-
A B C D
CQ
-
CQDelphi 70%
1 2
-
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.
-
!
!
!
-
! !
-
!
!
!
-
! !
-
!
-
!
-
!
!
MRCP ERCP
MRCP 38-100 2)4)-7)
MRCP ERCP60-100 1)-8) 82-100
1)-3)5)6) 40-80 3)4)6)7) 15 82 5) ERCP
MRCP X
1)-8)
1) , . MR Cholangiopancreatography
(MRCP) . Consensus Controversy.
1997: 58-62 EO
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)
1 MRCP
!
!
-
!
-
!
! !
-
!
-
!
!
!
1)
20% n=20 2)
1990-2007n=2,561 21.6% 3)
1 62.3% 32.1% 4.7%3)
15 9 7 28 1 4)-12)
7 1 1)
60.1±10.4 52.0±15.0 + 55.0±14.610
75 79 15 203)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:
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
. 1998; 34: 907–91.4 (CR) 9) , , , . 13
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 +
-
!
!
1990 2007 1)
0.0141%15
52.0 15.03 2)
3) 4)-6) 6)7)
3 6 8)-10)
1) , , , .
. 2010: 31: 1293-1299. (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: 2202-2205
(CS) 3) , , , . .
31 2008; 31: 48. (CS) 4) , , , . 1
. 2007; 43: 42-47. (CR) 5) , , , . . 31
-
2008; 31: 49 (CS) 6) , . . 2008; 29: 889-893. 7) , , , .
. 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 .
2004; 36: 462-465. (CS) 10) , , , . . 2006; 95: 673-677.
(EO)
!
2 C !
C !
( C)
1)2)
3)
cholangio-venous reflux4) lithostathine
Lithostathine pancreatic stone proteinLithostathine
lithostathine5) 6) Ⅴ
X MR
-
1)
1)
T1)7)
1)
2 3
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:
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.
! 1 B
! -1 B
! ( 1 B)
! D
1 10.6%21.6% 62.3% 32.1%
-
2
3 4
4 5
6
7 8
9 10
11)( 2)
11
12)
13-16) -A
15)
16
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)
! 1 C
!
2 C
!
C
-
1
80% 2
3
4 5
6
Historical control11.8 0
8-10
-Anon-RCT
10
11
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)
! Roux-en-Y
B
! Roux-en-Y
B
Roux-en-Ysimple
1 2
Roux-en-Y3 4)
6 meta-analysis outcome Bile leak Cholangitis
Anastomotic stricture Reflux/gastritis Adhesive intestinal
obstruction Reoperation rates Operative time Hospital stay Bile
leak RR:1.50[ 0.51-4.36 ](P=0.46) Cholangitis RR:1.07[ 0.41-2.81 ]
(P=0.89) Anastomotic stricture RR:1.45[ 0.36-5.79 ] (P=0.60)
Reflux/gastritis RR:0.08[ 0.02-0.39 ] (P=0.002)
-
Adhesive intestinal obstructionRR:2.77[ 0.27-27.92 ](P=0.39)
Reoperation ratesRR:2.14[ 0.67-6.89 ] (P=0.20) Operative time
Hospital stay MD:0.30[ 0.22-0.39 ] (P=0.29)5
5 6
Roux-en-YRoux-en-Y
1
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)
! 2 C
< >
1 ,
,2
, 3), 4 ,
-
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 :
23-25 (CS) 2 , . 7
. 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) , , , . .
2006 ; 42 : 497-501. (CR)
-
! , ,
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 : 478-481. (CS) 2) Chijiiwa K, Tanaka M.
Late complications after excisional operation in patients with
choledochal cyst. J Am College Surg 1994 ; 179 : 139-144. (CS) 3)
Saing H, Han H, Chan KL, et al. Early and late results of excision
of choledochal cysts. J Pediatr Surg 1999 ; 32 : 1563-1566. (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 : 165-167. (CS) 5) , , , . .
. 2010 ; 31 : 1319-1323. (CS) 6) Watanabe Y, Toki A, Todani T.
Bile duct cancer developed after cyst excision for
-
choledochal cyst. Hepatobiliary Pancreat Surg 1999 ; 6 :
207-212. (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 : 939-944.
(CS) 8) Strong RW. Late bile duct cancer complication
biliary-enteric anastomosis for benign disease. Am J Surg 1999 ;
177 : 472-474. (CS)
! 0.7~5.4%C
< >
l ,
, , ,
2 3,4
, , 5~8) , ,
9~11) , 12~14) , ,
,
1990 2007 18
997 79 7 . 9% 15
, , ,
, Watanabe 16)
0.7%, 17) 145 2 (1.4%), 18) 65 2 3.1%Kobayashi 19) 56 3 5.4%
, Todani 20) 10 3 2,
-
15,
,
1) .
l . . 2007 : 23-31. (CPG) 2 .
. 1992 ; 53 : 2631-2637. (CS) 3 ,
2008 ; 29 : 921-925. (CS) 4) Todani T, Watanabe Y, Urushihara N,
et al. Biliary complications after excisional procedure for
choledochal cyst. J Pediatr Surg 1995 ; 30 : 478-481. (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 : 1183-1187. (CS) 6 . 2004 ; 36 :
495-500.(EO) 7 , , , . 8
1 . 2007 ; 28 : 149-153. (CR) 8 , . 40
l . 2010 ; 31 : 329-332. (CS) 9 .
2001 ; 22 : 493-498. (CS) 10 , .
2001 ; 22 : 499-503. (CS) 11) Hara H, Morita S, Ishibashi T, et
al. Surgical treatment for non-dilated biliary tract with
pancreatico biliary maljunction should include excision of the
extrahepatic bile duct. Hepatogastroenterology 2011 ; 48 : 984-987.
(CS) 12 , , .
. 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 , ,
.
2010 ; 31 : 1301-1306. (CS) 18 , , ,
. 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)