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Kamisawa T, Ando H, Suyama M, et al. Japanese clinical practice guidelines for pancreaticobiliary maljunction. J Gastroenterol 47:731-759, 2012.

Kamisawa T, Ando H, Hamada Y, et al. and The Japanese Study Group on Pancreaticobiliary Maljunction. Diagnostic criteria for pancreaticobiliary maljunction 2013. J Hepatobiliary Pancreat Sci 21:159-161, 2014.

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, 1959.

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 134:263-269, 1977.

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 48:1045-50, 2013.

Kamisawa T, Ando H, Shimada M, et al. Recent advances and problems in the management of pancreaticobiliary maljunction: feedback from the guidelines committee J Hepatobiliary Pancreat Sci 21: 87-92, 2014.

12) Ando H, Ito T, Kaneko K, et al. Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Coll Surg 181:426-430, 1995.

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2) Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg 1998; 85: 911-916(CS) 3) Irie H, Honda H, Jimi M, et al. Value of MR cholangiopancreatography in evaluating choledochal cysts. Am J Roentgenol 1998; 171: 1381-1385(CS) 4) Hirohashi S, Hirohashi R, Uchida H, et al. Pancreatitis: evaluation with MR cholangiopancreatography in children. Radiology 1997; 203: 411-415 (CCS) 5) Sugiyama M, Baba M, Atomi Y, et al. Diagnosis of anomalous pancreaticobiliary junction: value of magnetic resonance cholangiopancreatography. Surgery 1998; 123: 391-397 (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: 443-448 (CS)

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: 209-214 (CS) 8 Kamisawa T, Tu Y, Egawa N, et al. MRCP of congenital pancreaticobiliary malformation. Abdom Imaging 2007; 32: 129-133 (CS)

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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: 345-351. (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: 377-384. (CS) 3) , , , .

. 2010: 31: 1293-1299. (CS) 4) , , , . . 1982; 79: 926-927. (CR)

5) , , , . 5 —10 . 1985; 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: 1261-1263. (CR) 7) , , , . 15

. 1997; 32: 314- 318. (CR) 8) , , , . 2

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1 . 2000; 21: 593-597. (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: 554-559. (CR) 12) holangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy. J Pediatr Surg 2009; 44: 2202-2205. (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: 551-555. (CS)

1 15 No.1 Nakasako 1982 14/M +2 Oyama 1985 10/F +3 Iwai 1990 12/F +4 Kuriyama 1997 15/F +5 Yamashita 1998 15/F -6 Ueda 2000 13/F +7 Tanaka 2006 11/M +8 Nakamura 2008 15/F +9 Saikusa 2009 3/M +

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. 31 2008; 31: 50-51. (CR) 8) Okada T, Sasaki F, Ueki S, et al. Postnatal management for prenatally diagnosed choledochal cysts. J Pediatr Surg 2004; 39: 1055-1058. (CS) 9) , , . . 6 .

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1) Kaneko K, Ando H, Ito T, et al. Protein plugs cause symptoms in patients with choledochal cysts. Am J Gastroenterol 1997; 92: 1018-1021. (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: 564-567. (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: 1187-1190. (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: 303-306. (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: 1979-1986. (CS)

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: 1475-1480.(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: 495-499. (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: 1265-1267. (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: 2099-2102(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: 317-321. (CS) 11) Komuro H, Makino SI, Yasuda Y, et al. Pancreatic complications in choledochal cyst and their surgical outcomes. World J Surg 2001; 25: 1519-1523. (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: 729-730.

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1 Todani T, Tabuchi K, Watanabe Y, et al. Carcinoma arising in the wall of congenital bile duct cysts. Cancer 1979; 44: 1134-1141. (CS) 2 Morine Y, Shimada M, Takamatsu H, et al. Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan-nationwide survey. J Hepatobiliary Pancreat Sci 2013; 20: 472-480. (CS) 3 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 1977; 134: 263-269. (CS) 4 Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet 1978; 146: 254-256. 5 Todani T, Watanabe Y, Toki A, et al. Carcinoma related to choledochal cysts with internal drainage operations. Surg GynecoI Obstet 1987; 164: 61-64. (CCS) 6) Strong RW. Late bile duct cancer complication biliary-enteric anastomosis for benign disease. Am J Surg 1999; 177: 472-474. (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: 939-944. (CS)

8 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg 1999; 6 : 207-212. (CS) 9 Yoshikawa 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 Gastroentero1 1986 : 81:378-384. (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: 2169-2174. (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: 317-321. (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: 482-484. (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: 148-152. (CS) 14 Kawarada Y, Das BC, Tabata M, et al. Surgical treatment of type choledochal cysts. J Hepatobiliary Pancreat Surg 2009; 16: 684-687. (CS) 15 Zheng 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: 2061–2066. (CS) 16 He 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: 819-826. (CS)

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1 Todani T, Watanabe Y, Urushihara N, et al. Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg 1995; 30: 478–481. (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: 426-430. (CS) 3 Ando 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: 491-494. (CS) 4 Todani T. Watanabe Y, Mizuguchi T. et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg 1981; 142: 584-587. (CCS) 5 Lilly JR. Total excision of choledochal cyst. Surg Gynecol Obstet 1978; 146: 254-256. 6 Todani T, Narusue M, Watanabe Y, et al. Management of congenital choledochal cyst with intrahepatic involvement. Ann Surg 1978; 187: 272-280. (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.

J Pediatr Surg 2012; 47: 2169-2174. (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: 148-152. (CS) 9 Kawarada Y, Das BC, Tabata M, et al. Surgical treatment of type Ⅳ choledochal cysts. J Hepatobiliary Pancreat Surg 2009; 16: 684-687. (CS) 10 Zheng 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, 2061-2066. (CS) 11 Koshinaga T Inoue M Ohashi K. et al. Persistent biliary dilatation and stenosis in postoperative congenital choledochal cyst. J Hepatobiliary Pancreat Sci 2011; 18: 47-52. (CS)

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Reflux/gastritis RR:0.08[ 0.02-0.39 ] (P=0.002)

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1980; 13 : 997-1007. (CS) 2 Todani T, Watanabe Y, Mizuguchi T, et al. Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst. Am J Surg 1981; 142: 584-587. (CS) 3 Takada 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) 4 Shimotakahara 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) 5 Narayanan 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: 2336–2342. (SR) 6 Santore MT Behar BJ Blinman TA et al. Hepaticoduodenostomy vs hepaticojejunostomy for reconstruction after resection of choledochal cyst. J Pediatr Surg 2011; 46: 209-213. (OS)

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. 2010 : 46:941-945. (CS) 3) Lilly JR, Weintraub WH, Altman RP. Spontaneous perforation of the extrahepatic bile ducts and bile peritonitis in infancy. Surgery 1974 ; 75 : 664-673. (CS) 4) Davies PA, Elliot-Smith A. Bile peritonitis in infancy. Arch Dis Child 1955 ; 30 : 174-176. (CS) 5) Snyder WH, Chaffin L, Oettinger L. Cholelithiasis and perforation of the gallbladder in an infant. with recovery. Am Med Assoc 1952 ; 149 : 1645-1646. (CS) 6 , , . . 1977 ; 9 : 1180-1190. (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 : 883-886. (CS) 9) . 1969 ; 32 : 945-949. (CS) 10) Todani T, Tabuchi K, Watanabe Y, et al. Perforated choledochal cyst in children. Z Kinderchir 1978 ; 23 : 280-286. (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 : 398-401. (CR) 12) , , , . .

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. 1996 ; 29 : 806-812. (CS) 13) Sugiyama M, Atomi Y. Anomalous pancreaticobiliary junction without congenital choledochal cyst. Br J Surg 1998 ; 85 : 911-916. (CS) 14) Aoki Y, Tsuchida A, Kasuya K, et al. Is preventive resection of the extrahepatic bile

duct necessary in case of pancreaticobiliary maljunction without dilatation of the bile duct? Jpn J Cli Oncol 2001 ; 31 : 107-111. (CCS) 15 , , , .

. 2010 ; 31 : 1293-1299. (CS) 16) Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. Hepatobiliary Pancreat Surg 1999 ; 6 : 207-212. (CS) 17 , , .

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. 2010 ; 31 : 1319-1323. (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 : 939-944. (CS) 20) Todani T, Watanabe Y, Toki A, et al. Carcinoma related to choledochal cysts with internal drainage operation. Surg Gynec Obstet 1987 ; 164 : 61-64. (CS)