A Study for Mechanism of Biliary Ductal Injury in Patients with Anomalous Pancreaticobiliary Ductal Union: A Study for Mechanism of Biliary Injury in APBDU / 한국간담췌외과학회지

PURPOSE: It has been known that clinical features of anomalous pancreaticobiliary ductal union (APBDU) are thought to result from cholestasis and regurgitation of pancreatic juice. The purpose of our study is to clarify the relationship of clinicopathological factors and to get information on mechanism of biliary ductal injury in APBDU. METHODS: A total of 23 patients who underwent operation for APBDU from March 1995 to March 2003 were examined. The results of patients were analyzed for their relevance to the clinicopathological factors such as age, sex, age at onset, size of cyst, grade of inflammation, type of APBDU, level of pancreatic enzymes sampled from biliary ductal system, and Ki67 labeling index. RESULTS: Level of amylase in biliary ductal system was decreased with age, especially after 20 years. The grade of hyperplasia in gallbladder was increased with level of amylase in gallbladder. The grade of inflammation in gallbladder was increased with age, but inflammation of gallbladder decreased with the level of amylase in gallbladder, and the size of cyst was increased with grade of inflammation and age. CONCLUSION: Level of amylase in the bile of biliary ductal system was decreased with age, especially after 20 years, which suggests that reflux of pancreatic amylase is closely related with onset of symptom in adult with APBDU. As far as mechanism of biliary ductal injury in APBDU is concerned, inflammation of biliary ductal system is increased with age and size of cyst. And hyperplasia of gallbladder is increased with the degree of regurgitation of pancreatic juice.

The Usefulness of Magnetic Resonance Cholangio-Pancreatography in the Diagnosis of Choledochal Cyst

PURPOSE: Choledochal cysts are cystic or diffuse dilatation of any portion of the biliary tree. An anomalous pancreaticobiliary ductal union (APBDU) is one of the commonly accepted causes of a cholodochal cyst. The ability of MRCP to demonstrate an APBDU has not been established in children and appears to have limited value in demonstrating an associated APBDU. We investigate the usefulness, and application, of MRCP in delineating the cyst type and an APBDU in patients with choledochal cysts, and compared it with operative cholangiography. METHODS: Ten children with choledochal cyst, who had both MRCP and cholangiography, as preoperative diagnostic modalities, at the Division of Pediatric Surgery, Keimyung University, Dongsan Medical Center, between March 1999 and August 2002, were selected for this study. We analyzed and compared their MRCP images with those of the intraoperative cholangiographies, with regard to the type of cyst, size and association of an APBDU. RESULTS: The types of the choledochal cyst, based on the MRCP, were two Ia, three Ic, and five IVa, whereas those based on the operative cholangiographies were one Ic and nine IVa. A common channel was demonstrated by MRCP and operative cholangiography in four (40%) and six (60%) of the ten patients, respectively. Each diagnostic modality correlated with the size (Pearson correlation, P<0.01) and the length of the cyst, but an APBDU on both modalities did not reach statistical significance, even though the common channel was not identified in a large cyst on MRCP. CONCLUSION: MRCP is a noninvasive and safe diagnostic modality for the delineation an APBDU in children with choledochal cysts. However, it has limited value for demonstrating an APBDU in children with a large cyst due to overlapping with the pancreaticobiliary ductal system. Operative cholangiography seems to be more valuable than MRCP in patients with large choledochal cysts.

Choledochal Cyst in Childhood

BACKGROUND: Choledochal cysts are not so rare in far east Asian countries as in western countries and are found mainly in children. Reflux of pancreatic juice to the bile duct through anomalous pancreaticobiliary ductal union (APBDU) is considered to be an important etiologic factor. METHOD: To study the clinical characteristics of choledochal cysts in children and the significance of APBDU in this disease, we reviewed 80 choledochal cyst cases. APBDU is defined as a long common channel (>1.5 cm). RESULTS: Ages ranged from 19 days to 13 years, and the male to female ratio was 1 to 4 which revealed a predominance among females. The symptoms, in order of frequency, were abdominal pain (63%), jaundice (48%), and abdominal mass (26%); classic triads were present in 3 cases (4%). The diagnosis was made mainly with ultrasonography. Todani type I (88%) and IV (11%) were the two most common types of cysts. An operative cholangiography was performed in 80 cases, among which an analysis of the status of the pancreaticobiliary ductal union was possible in 21 cases. A long common channel (>1.5 cm) was found in all 21 cases, 16 cases being of the P- type and 5 cases of the B- type. A cyst excision and hepaticojejunostomy was performed in 79 cases of type I and type IV cysts, and a liver biopsy only was performed in one case of a type V cyst. A common bile duct stone was associated with one case and cholangitis was associated with two cases as postoperative complications. One patient expired due to heart disease. Seventy-ine patients were followed up for a mean period of 7.2 years after the operation. Seventy-ight patients are healthy now, and liver function tests have returned to normal level. One Carolie's disease patient has been followed up outpatiently without bad liver function. An intraoperative liver biopsy was performed in 73 cases. The results were normal in 3 cases; cholestasis was noted in 2 cases, inflammation in 26, fibrosis in 38, and cirrhosis in 4. CONCLUSIONS: APBDU seems to play an important role in the pathogenesis of choledochal cysts, especially type I and type IV cysts. Excision of the cyst is the procedure of choice for treatment because it eliminates the APBDU and prevents both hepatic cirrhosis and the potential for developing a malignancy.