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Clinical significance of anomalous pancreaticobiliary union
0016-5107/83/2902-0094$02.00/0 GASTROINTESTINAL ENDOSCOPY Copyright © 1983 by the American Society for Gastrointestinal Endoscopy
Clinical significance of anomalous pancreaticobiliary union Osamu Kato, MD Kazuhiko Hattori, MD Takashi Suzuki, MD Fumio Tachino, MD Tomoyo Yuasa, MD Nagoya, Japan Anomalous pancreaticobiliary union was found in nine cases among 300 consecutive adult patients examined by endoscopic retrograde cholangiopancreatography. Three had congenital choledochal cysts: one had a cyst of the choledochus associated with a cyst of the intrahepatic bile duct, another had a fusiform dilation of the choledochus, and the third had a choledochal diverticulum. Five of the nine patients had biliary malignancies (55.6%): four carcinomas of the gallbladder and one carcinoma of the choledochus. On the other hand, 18 of 291 patients without anomalous pancreaticobiliary union had biliary malignancies (6.2%): four carcinomas of the gallbladder and 14 carcinomas of the choledochus. When anomalous pancreaticobiliary union is detected, biliary malignancy, especially carcinoma of the gallbladder, should be considered as a possible complication.
Anomalous pancreaticobiliary (P-B) union has attracted our attention due to its association with congenital choledochal cyst. 1, 2 With the introduction of endoscopic retrograde cholangiopancreatography (ERCP) the recognition of anomalous P-B union became feasible and the condition was not always found to be associated with congenital choledochal cyst. 3 In this article, we review nine patients with anomalous P-B union and discuss the radiologic appearance of the P-B system, its clinical significance, and complications. MATERIALS AND METHODS
During the period from April 1978 to December 1981, 300 patients suspected of having pancreatic or biliary disorders were examined by ERCP. ERCP was performed utilizing a lateral viewing instrument in almost every patient. A forward viewing instrument was used in two patients who had Billroth-II gastrojejunostomies. The diagnosis of anomalous P-B union was made From the Department of Internal Medicine, Fujita Gakuen University School of Medicine, The Second Hospital, Nagoya, Japan. Reprint requests: Osamu Kato, MD, Department of Internal Medicine, Fujita Gakuen University School of Medicine, The Second Hospital, 3-6-10 Otobashi, Nakagawa-ku, Nagoya 454, Japan. 94
when a common channel longer than 20 mm was found and/or one duct joined perpendicularly to another. RESULTS
Nine among 300 patients (3.0%) examined by ERCP had anomalous P-B union. Their ages ranged from 39 to 80 years; five were women and four were men. As to their clinical symptoms and laboratory data, upper abdominal pain, the most preponderant complaint, was seen in six and jaundice was noted in six. An elevated serum alkaline phosphatase was observed in all except one patient. Transient hyperamylasemia was observed in four patients who had clinical evidence of acute pancreatitis (Table 1). Various modes of anomalous P-B union were noted as shown in Figure 1. Three had long common channels (type A) (Fig. 2). Four had common bile ducts joining perpendicularly to the main pancreatic duct (type B) (Fig. 3), and the other two had the reverse (type C). The diameters of the common bile duct ranged from 5 to 60 mm. Five cases had dilated common bile ducts more than 15 mm in diameter, and two cases were considered to have congenital dilation of the bile ducts (Figs. 4 and 5). Dilation of the bile duct in the other three cases was believed due to biliary calculi. One of the four patients without dilation of the bile duct had GASTROINTESTINAL ENDOSCOPY
Table 1. Clinical profile in patients with anomalous pancreaticobiliary union. Case
Age (year)
1 2 3
50 71 51
4 5 6 7
80 59 77
F F F
39
8 9
75 77
a b
Sex
Chief complaint
F F
Alkaline phosb h tas a Serum amylase poe ( .) (unit) urut
Total bilirubin (mg/dl)
Epigastric pain Anorexia Right upper quadrant pain
20.7 43.9 31.0
434 176 150
2.6 13.0 0.7
M
Right upper quadrant pain Anorexia Right upper quadrant pain Back pain
18.5 27.8 14.4 17.9
476 671 278 963
8.2 6.5 0.6 7.6
M M
Right upper quadrant pain Epigastric pain
66.4 8.2
35 185
16.3 0.6
M
Diagnosis Cancer of the gallbladder Cancer of the gallbladder Cancer of the gallbladder, choledochal cyst Choledocholithiasis Cholelithiasis Cancer of the gallbladder Choledocholithiasis, biliary cysts Cancer of the choledochus Choledochal cyst (diverticulum)
Normal: 2.0 to 10.0 units. Normal: 60 to 390 units.
Type
yw
Type
C
Case 1*
A
Case 6*
A
Case 7
0
Case 2*
C PW 0
Case 3
*
}'W .J-w C
0
Case 4
0
Case 5
A
case
C
W
o~ C
0-'-
8**
B
B
W C
C
~W 0 B
Case 9
C C
o~
B
C
o~w 0: Orifice C: Choledochus
* **
Ca. of the gall bladder Ca.of the choledochus
W: Wirsung duct S: Santorini duct D: Diverticulum
Figure 1. Schema of the pancreaticobiliary union in nine patients.
a choledochal diverticulum (Fig. 6). The length of the pancreaticobiliary common channel varied from 5 to 35 mm. Five cases had common channels longer than 20 mm in length. VOLUME 29, NO.2, 1983
In eight of the nine patients anomalous P-B union was complicated by some other biliary disorder (Table 1). Five of the nine patients had biliary malignancies (55.6%): four had carcinomas of the gallbladder and 95
Figure 2. ERCP (case 1). Pancreaticobiliary union occurs at a high position (long common channel).
Figure 3. ERCP (case 6). The common bile duct enters the Wirsung duct at a high position. The Santorini duct constitutes the main pancreatic duct and the Wirsung duct barely communicates with the Santorini duct via a branch.
one had carcinoma of the common bile duct. By contrast, only 18 of the 291 patients without anomalous P-B union had biliary malignancies (6.2%): four had carcinomas of the gallbladder and 14 had carcinomas of the choledochus. A pancreatogram was obtained in all nine patients, seven of whom had abnormal pancreatograms (77.8%), 96
suggesting the existence of chronic pancreatitis. On the other hand, an abnormal pancreatogram was detected in only 24 of 65 patients (36.9%) who had some kind of biliary disorder but no anomalous P-B union. Furthermore, an episode of acute pancreatitis was encountered in four patients with anomalous P-B union, including a case having a normal pancreatogram. GASTROINTESTINAL ENDOSCOPY
DISCUSSION
Figure 4. ERCP (case 3). Pancreaticobiliary union occurs at a high position. Fusiform dilation of the choledochus is seen.
Anomalous P-B union has often been associated with choledochal cyst. I, 2 Although many descriptions of anomalous P-B union have been found, especially in the Japanese literature,3-5 a definition of anomalous P-B union has yet to be established. Six of the nine patients reported had no congenital choledochal cyst. Some authors have already noted that anomalous PB union is not always associated with dilation of the bile duct. 3, 6 Anomalous P-B union cannot be regarded as the only etiologic factor for choledochal cyst if it is not always associated with choloedochal cyst. Kozloff et al. 7 had five adult cases of biliary cysts which they believed were acquired rather than congenital. The high incidence of biliary carcinoma in patients with choledochal cyst has been stressed. 8 Recently, association of carcinoma of the gallbladder in patients with anomalous P-B union has been reported by several authors. 9- 11 Our study verifies the association of carcinoma of the gallbladder with anomalous P-B union. Do patients with anomalous P-B union develop carcinoma of the gallbladder because of reflux and stagnation of pancreatic juice in the gallbladder? 10, II This hypothesis, although interesting, remains unproven. Although several authors report that an abnormal pancreatogram is uncommon in patients with anomalous P-B union,3, 5, 9,12 we detected an abnormal pancreatogram in seven of our nine patients. This finding might be expected as bile flows easily into the pancreatic duct via the anomalous P-B union. We conclude that the most serious complication of anomalous P-B union is carcinoma of the gallbladder; other fre-
Figure 5. ERCP (case 7). The common bile duct enters the main pancreatic duct perpendicularly. A cyst of the choledochus is seen. VOLUME 29, NO.2, 1983
97
Figure 6. ERCP (case 9). The main pancreatic duct bends sharply and narrows where it joins perpendicularly to the common bile duct. A diverticulum is also seen.
quent complications are pancreatitis and biliary calculi. REFERENCES 1. Babbit DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of common bile duct and pancreatic bulb. Ann Radioll969;12:231-40. 2. Babbit DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J RoentgenoI1973;119:57-62. 3. Yoshimoto S, Sakon M, Hirai T, et a1. Clinical study on anomalous relationships of common bile duct and pancreatic duct. Gastroenterol Endosc 1979;21:170-7. 4. Oi I, Hara T. Abnormal connection between the choledochus and the pancreatic duct in case of congenital choledochal cyst examined by endoscopic pancreatocholangiography. Jpn J Pediat Surg 1977;9:1121-9. 5. Kimura K. Studies in 28 cases of congenital cystic dilatation of the common bile duct in adults. Roentgenological features and a union between the choledochus and the main pancreatic duct. Jpn J GastroenteroI1976;73:401-14.
98
6. McNulty JG. Radiology of the liver. Philadelphia, WB Saunders, 1977:178-88. 7. Kozloff L, Joseph WL. Cystic dilatation of the common bile duct in adults. Med Ann DC 1973;42:595-7. 8. Flanigan DP. Biliary cysts. Ann Surg 1975;182:635-43. 9. Sano H, Nakazawa S, Naito Y. Radiological study ofthe abnormal pancreaticocholangio-connection, with special reference to the "congenital choledochal cyst." Gastroenterol Endosc 1981;23:1722-35. 10. Kinoshita H, Nagata E, Machi Y, et a1. Carcinoma of the gall bladder with anomalous arrangement between the choledochus and pancreatic duct. The biliary tract and pancreas (Japanese). 1981;2:1701-9. 11. Konishi T, Nagakawa T, Jinno M, et a1. Four cases of anomalous arrangement ofpancreaticobiliaryduct associated with gall bladder cancer. The biliary tract and pancreas (Japanese). 1981;2:435-41. 12. Miyata S, Shibue T, Osame T, et al. A clinical investigation of 17 cases of idiopathic choledochus dilatation in adult diagnosed by ERCP with special reference to cholangiogram and anomalous pancreaticobiliary ductal anastomosis. Gastroenterol Endose 1979;21:430-9.
GASTROINTESTINAL ENDOSCOPY
Clinical significance of anomalous pancreaticobiliary union Osamu Kato, MD Kazuhiko Hattori, MD Takashi Suzuki, MD Fumio Tachino, MD Tomoyo Yuasa, MD Nagoya, Japan Anomalous pancreaticobiliary union was found in nine cases among 300 consecutive adult patients examined by endoscopic retrograde cholangiopancreatography. Three had congenital choledochal cysts: one had a cyst of the choledochus associated with a cyst of the intrahepatic bile duct, another had a fusiform dilation of the choledochus, and the third had a choledochal diverticulum. Five of the nine patients had biliary malignancies (55.6%): four carcinomas of the gallbladder and one carcinoma of the choledochus. On the other hand, 18 of 291 patients without anomalous pancreaticobiliary union had biliary malignancies (6.2%): four carcinomas of the gallbladder and 14 carcinomas of the choledochus. When anomalous pancreaticobiliary union is detected, biliary malignancy, especially carcinoma of the gallbladder, should be considered as a possible complication.
Anomalous pancreaticobiliary (P-B) union has attracted our attention due to its association with congenital choledochal cyst. 1, 2 With the introduction of endoscopic retrograde cholangiopancreatography (ERCP) the recognition of anomalous P-B union became feasible and the condition was not always found to be associated with congenital choledochal cyst. 3 In this article, we review nine patients with anomalous P-B union and discuss the radiologic appearance of the P-B system, its clinical significance, and complications. MATERIALS AND METHODS
During the period from April 1978 to December 1981, 300 patients suspected of having pancreatic or biliary disorders were examined by ERCP. ERCP was performed utilizing a lateral viewing instrument in almost every patient. A forward viewing instrument was used in two patients who had Billroth-II gastrojejunostomies. The diagnosis of anomalous P-B union was made From the Department of Internal Medicine, Fujita Gakuen University School of Medicine, The Second Hospital, Nagoya, Japan. Reprint requests: Osamu Kato, MD, Department of Internal Medicine, Fujita Gakuen University School of Medicine, The Second Hospital, 3-6-10 Otobashi, Nakagawa-ku, Nagoya 454, Japan. 94
when a common channel longer than 20 mm was found and/or one duct joined perpendicularly to another. RESULTS
Nine among 300 patients (3.0%) examined by ERCP had anomalous P-B union. Their ages ranged from 39 to 80 years; five were women and four were men. As to their clinical symptoms and laboratory data, upper abdominal pain, the most preponderant complaint, was seen in six and jaundice was noted in six. An elevated serum alkaline phosphatase was observed in all except one patient. Transient hyperamylasemia was observed in four patients who had clinical evidence of acute pancreatitis (Table 1). Various modes of anomalous P-B union were noted as shown in Figure 1. Three had long common channels (type A) (Fig. 2). Four had common bile ducts joining perpendicularly to the main pancreatic duct (type B) (Fig. 3), and the other two had the reverse (type C). The diameters of the common bile duct ranged from 5 to 60 mm. Five cases had dilated common bile ducts more than 15 mm in diameter, and two cases were considered to have congenital dilation of the bile ducts (Figs. 4 and 5). Dilation of the bile duct in the other three cases was believed due to biliary calculi. One of the four patients without dilation of the bile duct had GASTROINTESTINAL ENDOSCOPY
Table 1. Clinical profile in patients with anomalous pancreaticobiliary union. Case
Age (year)
1 2 3
50 71 51
4 5 6 7
80 59 77
F F F
39
8 9
75 77
a b
Sex
Chief complaint
F F
Alkaline phosb h tas a Serum amylase poe ( .) (unit) urut
Total bilirubin (mg/dl)
Epigastric pain Anorexia Right upper quadrant pain
20.7 43.9 31.0
434 176 150
2.6 13.0 0.7
M
Right upper quadrant pain Anorexia Right upper quadrant pain Back pain
18.5 27.8 14.4 17.9
476 671 278 963
8.2 6.5 0.6 7.6
M M
Right upper quadrant pain Epigastric pain
66.4 8.2
35 185
16.3 0.6
M
Diagnosis Cancer of the gallbladder Cancer of the gallbladder Cancer of the gallbladder, choledochal cyst Choledocholithiasis Cholelithiasis Cancer of the gallbladder Choledocholithiasis, biliary cysts Cancer of the choledochus Choledochal cyst (diverticulum)
Normal: 2.0 to 10.0 units. Normal: 60 to 390 units.
Type
yw
Type
C
Case 1*
A
Case 6*
A
Case 7
0
Case 2*
C PW 0
Case 3
*
}'W .J-w C
0
Case 4
0
Case 5
A
case
C
W
o~ C
0-'-
8**
B
B
W C
C
~W 0 B
Case 9
C C
o~
B
C
o~w 0: Orifice C: Choledochus
* **
Ca. of the gall bladder Ca.of the choledochus
W: Wirsung duct S: Santorini duct D: Diverticulum
Figure 1. Schema of the pancreaticobiliary union in nine patients.
a choledochal diverticulum (Fig. 6). The length of the pancreaticobiliary common channel varied from 5 to 35 mm. Five cases had common channels longer than 20 mm in length. VOLUME 29, NO.2, 1983
In eight of the nine patients anomalous P-B union was complicated by some other biliary disorder (Table 1). Five of the nine patients had biliary malignancies (55.6%): four had carcinomas of the gallbladder and 95
Figure 2. ERCP (case 1). Pancreaticobiliary union occurs at a high position (long common channel).
Figure 3. ERCP (case 6). The common bile duct enters the Wirsung duct at a high position. The Santorini duct constitutes the main pancreatic duct and the Wirsung duct barely communicates with the Santorini duct via a branch.
one had carcinoma of the common bile duct. By contrast, only 18 of the 291 patients without anomalous P-B union had biliary malignancies (6.2%): four had carcinomas of the gallbladder and 14 had carcinomas of the choledochus. A pancreatogram was obtained in all nine patients, seven of whom had abnormal pancreatograms (77.8%), 96
suggesting the existence of chronic pancreatitis. On the other hand, an abnormal pancreatogram was detected in only 24 of 65 patients (36.9%) who had some kind of biliary disorder but no anomalous P-B union. Furthermore, an episode of acute pancreatitis was encountered in four patients with anomalous P-B union, including a case having a normal pancreatogram. GASTROINTESTINAL ENDOSCOPY
DISCUSSION
Figure 4. ERCP (case 3). Pancreaticobiliary union occurs at a high position. Fusiform dilation of the choledochus is seen.
Anomalous P-B union has often been associated with choledochal cyst. I, 2 Although many descriptions of anomalous P-B union have been found, especially in the Japanese literature,3-5 a definition of anomalous P-B union has yet to be established. Six of the nine patients reported had no congenital choledochal cyst. Some authors have already noted that anomalous PB union is not always associated with dilation of the bile duct. 3, 6 Anomalous P-B union cannot be regarded as the only etiologic factor for choledochal cyst if it is not always associated with choloedochal cyst. Kozloff et al. 7 had five adult cases of biliary cysts which they believed were acquired rather than congenital. The high incidence of biliary carcinoma in patients with choledochal cyst has been stressed. 8 Recently, association of carcinoma of the gallbladder in patients with anomalous P-B union has been reported by several authors. 9- 11 Our study verifies the association of carcinoma of the gallbladder with anomalous P-B union. Do patients with anomalous P-B union develop carcinoma of the gallbladder because of reflux and stagnation of pancreatic juice in the gallbladder? 10, II This hypothesis, although interesting, remains unproven. Although several authors report that an abnormal pancreatogram is uncommon in patients with anomalous P-B union,3, 5, 9,12 we detected an abnormal pancreatogram in seven of our nine patients. This finding might be expected as bile flows easily into the pancreatic duct via the anomalous P-B union. We conclude that the most serious complication of anomalous P-B union is carcinoma of the gallbladder; other fre-
Figure 5. ERCP (case 7). The common bile duct enters the main pancreatic duct perpendicularly. A cyst of the choledochus is seen. VOLUME 29, NO.2, 1983
97
Figure 6. ERCP (case 9). The main pancreatic duct bends sharply and narrows where it joins perpendicularly to the common bile duct. A diverticulum is also seen.
quent complications are pancreatitis and biliary calculi. REFERENCES 1. Babbit DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of common bile duct and pancreatic bulb. Ann Radioll969;12:231-40. 2. Babbit DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J RoentgenoI1973;119:57-62. 3. Yoshimoto S, Sakon M, Hirai T, et a1. Clinical study on anomalous relationships of common bile duct and pancreatic duct. Gastroenterol Endosc 1979;21:170-7. 4. Oi I, Hara T. Abnormal connection between the choledochus and the pancreatic duct in case of congenital choledochal cyst examined by endoscopic pancreatocholangiography. Jpn J Pediat Surg 1977;9:1121-9. 5. Kimura K. Studies in 28 cases of congenital cystic dilatation of the common bile duct in adults. Roentgenological features and a union between the choledochus and the main pancreatic duct. Jpn J GastroenteroI1976;73:401-14.
98
6. McNulty JG. Radiology of the liver. Philadelphia, WB Saunders, 1977:178-88. 7. Kozloff L, Joseph WL. Cystic dilatation of the common bile duct in adults. Med Ann DC 1973;42:595-7. 8. Flanigan DP. Biliary cysts. Ann Surg 1975;182:635-43. 9. Sano H, Nakazawa S, Naito Y. Radiological study ofthe abnormal pancreaticocholangio-connection, with special reference to the "congenital choledochal cyst." Gastroenterol Endosc 1981;23:1722-35. 10. Kinoshita H, Nagata E, Machi Y, et a1. Carcinoma of the gall bladder with anomalous arrangement between the choledochus and pancreatic duct. The biliary tract and pancreas (Japanese). 1981;2:1701-9. 11. Konishi T, Nagakawa T, Jinno M, et a1. Four cases of anomalous arrangement ofpancreaticobiliaryduct associated with gall bladder cancer. The biliary tract and pancreas (Japanese). 1981;2:435-41. 12. Miyata S, Shibue T, Osame T, et al. A clinical investigation of 17 cases of idiopathic choledochus dilatation in adult diagnosed by ERCP with special reference to cholangiogram and anomalous pancreaticobiliary ductal anastomosis. Gastroenterol Endose 1979;21:430-9.
GASTROINTESTINAL ENDOSCOPY