Kamisawa T, Ando H, Suyama M, et al. Japanese clinical practice guidelines for pancreaticobiliary maljunction. J Gastroenterol 47: , 2012.

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4 Kamisawa T, Ando H, Suyama M, et al. Japanese clinical practice guidelines for pancreaticobiliary maljunction. J Gastroenterol 47: , 2012.

5 Kamisawa T, Ando H, Hamada Y, et al. and The Japanese Study Group on Pancreaticobiliary Maljunction. Diagnostic criteria for pancreaticobiliary maljunction J Hepatobiliary Pancreat Sci 21: , Alonso-Lej F, Rever WB Jr, Pessagno DJ. Congenital choledochal cyst, with a report of 2 and analysis of 94 cases. Int Abstr Surg 108:1-30, Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134: , Itoi T, Kamisawa T, Fujii H, et al. Extrahepatic bile duct measurement by using transabdominal ultrasound in Japanese adults: multi-center prospective study. J. Gastroenterol 48: , Kamisawa T, Ando H, Shimada M, et al. Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committeej Hepatobiliary Pancreat Sci 21: 87-92, ) Ando H, Ito T, Kaneko K, et al. Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Coll Surg 181: , 1995.

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15 !! MRCP ERCP MRCP )4)-7) MRCP ERCP )-8) )-3)5)6) )4)6)7) ) ERCP MRCP X 1)-8) 1),. MR Cholangiopancreatography (MRCP). Consensus Controversy. 1997: 58-62EO 2) Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg 1998; 85: (CS) 3) Irie H, Honda H, Jimi M, et al. Value of MR cholangiopancreatography in evaluating choledochal cysts. Am J Roentgenol 1998; 171: (CS) 4) Hirohashi S, Hirohashi R, Uchida H, et al. Pancreatitis: evaluation with MR cholangiopancreatography in children. Radiology 1997; 203: (CCS) 5) Sugiyama M, Baba M, Atomi Y, et al. Diagnosis of anomalous pancreaticobiliary junction: value of magnetic resonance cholangiopancreatography. Surgery 1998; 123: (CS) 6) Matos C,Nicaise N, Deviere J, et al. Choledochal cysts: comparison of findings at MR cholangiopancreatography and endoscopic retrograde cholangiopancreatography in eight patients. Radiology 1998; 209: (CS)

16 7) Kim MJ, Han SJ, Yoon CS, et al. Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. Am J Roentgenol 2002; 179: (CS) 8Kamisawa T, Tu Y, Egawa N, et al. MRCP of congenital pancreaticobiliary malformation. Abdom Imaging 2007; 32: (CS) 1 MRCP!!

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24 !!! 1) 20%n=20 2) n=2, % 3) %32.1%4.7% 3) )-12) 71 1) 60.1± ± ± )13) 1) Tashiro S, Imaizumi T, Ohkawa H, et al. Committee for Registration of the Japanese Study Group on Pancreaticobiliary Maljunction. Pancreaticobiliary maljunction: retrospective and nationwide survey in Japan. J Hepatobiliary Pancreat Surg 2003; 10: (CS) 2) Rossi RL, Silverman ML, Braasch JW, et al. Carcinomas arising in cystic conditions of the bile ducts. A clinical and pathologic study. Ann Surg 1987; 205: (CS) 3),,, : 31: (CS) 4),,, ; 79: (CR)

25 5),,, ; 18: 534. (CS) 6) Iwai N, Deguchi E, Yanagihara J, et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg 1990; 25: (CR) 7),,, ; 32: (CR) 8),,, ; 34: (CR) 9),,, ; 21: (CR) 10) Tanaka S, Kubota M, Yagi M, et al. An 11-year-old male patient demonstrating cholangiocarcinoma associated with congenital biliary dilatation. J Pediatr Surg 2006; 41: e15-e19. (CR) 11) Nakamura H, Katayose Y, Rikiyama T, et al. Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease. J Hepatobiliary Pancreat Surg 2008; 15: (CR) 12) holangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy. J Pediatr Surg 2009; 44: (CR) 13) Hasumi A, Matsui H, Sugioka A, et al. Precancerous conditions of biliary tract cancer in patients with pancreaticobiliary maljunction: reappraisal of nationwide survey in Japan. J Hepatobiliary Pancreat Surg 2000; 7: (CS) 115 No. 1 Nakasako /M + 2 Oyama /F + 3 Iwai /F + 4 Kuriyama /F + 5 Yamashita /F - 6 Ueda /F + 7 Tanaka /M + 8 Nakamura /F + 9 Saikusa /M +

26 !! ) % ) 3) 4)-6) 6)7) 36 8)-10) 1),,, : 31: (CS) 2) Saikusa N, Naito S, Iinuma Y, et al. Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy. J Pediatr Surg 2009; 44: (CS) 3),,, ; 31: 48. (CS) 4),,, ; 43: (CR) 5),,,.. 31

27 2008; 31: 49 (CS) 6), ; 29: ),,, ; 31: (CR) 8) Okada T, Sasaki F, Ueki S, et al. Postnatal management for prenatally diagnosed choledochal cysts. J Pediatr Surg 2004; 39: (CS) 9),, ; 36: (CS) 10),,, ; 95: (EO)! 2 C! C! ( C) 1)2) 3) cholangio-venous reflux 4) lithostathine Lithostathine pancreatic stone protein Lithostathine lithostathine 5) 6) Ⅴ X MR

28 1) 1) T 1)7) 1) 23 8)9) 8) 9) 10) 11) 12) lithostathine 1) Kaneko K, Ando H, Ito T, et al. Protein plugs cause symptoms in patients with choledochal cysts. Am J Gastroenterol 1997; 92: (CS) 2) Kaneko K, Ono Y, Tainaka T, et al. Fatty acid calcium stones in patients with pancreaticobiliary maljunction/choledochal cyst as another cause of obstructive symptoms besides protein plugs. J Pediatr Surg 2008; 43: (CS) 3) Jeong JB, Whang JH, Ryu JK, et al. Risk factors for pancreatitis in patients with anomalous union of pancreatobiliary duct. Hepatogastroenterology 2004; 51: (CS) 4) Todani T, Urushihara N, Watanabe Y, et al. Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum. J Pediatr Surg 1990; 25: (CS) 5) Kaneko K, Ando H, Seo T, et al. Proteomic analysis of protein plugs: causative agent of symptoms in patients with choledochal cyst. Dig Dis Sci 2007; 52: (CS)

29 6) Kaneko K, Ono Y, Tainaka T, et al. Acidic and basic solutions dissolve protein plugs made of lithostathine complicating choledochal cyst/pancreaticobiliary maljunction. Dig Dis Sci 2009; 54: (CR) 7) Terui K, Yoshida H, Kouchi K, et al. Endoscopic sphincterotomy is a useful preoperative management for refractory pancreatitis associated with pancreaticobiliary maljunction. J Pediatr Surg 2008; 43: (CS) 8) Ando H, Kaneko K, Ito F, et al. Surgical removal of protein plugs complicating choledochal cysts: primary repair after adequate opening of the pancreatic duct. J Pediatr Surg 1998; 33: (CS) 9) Diao M, Li L, Zhang JS, et al. Laparoscopic-assisted clearance of protein plugs in the common channel in children with choledochal cysts. J Pediatr Surg 2010; 45: (CS) 10) Ando H, Kaneko K, Ito T, et al. Complete excision of the intrapancreatic portion of choledochal cysts. J Am Coll Surg 1996; 183: (CS) 11) Komuro H, Makino SI, Yasuda Y, et al. Pancreatic complications in choledochal cyst and their surgical outcomes. World J Surg 2001; 25: (CS) 12) Chiba K, Kamisawa T, Egawa N. Relapsing acute pancreatitis caused by protein plugs in a remnant choledochal cyst. J Hepatobiliary Pancreat Sci 2010; 17: ! 1 B! - 1 B! ( 1 B)! D % 21.6% 62.3% 32.1%

30 ) ( 2) 11 12) 13-16) -A 15) 16 1Todani T, Tabuchi K, Watanabe Y, et al. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979; 44: (CS) 2Morine Y, Shimada M, Takamatsu H, et al. Clinical features of pancreaticobiliary maljunction: update analysis of 2 nd Japan-nationwide survey. J Hepatobiliary Pancreat Sci 2013; 20: (CS) 3Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977; 134: (CS) 4Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet 1978; 146: Todani T, Watanabe Y, Toki A, et al. Carcinoma related to choledochal cysts with internal drainage operations. Surg GynecoI Obstet 1987; 164: (CCS) 6) Strong RW. Late bile duct cancer complication biliary-enteric anastomosis for benign disease. Am J Surg 1999; 177: (CCS) 7) Kobayashi S, Asano T, Yamasaki M, et al. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999; 126: (CS)

31 8Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6 : (CS) 9Yoshikawa K, Yoshida K, Shirai Y, et al. A case of carcinoma arising in the intrapancreatic terminal choledochus 12 years after primary excision of a giant choledochal cyst : Am J Gastroentero : 81: (CR) 10) Urushihara N, Fukumoto K, Fukuzawa H, et al. Long-term outcomes after excision of choledochal cysts in a single institution: operative procedures and late complications. J Pediatr Surg 2012; 47: (CS) 11) Ando H, Kaneko K, Ito T, et al. Complete excision of the intrapancreatic portion of choledochal cysts. J Am Coll Surg 1996; 183: (CCS) 12) Miyano T, Yamataka A, Kato Y, et al. Choledochal cyst: special emphasis on the usefulness of intraoperative endoscopy. J Pedatr Surg 1995; 30: (CS) 13) Pal K, Singh VP, Mitra DK. Partial hepatectomy and total cyst excision is curative for localized type IV-a biliary duct cysts - report of four cases and review of management. Eur J Pediatr Surg. 2009; 19: (CS) 14Kawarada Y, Das BC, Tabata M, et al. Surgical treatment of type choledochal cysts. J Hepatobiliary Pancreat Surg 2009; 16: (CS) 15Zheng X, Gu W, Xia H, et al. Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg 2013; 48: (CS) 16He XD, Wang L, Liu Q, et al. The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases. Ann Hepatol 2014; 13: (CS)! 1 C! 2 C! C

32 1 80% Historical control A non-rct Todani T, Watanabe Y, Urushihara N, et al. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 1995; 30: (CS) 2) Ando H Ito T Kaneko K et a1. Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Coll Surg l995; 181: (CS) 3Ando H, Kaneko K, Ito F, et al. Operative treatment of congenital stenosis of the intrahepatic bile ducts in patients with choledochal cysts. Am J Surg 1997; 173: (CS) 4Todani T. Watanabe Y, Mizuguchi T. et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg 1981; 142: (CCS) 5Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet 1978; 146: Todani T, Narusue M, Watanabe Y, et al. Management of congenital choledochal cyst with intrahepatic involvement. Ann Surg 1978; 187: (EO) 7) Urushihara N, Fukumoto K, Fukuzawa H, et al. Long-term outcomes after excision of choledochal cysts in a single institution: operative procedures and late complications.

33 J Pediatr Surg 2012; 47: (CS) 8) Pal K, Singh VP, Mitra DK. Partial hepatectomy and total cyst excision is curative for localized type IV-a biliary duct cysts - report of four cases and review of management. Eur J Pediatr Surg. 2009; 19: (CS) 9Kawarada Y, Das BC, Tabata M, et al. Surgical treatment of type Ⅳ choledochal cysts. J Hepatobiliary Pancreat Surg 2009; 16: (CS) 10Zheng X, Gu W, Xia H, et al. Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg 2013; 48, (CS) 11Koshinaga TInoue MOhashi K. et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci 2011; 18: (CS)! Roux-en-Y B! Roux-en-Y B Roux-en-Y simple 12 Roux-en-Y 34) 6 meta-analysis outcome Bile leakcholangitis Anastomotic stricturereflux/gastritisadhesive intestinal obstructionreoperation ratesoperative timehospital stay Bile leak RR:1.50[ ](P=0.46)Cholangitis RR:1.07[ ] (P=0.89)Anastomotic stricture RR:1.45[ ] (P=0.60) Reflux/gastritis RR:0.08[ ] (P=0.002)

34 Adhesive intestinal obstruction RR:2.77[ ](P=0.39) Reoperation rates RR:2.14[ ] (P=0.20)Operative time Hospital stay MD:0.30[ ] (P=0.29) 5 56 Roux-en-Y Roux-en-Y ; 13 : (CS) 2Todani T, Watanabe Y, Mizuguchi T, et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg 1981; 142: (CS) 3Takada K, Hamada Y, Watanabe K, et al. Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst. Pediatr Surg lnt 2005; 21:1-4. (CS) 4Shimotakahara A,Yamataka A, Yanai T, et al. Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst; which is better? Pediatr Surg lnt 2005; 21: 5-7. (OS) 5Narayanan SK, Chen Y, Narasimhan KL, et al. Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: A systematic review and meta-analysis. J Pediatr Surg 2013; 48: (SR) 6Santore MTBehar BJ Blinman TAet al. Hepaticoduodenostomy vs hepaticojejunostomy for reconstruction after resection of choledochal cyst. J Pediatr Surg 2011; 46: (OS)! 2 C <> 1,, 2, 3), 4,

35 5, 6), 7), T 8,, 9), T, l0,11 12) 1) Ando K. Miyano T. Kohno S. et al. Spontaneous perforation of choledochal cyst a study of 13 cases. Eur J Pediatr Surg 1998 ; 8 : (CS) 2, : 46: (CS) 3) Lilly JR, Weintraub WH, Altman RP. Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 1974 ; 75 : (CS) 4) Davies PA, Elliot-Smith A. Bile peritonitis in infancy. Arch Dis Child 1955 ; 30 : (CS) 5) Snyder WH, Chaffin L, Oettinger L. Cholelithiasis and perforation of the gallbladder in an infant. with recovery. Am Med Assoc 1952 ; 149 : (CS) 6,, ; 9 : (CS) 7) Dunn DC, Lees VC. Spontaneous perforation of the common bile duct in infancy. Br J Surg 1986 ; 73 : 929. (CS) 8) Howard ER, Johnston DI, Mowat AP. Spontaneous perforation of common bile duct in infants. Arch Dis Child 1976 ; 51 : (CS) 9) ; 32 : (CS) 10) Todani T, Tabuchi K, Watanabe Y, et al. Perforated choledochal cyst in children. Z Kinderchir 1978 ; 23 : (CS) 11) Franga DL, Howell CG, Mellinger JD, et al. Single-stage reconstruction of perforated choledochal cyst : case report and review of the literature. Am Surg 2005 ; 7 : (CR) 12),,, ; 42 : (CR)

36 !,, C! ( C) <>,,,, l IV-A, 10% 1 5,, 0.7% 120~200 6,7 8,, 6,7 1) Todani T, Watanabe Y, Urushihara N, et al. Biliary complications after excisional procedure for choledochal cyst. Pediatr Surg 1995 ; 30 : (CS) 2) Chijiiwa K, Tanaka M. Late complications after excisional operation in patients with choledochal cyst. J Am College Surg 1994 ; 179 : (CS) 3) Saing H, Han H, Chan KL, et al. Early and late results of excision of choledochal cysts. J Pediatr Surg 1999 ; 32 : (CS) 4) Tsuchida Y, Takahashi A, Suzuki N, et al. Development of intrahepatic biliary stones after excision of choledochal cysts. J Pediatr Surg 2002 : 37 : (CS) 5),,, ; 31 : (CS) 6) Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for

37 choledochal cyst. Hepatobiliary Pancreat Surg 1999 ; 6 : (CS) 7) Kobayashi S, Asano T, Yamasaki M, et al. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary ma!junction. Surgery 1999 ; 126 : (CS) 8) Strong RW. Late bile duct cancer complication biliary-enteric anastomosis for benign disease. Am J Surg 1999 ; 177 : (CS)! 0.7~5.4% C <> l,,,, 2 3,4,, 5~8),, 9~11), 12~14),,, % 15,,,, Watanabe 16) 0.7%, 17) (1.4%), 18) % Kobayashi 19) %, Todani 20) ,

38 15,, 1). l : (CPG) ; 53 : (CS) 3, 2008 ; 29 : (CS) 4) Todani T, Watanabe Y, Urushihara N, et al. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 1995 ; 30 : (CS) 5) Tsuchida A, Kosuya K, Endo M, et al. High risk of bile duct carcinogenesis after primary resection of a congenital biliary dilatation. Oncol Rep 2003 ; 10 : (CS) ; 36 : (EO) 7,,, ; 28 : (CR) 8,. 40 l ; 31 : (CS) ; 22 : (CS) 10, ; 22 : (CS) 11) Hara H, Morita S, Ishibashi T, et al. Surgical treatment for non-dilated biliary tract with pancreaticobiliary maljunction should include excision of the extrahepatic bile duct. Hepatogastroenterology 2011 ; 48 : (CS) 12,, ; 29 : (CS) 13) Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg 1998 ; 85 : (CS) 14) Aoki Y, Tsuchida A, Kasuya K, et al. Is preventive resection of the extrahepatic bile

39 duct necessary in case of pancreaticobiliary maljunction without dilatation of the bile duct? Jpn J Cli Oncol 2001 ; 31 : (CCS) 15,,, ; 31 : (CS) 16) Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. Hepatobiliary Pancreat Surg 1999 ; 6 : (CS) 17,, ; 31 : (CS) 18,,, ; 31 : (CS) 19) Kobayashi S, Asano T, Yamasaki M, et al. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery 1999 ; 126 : (CS) 20) Todani T, Watanabe Y, Toki A, et al. Carcinoma related to choledochal cysts with internal drainage operation. Surg Gynec Obstet 1987 ; 164 : (CS)