Download PDF
Eur J Pediatr Surg 2009; 19(2): 63-67
DOI: 10.1055/s-0029-1215584
Review

© Georg Thieme Verlag KG Stuttgart · New York

Congenital Choledochal Malformations – A European Perspective

M. Davenport1 , C. K. Sinha1
  • 1Department of Paediatric Surgery, King's College Hospital, London, United Kingdom
Further Information

Publication History

received January 26, 2009

accepted after revision February 19, 2009

Publication Date:
18 June 2010 (online)

Also available at
    Permissions and Reprints

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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 8 Chijiiwa K, Tanaka M. Late complications after excisional operation in patients with choledochal cyst.  J Am Coll Surg. 1994;  179 139-144
    Google Scholar
  • 9 Douglas H. Case of dilatation of the common bile duct.  Monthly Journal of Medical Science. 1882;  14 97-101
    Google Scholar
  • 10 Dewbury KC, Aluwihare AP, Birch SJ, Freeman NV. Prenatal ultrasound demonstration of a choledochal cyst.  Br J Radiol. 1980;  53 906-907
    Google Scholar
  • 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
    Google Scholar
  • 12 Davenport M, Basu R. Choledochal malformation manometry – under pressure.  J Pediatr Surg. 2005;  40 331-335
    Google Scholar
  • 13 Davenport M, Stringer MD, Howard ER. Biliary amylase and congenital choledochal dilatation.  J Pediatr Surg. 1995;  30 474-477
    Google Scholar
  • 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
    Google Scholar
  • 15 Gibson L, Haller JA. Acute pancreatitis associated with congenital cyst of the common bile duct.  J Pediatr. 1959;  55 650-657
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 21 Liu YP, Shih SL. Prenatal diagnosis of choledochal cyst by magnetic resonance cholangiography.  Pediatr Radiol. 2006;  36 1112
    Google Scholar
  • 22 Lilly JR, Stellin GP, Karrer FM. Forme fruste choledochal cyst.  J Pediatr Surg. 1985;  20 449-451
    Google Scholar
  • 23 Lilly JR. The surgical treatment of choledochal cyst.  Surg Gynae Obst. 1979;  149 36-42
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 30 Miyano T, Yamataka A, Kato Y. Choledochal cysts: special emphasis on the usefulness of intraoperative endoscopy.  J Pediatr Surg. 1995;  30 482-484
    Google Scholar
  • 31 Martinez-Ferro M, Esteves E, Laje P. Laparoscopic treatment of biliary atresia and choledochal cyst.  Semin Pediatr Surg. 2005;  14 206-215
    Google Scholar
  • 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
    Google Scholar
  • 33 Miyano T, Yamataka A. Choledochal cysts.  Curr Opinion Pediatric. 1997;  9 283-288
    Google Scholar
  • 34 MacWhorter G. Congenital cystic dilatation of the common bile duct.  Arch Surg. 1924;  8 604-626
    Google Scholar
  • 35 Nevalaninn TJ. The role of phospholipase A in acute pancreatitis.  Scan J Gastenterol. 1980;  15 641-650
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 42 Redkar R, Davenport M, Howard ER. Antenatal diagnosis of congenital anomalies of the biliary tract.  J Pediatr Surg. 1998;  33 700-704
    Google Scholar
  • 43 Rao KL, Chowdhary SK, Kumar D. Choledochal cyst associated with portal hypertension.  Pediatr Surg Int. 2003;  19 729-732
    Google Scholar
  • 44 Spitz L. Experimental production of cystic dilatation of the common bile duct in neonatal lambs.  J Pediatr Surg. 1977;  12 39-42
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 48 Swain WP. A case of cholecystenterostomy with the use of Murphy's button.  Lancet. 1895;  (i) 743-744
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 58 Todani T, Watanabe Y, Urushihara N. et al . Biliary complications after excisional procedure for choledochal cyst.  J Pediatr Surg. 1995;  30 478-481
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 62 Vater A, Ezler CS. Dissertatio de scirrhis viserum occasione sections viri tympanite defunte.  Wittenburgae. 1723;  4 Pamphlers 881 22
    Google Scholar
  • 63 Vades P, Pruzanski W. Role of secretory phospholipase A2 in the pathobiology of disease.  Lab Invest. 1986;  55 391-404
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 66 Watanabe Y, Toki A, Todani T. Bile duct cancer developed after excision for choledochal cyst.  J Hepatobiliary Pancreat Surg. 1999;  6 207-212
    Google Scholar
  • 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
    Google Scholar
  • 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
    Google Scholar
  • 69 Yonem O, Bayraktar Y. Clinical characteristics of Caroli's disease.  World J Gastroenterol. 2007;  13 1934-1937
    Google Scholar
  • 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
    Google Scholar

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