance of occurrence of CBD and APBD in Japanese people. The purpose of this paper is to explain whether Chinese people also have a predominance in occurrence of CBD and APBD. From our investigation, about 4.2 cases were diagnosed and treated in one institute during one year in China. A high incidence of mortality of CBD and APBD is seen. In a total of 1482 patients with CBD, only 191 cases were examined to clarify whether they were associated with APBD or not, and in 48 out of 191 cases, the biliary duct and pancreatic duct could not be visualized for various reasons. Nevertheless, all of the other 143 patients successfully examined proved to be associated with APBD. According to our results, the evidence is worth noticing that Chinese patients with CBD also showed an association with APBD. Therefore it would appear that there is also a high frequency in occurrence of CBD and APBD among Chinese people due to their oriental background. From our materials, 478 out of 1482 patients were followed up and investigated for biliary carcinoma. Of these 478, 9.6 % (45 cases ) had malignant changes arising in the biliary tract. Of the 45 patients, 35 patients received several kinds of initial operation. The initial operative procedures of 34 cases involved internal enteric drainage, e.g. choledochoduodenostomy or choledochojejunostomy. Only one patient who underwent complete excision of extrahepatic biliary ducts followed by hepaticojejunostomy had malignant change in biliary tract. It has been suggested that CBD patients who had undergone surgery, especially bypass surgery, were at an increased risk for developing cancer. In our studies, the carcinogenesis rate of 9.6 % in biliary tract was high. This percentage might be shown to be higher by more intensive examination and follow up. The youngest patient with CBD developed biliary carcinoma at the age of eight years old. This may be the youngest case with a malignant change reported in China. Another distinguishing feature was that in all the 45 cases complicated with malignant change, 22% of them had no previous operation. The carcinogenesis rate of CBD patients which had no previous operation was much higher than that reported by some journals (11)-(13). The author thinks that in developed countries, patients with CBD usually receive immediate diagnosis and treatment once the first symptoms occur, but in certain districts of China, many patients with CBD can not get the proper treatment even though they have pancreaticobiliary symptoms for several years. The delayed treatments lead to a much higher carcinogenesis rate in the CBD patients who had no previous operation. Therefore, the problem of biliary carcinogenesis in the basis of CBD is more severe in developing countries. Although nearly half of the total patients underwent the operative procedure of complete excision of extrahepatic biliary ducts followed by hepaticojejunostomy, only one patient had a malignant change. For this patient, during the first operation, the adenocarcinoma may not have been discovered and remained in the residual bile duct. Not only in the patients with CBD but also in the APBD patients without CBD，the high incidence (18 % carcinogenesis rate) of biliary carcinoma is observed. Therefore, the mechanism of high incidence of biliary carcinoma, like the hypothesis pointed out by our previously published paper, the biliary carcinogenesis is due to the refluxed pancreatic juice in the biliary tract as the basis for APBD(14). Finally, we suggest that early diagnosis followed by definitive surgical treatment of complete excision of extrahepatic biliary ducts and hepaticojejunostomy is essential to prevent malignant changes.
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