Kongenitale zystische Dilatation des Ductus cysticus in Kombination mit einer Anomalie der pankreatikobiliären duktalen Junktion

Zusammenfassung

Wir berichten über eine zystische Dilatation des Ductus cysticus bei einer 40-jährigen Patientin. Sie hatte seit zwei Jahren nahrungsunabhängige, rechtsseitige Oberbauchschmerzen. In der B-Bildsonographie fand sich eine zystische Formation in unmittelbarer Nachbarschaft zur vergrößerten Gallenblase, zum Ductus hepatocholedochus und zur Pfortader. Durch eine endoskopische retrograde Cholangiographie gelang die Diagnosesicherung der zystischen Dilatation des Ductus cysticus sowie einer Anomalie der pankreatikobiliären duktalen Junktion (APBDJ; common channel). Eine Cholezystektomie mit Exzision des gesamten Ductus cysticus und des Ductus hepatocholedochus sowie Anlage einer Roux-Y-Hepatikojejunostomie ist aufgrund des erhöhten Risikos der Entstehung von Gallenwegs- und Gallenblasenkarzinomen bei Vorliegen einer zystischen Anomalie der Gallenwege sowie einer APBDJ Therapie der Wahl.

Abstract

We report a case of congenital cystic dilatation of the cystic duct detected in a 40-year-old woman. She had intermittent pain in the right upper quadrant of the abdomen independent of the intake of meals for the last two years. In the ultrasound we found a cystic formation in the vicinity of the enlarged gallbladder, the common hepatic duct/common bile duct and the portal vein. The endoscopic retrograde cholangiography confirmed a cystic duct malformation, which was associated with an anomalous pancreaticobiliary ductal junction (APBDJ). A cholecystectomy with excision of the whole cystic duct and common bile duct and Roux-en-Y hepaticojejunostomy is indicated because there is an increased risk of the development of bile duct cancer and gallbladder cancer in the presence of biliary cystic duct anomalies and APBDJ.

Schlüsselwörter

Zystische Dilatation des Ductus cysticus - Anomalie der pankreatikobiliären duktalen Junktion - Endoskopische retrograde Cholangiopankreatikographie - Therapie

Key words

Cystic dilatation of the cystic duct - Anomaly of the pancreaticobiliary junction - Endoscopic retrograde pancreatico-cholangiography - Therapy

Literatur

• 1 Tan K C, Howard E R. Choledochal cyst: A 14-year surgical experience with 36 patients.  Br J Surg. 1988;  75 892-895
• 2 Nagorney D M. Choledochal cysts in adult life. In: Blumgart LH (Ed) Surgery of the Liver and Biliary Tract. Vol. 2. Edinburgh, London, Melbourne, New York; Churchill Livingstone 1980: 1003-1012
• 3 Kimura K, Ohto M, Saisho H, Onozawa T, Tsuchiya Y, Morita M, Ebara M, Matsutani S, Okuda K. Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union.  Gastroenterology. 1985;  89 1258-1265
• 4 Chijiiwa K, Kimura H, Tanaka M. Malignant potential of the gallbladder in patients with anomalous pancreaticobiliary ductal union. The difference in risk between patients with and without choledochal cyst.  Int Surg. 1995;  80 61-64
• 5 Misra S P, Dwivedi M. Pancreaticobiliary ductal union.  Gut. 1990;  31 1144-1149
• 6 Yamaguchi S, Koga A, Matsumoto S, Tanaka M, Nakayama F. Anomalous junction of pancreaticobiliary duct without congenital choledochal cyst: a possible risk factor for gallbladder cancer.  Am J Gastroenterol. 1987;  82 20-24
• 7 Todani T, Watanabe Y, Fujii T, Toki A, Uemura S, Koike Y, Inoue T. Carcinoma arising from the bile duct in choledochal cyst and anomalous arrangement of the pancreaticobiliary ductal union (in Japanese).  Tan To Sui (J Bil Panc). 1985;  6 525-535
• 8 Aoki H, Sugaya H, Shimazu M. A clinical study on cancer of the bile duct associated with anomalous arrangements of pancreatico-biliary ductal system. Analysis of 569 cases collected in Japan (in Japanese).  Tan To Sui (J Bil Panc). 1987;  8 1539-1551
• 9 Voyles C R, Smadja C, Shands C, Blumgart L H. Carcinoma in choledochal cysts. Age-related incidence.  Arch Surg. 1983;  118 986-988
• 10 Reveille R M, van Stiegmann G, Everson G T. Increased secondary bile acids in choledochal cysts. Possible role in biliary metaplasia and carcinoma.  Gastroenterology. 1990;  99 525-527
• 11 Sasatoni E, Tokunaga O, Miyazakik K. Precancerous conditions of gallbladder carcinoma: overview of histopathologic characteristics and molecular genetic findings.  J Hepatobiliary Pancreat Surg. 2000;  7 556-567
• 12 Suzuki S, Nakamura S, Ochiai H, Baba S, Sakaguchi T, Tsuchiya Y, Kojima Y, Konno H. Double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction without a choledochal cyst: Report of a case.  Surg Today. 1999;  29 651-655
• 13 Lindberg C G, Hammarström L E, Holmin T, Lundsted C. Cholangiographic appearance of bile-duct cysts.  Abdom Imaging. 1998;  23 611-615
• 14 Nagorney D M, McPherson G A. Carcinoma of the gallbladder and extrahepatic bile ducts.  Semin Oncol. 1988;  15 106-115
• 15 Maram E S, Ludwig J, Kurland L T. Carcinoma of the gallbladder and extrahepatic biliary ducts in Rochester, Minnesota, 1935-1971.  Am J Epidemiol. 1979;  109 152-157
• 16 Flanigan D P. Biliary cyst.  Ann Surg. 1975;  182 635-643
• 17 Yoshida H, Itai Y, Minami M, Kokubo T, Ohtomo K, Kuroda A. Biliary malignancies occurring in choledochal cysts.  Radiology. 1989;  173 389-392
• 18 Bloustein P A. Association of carcinoma with congenital cystic conditions of the liver and bile ducts.  Am J Gastroenterol. 1977;  67 40-46
• 19 Crittenden S L, McKinley M J. Choledochal cyst-clinical features and classification.  Am J Gastroenterol. 1985;  88 643-647
• 20 Yosikane H, Hashimoto S, Hidano H, Sakakibara A, Ayakawa T, Mori S, Kojima Y. Multiple early bile duct carcinoma associated with congenital choledochal cyst.  J Gastroenterol. 1998;  33 454-457
• 21 Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst.  Am Surg. 1970;  172 844-851
• 22 Saito S, Ishida M. Congenital choledochal cyst. Cystic dilatation of the common bile duct.  Progr Pediat Surg. 1974;  6 63-90
• 23 Filler R M, Stringel G. Treatment of choledochal cyst by excision.  J Pediatr Surg. 1980;  15 437-442
• 24 Kim S H. Choledochal cyst: Survey by the surgical section of the American Academy of Pediatrics.  J Pediatr Surg. 1981;  16 402-407
• 25 Lilly J R. The surgical treatment of choledochal cyst.  Surg Gynecol Obstet. 1979;  149 36-42
• 26 Nunez-Hoyo M, Lees C D, Hermann R E. Bile duct cyst. Experience with 15 patients.  Am J Surg. 1982;  144 295-299
• 27 Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts. Classification, operative procedure, and review of thirty-seven cases including cancer arising from choledochal cyst.  Am J Surg. 1977;  134 263-269
• 28 Yamaguchi M. Congenital choledochal cyst.  Am J Surg. 1980;  140 653-657
• 29 Bode W E, Aust J B. Isolated cystic dilatation of the cystic duct.  Am J Surg. 1983;  154 828-829
• 30 Serradel A FS, Linares E S, Goepfert R H. Cystic dilatation of the cystic duct: A new type of biliary cyst.  Surgery. 1991;  109 320-322
• 31 Alonso-Lej F, Rever W B Jr, Pessagno D J. Congenital choledochal cyst, with a report of two, and an analysis of 94 cases.  Int Abst Surg. 1959;  108 1-30
• 32 Nagorney D M, McIlrath D C, Adson M A. Choledochal cysts in adults. Clinical management.  Surgery. 1984;  96 656-663
• 33 Weishorn H D, Seitz K. Sonographische Diagnostik bei Choledochuszysten.  Ultraschall Med. 1989;  10 132-135
• 34 Thatcher B S, Sivak M V, Hermann R E. ERCP in evaluation and diagnosis of choledochal cyst: report of five cases.  Gastrointest Endosc. 1986;  32 27-31
• 35 Little K H, Loeb P M. Choledochal cyst.  South Med J. 1989;  82 255-258
• 36 Todani T, Watanabe Y, Fujii T, Uemura S. Anomalous arrangement of the pancreaticobiliary ductal system in patients with a choledochal cyst.  Am J Surg. 1984;  147 672-676
• 37 Kagawa Y, Kashihara S, Kuramoto S, Martani S. Carcinoma arising in a congenitally dilated biliary tract. Report of a case and review of the literature.  Gastroenterology. 1978;  74 1286-1294
• 38 Shimada K, Yanagisawa J, Nakayama F. Increased lysophosphatidylcholine and pancreatic enzyme content in bile of patients with anomalous pancreaticobiliary ductal junction.  Hepatology. 1991;  13 438-444
• 39 Todani T, Tabuchi K, Mizuguchi T, Fujii T, Toki A. Hepaticoduodenostomy at hepatic hilium after excision of choledochal cysts.  Am J Surg. 1981;  142 584-587
• 40 Azuma T, Hanyu F, Nakamura M, Imaizumi T, Yoshikawa T, Araida T, Takasaki K. A clinical study on surgical treatment for anomalous arrangement of the pancreaticobiliary ductal system without dilatation of common bile duct (in Japanese with English abstract).  Jpn J Gastroenterol Surg. 1996;  29 806-812
• 41 Kaji K, Iwagami S, Hirose K, Hanatate F, Katada S, Nishiura K, Takabatake I, Mori Y, Hayashi T, Yamada T, Kitagawa S, Nakagawa M, Hayashi M. Occurrence of bile duct carcinoma after cholecystectomy associated with anomalous arrangement of pancreaticobiliary duct (in Japanese).  Jpn J Gastroenterol. 1989;  86 1589