Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000015.xml
Eur J Pediatr Surg 2009; 19(2): 63-67
DOI: 10.1055/s-0029-1215584
DOI: 10.1055/s-0029-1215584
Review
© Georg Thieme Verlag KG Stuttgart · New York
Congenital Choledochal Malformations – A European Perspective
Further Information
Publication History
received January 26, 2009
accepted after revision February 19, 2009
Publication Date:
18 June 2010 (online)
![](https://www.thieme-connect.de/media/ejps/200902/lookinside/thumbnails/10.1055-s-0029-1215584-1.jpg)
Abstract
Congenital choledochal malformations (CCM) may be a cause of abdominal pain, jaundice and sepsis in children but are easily diagnosed with modern ultrasound techniques. A review of the current approach to surgical management is presented together with an updated practical classification of the spectrum of possible anomalies.
Key words
choledochal cyst - Caroli's disease - biliary tract
References
- 1 Alonso-Lej F, Rever WB, Pessagno DJ. Congenital choledochal cyst, with a report of 2 and analysis of 94 cases. Surg Gynecol Obstret (Int Abstr Surg). 1959; 108 1-30
- 2 Aspelund G, Ling SC, Ng V, Kim PC. A role for laparoscopic approach in the treatment of biliary atresia and choledochal cyst. J Pediatr Surg. 2007; 42 869-872
- 3 Bowles BJ, Abdul-Ghani A, Zhang J, Shim WK. Fifteen years’ experience with an antirefluxing biliary drainage valve. J Pediatr Surg. 1999; 34 1711-1714
- 4 Benhidjeb T, Said S, Rudolph B, Siegmund E. Anomalous pancreatico-biliary junction – report of a new experimental model and review of the literature. J Pediatr Surg. 1996; 31 1670-1674
- 5 Caroli J, Couinaud C, Soupault R. et al . Une affection nouvelle, sans doute congénitale, des voies biliaires. La dilatation kystique unilobulaire des canaux hépatiques. Sem Hôp Paris. 1958; 34 496-502
- 6 Caroli J, Soupault J, Kossakowski J, Plocker L, Paradowska M. La dilatation polykystique congénitale des voies biliaires intrahépatique. Essai de classification. Sem Hôp Paris. 1958; 34 488-495
- 7 Charlesworth P, Ade-Ajayi N, Davenport M. Natural history and long-term follow-up of antenatally detected liver cysts. J Pediatr Surg. 2007; 42 494-499
- 8 Chijiiwa K, Tanaka M. Late complications after excisional operation in patients with choledochal cyst. J Am Coll Surg. 1994; 179 139-144
- 9 Douglas H. Case of dilatation of the common bile duct. Monthly Journal of Medical Science. 1882; 14 97-101
- 10 Dewbury KC, Aluwihare AP, Birch SJ, Freeman NV. Prenatal ultrasound demonstration of a choledochal cyst. Br J Radiol. 1980; 53 906-907
- 11 Davenport M, Caponcelli E, Livesey E, Hadzic N, Howard E. Surgical outcome in biliary atresia: etiology affects the influence of age at surgery. Ann Surg. 2008; 247 694-698
- 12 Davenport M, Basu R. Choledochal malformation manometry – under pressure. J Pediatr Surg. 2005; 40 331-335
- 13 Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation. J Pediatr Surg. 1995; 30 474-477
- 14 Farello GA, Cerofolini A, Rebonato M, Bergamaschi G, Ferrari C, Chiappetta A. Congenital choledochal cyst: video-guided laparoscopic treatment. Surg Laparosc Endosc. 1995; 5 354-358
- 15 Gibson L, Haller JA. Acute pancreatitis associated with congenital cyst of the common bile duct. J Pediatr. 1959; 55 650-657
- 16 Hata Y, Sasaki F, Takahashi H. et al . Surgical treatment of congenital biliary dilatation associated with pancreaticobiliary maljunction. Surg Gynecol Obstet. 1993; 176 581-587
- 17 Houben CH, Chiu PW, Lau J, Lee KH, Ng EK, Tam YH, Yeung CK. Preoperative endoscopic retrograde cholangigraphic treatment of complicated choledochal cysts in children: a retrospective case series. Endoscopy. 2007; 39 836-839
- 18 Hong L, Wu Y, Yan Z, Xu M, Chu J, Chen QM. Laparoscopic surgery for choledochal cyst in children: a case review of 31 patients. Eur J Pediatr Surg. 2008; 18 67-71
- 19 Imazu M, Iwai N, Tokiwa K, Shimotake T, Kimura O, Ono S. Factors of biliary carcinogenesis in choledochal cysts. Eur J Pediatr Surg. 2001; 11 24-27
- 20 Kang CM, Chi HS, Kim JY, Choi GH, Kim KS, Choi JS, Lee WJ, Kim BR. A case of robot-assisted excision of choledochal cyst, hepaticojejunostomy and extracorporeal Roux-en-Y anastomosis using the da Vinci surgical system. Surg Laparosc Endosc Percutan Tech. 2007; 17 538-541
- 21 Liu YP, Shih SL. Prenatal diagnosis of choledochal cyst by magnetic resonance cholangiography. Pediatr Radiol. 2006; 36 1112
- 22 Lilly JR, Stellin GP, Karrer FM. Forme fruste choledochal cyst. J Pediatr Surg. 1985; 20 449-451
- 23 Lilly JR. The surgical treatment of choledochal cyst. Surg Gynae Obst. 1979; 149 36-42
- 24 Liem NT, Dung LA, Son TN. Laparoscopic complete cyst excision and hepaticoduodenostomy for choledochal cyst: early results in 74 cases. J Laparoendosc Adv Surg Tech A. 2008; DOI: doi:10.1089/lap.2008.0169
- 25 Li L, Feng W, Jing-Bo F, Qi-Zhi Y, Gang L, Liu-Ming H, Yu L, Jun J, Ping W. Laparoscopic assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy. J Pediatr Surg. 2004; 39 1663-1666
- 26 Laje P, Questa H, Bailez M. Laparoscopic leak-free technique for the treatment of choledochal cysts. J Laparoendosc Adv Surg Tech A. 2007; 17 519-521
- 27 Lee H, Hirose S, Bratton B, Farmer D. Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst. J Pediatr Surg. 2004; 39 804-807
- 28 Matsufuji H, Araki Y, Nakamura A, Ohigashi S, Watanabe F. Dynamic study of pancreaticobiliary reflux using secretin-stimulated magnetic resonance cholangiopancreatography in patients with choledochal cysts. J Pediatr Surg. 2006; 41 1652-1656
- 29 Miyano T, Yamataka A, Kato Y. et al . Hepaticoenterostomy after excision of choledochal cyst in children: a 30-year experience with 180 cases. J Pediatr Surg. 1996; 31 1417-1421
- 30 Miyano T, Yamataka A, Kato Y. Choledochal cysts: special emphasis on the usefulness of intraoperative endoscopy. J Pediatr Surg. 1995; 30 482-484
- 31 Martinez-Ferro M, Esteves E, Laje P. Laparoscopic treatment of biliary atresia and choledochal cyst. Semin Pediatr Surg. 2005; 14 206-215
- 32 Matsumoto Y, Fujii H, Itakura J, Matsuda M, Yang Y, Nobukawa B, Suda K. Pancreaticobiliary maljunction: pathophysiological and clinical aspects and the impact on biliary carcinogenesis. Langenbecks Arch Surg. 2003; 388 122-131
- 33 Miyano T, Yamataka A. Choledochal cysts. Curr Opinion Pediatric. 1997; 9 283-288
- 34 MacWhorter G. Congenital cystic dilatation of the common bile duct. Arch Surg. 1924; 8 604-626
- 35 Nevalaninn TJ. The role of phospholipase A in acute pancreatitis. Scan J Gastenterol. 1980; 15 641-650
- 36 Nakamura T, Okada A, Higaki J, Tojo H, Okamoto M. Pancreaticobiliary maljunction – associated pancreatitis: an experimental study on the activation of pancreatic phospholipase A2. World J Surg. 1996; 20 543-550
- 37 Ono Y, Kaneko K, Tainaka T, Sumida W, Ando H. Pancreaticobiliary maljunction without bile duct dilatation in children: distinction from choledochal cyst. J Pediatr Gastroenterol Nutr. 2008; 46 555-560
- 38 Okada T, Itoh T, Sasaki F, Cho K, Honda S, Todo S. Comparison between prenatally diagnosed choledochal cyst and type-1 cystic biliary aresia by CD56-immunostaining using liver biopsy specimens. Eur J Pediatr Surg. 2007; 17 6-11
- 39 Ohi R, Yaoita S, Kamiyama T. et al . Surgical treatment of congenital dilatation of the bile duct with special reference to late complications after total excisional operation. J Pediatr Surg. 1990; 25 613-617
- 40 Ohi R, Koike N, Matsumoto Y. et al . Changes of intrahepatic bile duct dilatation after surgery for congenital dilatation of the bile duct. J Pediatr Surg. 1985; 20 138-142
- 41 Park DH, Kim MH, Lee SK. et al . Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts?. Gastrointest Endosc. 2005; 62 360-366
- 42 Redkar R, Davenport M, Howard ER. Antenatal diagnosis of congenital anomalies of the biliary tract. J Pediatr Surg. 1998; 33 700-704
- 43 Rao KL, Chowdhary SK, Kumar D. Choledochal cyst associated with portal hypertension. Pediatr Surg Int. 2003; 19 729-732
- 44 Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs. J Pediatr Surg. 1977; 12 39-42
- 45 Stringer MD, Dhawan A, Davenport M, Mieli-Vergani G, Mowat AP, Howard ER. Choledochal cysts: lessons from a 20 year experience. Arch Dis Child. 1995; 73 528-531
- 46 Shimotakahara A, Yamataka A, Kobayashi H, Okada Y, Yanai T, Lane GJ, Miyano T. Forme fruste choledochal cyst: long-term follow-up with special reference to surgical technique. J Pediatr Surg. 2003; 38 1833-1836
- 47 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 Int. 2005; 21 5-7
- 48 Swain WP. A case of cholecystenterostomy with the use of Murphy's button. Lancet. 1895; (i) 743-744
- 49 Shimotake T, Aoi S, Tomiyama H, Iwai N. DPC-4 (Smad-4) and K-ras gene mutations in biliary tract epithelium in children with anomalous pancreaticobiliary ductal union. J Pediatr Surg. 2003; 38 694-697
- 50 Schimpl G, Aigner R, Sorantin E, Mayr J, Sauer H. Comparison of hepaticoantrostomy and hepaticojejunostomy for biliary reconstruction after resection of a choledochal cyst. Pediatr Surg Int. 1997; 12 271-275
- 51 Sartorelli KH, Holland RM, Allshouse MJ. et al . The intussusception antireflux valve is ineffective in preventing cholangitis in biliary atresia. J Pediatr Surg. 1996; 31 403-406
- 52 Srimurthy KR, Ramesh S. Laparoscopic management of pediatric choledochal cysts in developing countries: review of ten cases. Pediatr Surg Int. 2006; 22 144-149
- 53 Takada K, Hamada Y, Watanabe K, Tanano A, Tokuhara K, Kamiyama Y. Duodenogastric reflux following biliary reconstruction after excision of choledochal cyst. Pediatr Surg Int. 2005; 21 1-4
- 54 Terui K, Yoshida H, Kouchi K, Hishiki T, Saito T, Mitsunaga T, Takenouchi A, Tsuyuguchi T, Yamaguchi T, Ohnuma N. Endoscopic sphincterotomy is a useful preoperative management for refractory pancreatitis associated with pancreaticobiliary malunion. J Pediatr Surg. 2008; 43 495-499
- 55 Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. 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
- 56 Todani T, Watanabe Y, Fujii T. Cylindrical dilatation of the choledochus: a special type of congenital bile duct dilatation. Surgery. 1985; 98 964-969
- 57 Todani T, Watanabe Y, Toki A, Morotomi Y. Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg. 2003; 10 340-344
- 58 Todani T, Watanabe Y, Urushihara N. et al . Biliary complications after excisional procedure for choledochal cyst. J Pediatr Surg. 1995; 30 478-481
- 59 Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet. 1987; 164 61-64
- 60 Ure BM, Nustede R, Becker H. Laparoscopic resection of congenital choledochal cyst, hepaticojejunostomy, and externally made Roux-en-Y anastomosis. J Pediatr Surg. 2005; 40 728-730
- 61 Ure BM, Schier F, Schmidt AI, Nustede R, Petersen C, Jesch NK. Laparoscopic resection of congenital choledochal cyst, choledochojejunostomy, and extraabdominal Roux-en-Y anastomosis. Surg Endosc. 2005; 19 1055-1057
- 62 Vater A, Ezler CS. Dissertatio de scirrhis viserum occasione sections viri tympanite defunte. Wittenburgae. 1723; 4 Pamphlers 881 22
- 63 Vades P, Pruzanski W. Role of secretory phospholipase A2 in the pathobiology of disease. Lab Invest. 1986; 55 391-404
- 64 Wong KC, Lister J. Human fetal development of the hepato-pancreatic duct junction – a possible explanation of congenital dilatation of the biliary tract. J Pediatr Surg. 1981; 16 139-145
- 65 Woo R, Le D, Albanese CT, Kim SS. Robot assisted laparoscopic resection of a type I choledochal cyst in a child. J Laparoendosc Adv Surg Tech A. 2006; 16 179-183
- 66 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after excision for choledochal cyst. J Hepatobiliary Pancreat Surg. 1999; 6 207-212
- 67 Wu GS, Zou SQ, Luo XW, Wu JH, Liu ZR. Proliferative activity of bile from congenital choledochal cyst patients. World J Gastroenterol. 2003; 9 184-187
- 68 Yamataka A, Ohshiro K, Okada Y. et al . Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults. J Pediatr Surg. 1997; 32 1097-1102
- 69 Yonem O, Bayraktar Y. Clinical characteristics of Caroli's disease. World J Gastroenterol. 2007; 13 1934-1937
- 70 Zhan JH, Hu XL, Dai CJ, Niu J, Gu JQ. Expressions of p53 and inducible nitric oxide synthase in congenital choledochal cysts. Hepatobiliary Pancreat Dis Int. 2004; 3 120-123
Correspondence
Prof. M. Davenport
King's College Hospital
Department of Paediatric Surgery
Denmark Hill
London
United Kingdom
SE5 9RS
Phone: +44/20/3299 33 50
Fax: +44/20/3299 40 21
Email: markdav2@ntlworld.com