Neonatology pp 659-663 | Cite as

Surgical Treatment of Biliary Tract Malformations

  • George Ekema
  • Pierluigi Pedersini


Biliary atresia (BA) is a progressive necroinflammatory process involving the extrahepatic biliary tree, either partially or completely. The disease shows the onset during the first days of life and usually evolves into obliteration of the extrahepatic bile duct lumen with interruption of bile flow, resulting in cholestasis and chronic liver damage. If untreated, the disease leads to exitus within the first 2 years of life. Early diagnosis is mandatory to plan a timely surgery, preferably within 80 days of life.


Bile Duct Common Bile Duct Biliary Atresia Choledochal Cyst Magnetic Resonance Cholangiography 


  1. 1.
    Caccia G, Ekema G, Falchetti D, Pedersini P (2004) Atresia delle vie biliari: attualità e prospettive. Prosp Pediatr 34: 39–43Google Scholar
  2. 2.
    Petersen C, Ure BM (2003) What’s new in biliary atresia? Eur J Pediatr Surg 13: 1–6PubMedCrossRefGoogle Scholar
  3. 3.
    Chardot C (2006) Biliary atresia. Orphanet J Rare Dis 1: 28PubMedCrossRefGoogle Scholar
  4. 4.
    Davenport M (2005) Biliary atresia. Semin Pediatr Surg 14: 42–48PubMedCrossRefGoogle Scholar
  5. 5.
    Sokol RJ, Mack C, Narkewicz MR, Karrer FM (2003) Pathogenesis and outcome of biliary atresia: currents concepts. J Pediatr Gastroenterol Nutr 37: 4–21PubMedCrossRefGoogle Scholar
  6. 6.
    Haber BA, Russo P (2003) Biliary atresia. Gastroenterol Clin North Am 32: 891–911PubMedCrossRefGoogle Scholar
  7. 7.
    Mack CL, Sokol RJ (2005) Unraveling the pathogenesis and etiology of biliary atresia. Pediatr Res 57: 87R–94RPubMedCrossRefGoogle Scholar
  8. 8.
    Park WH, Choi SO, Lee HJ (2001) Technical innovation for noninvasive and early diagnosis of biliary atresia: the ultrasonographic “triangular cord” sign. J Hepatobiliary Pancreat Surg 8: 337–341PubMedCrossRefGoogle Scholar
  9. 9.
    Tan Kendrick AP, Phua KB, Ooi BC, Tan CE (2003) Biliary atresia: making the diagnosis by the gallbladder ghost triad. Pediatr Radiol 33: 311–315CrossRefGoogle Scholar
  10. 10.
    Linuma Y, Narisawa R, Iwaguchi M et al (2000) The role of endoscopic retrograde cholangiopancreatography in infants with cholestasis. J Pediatr Surg 35: 545–549CrossRefGoogle Scholar
  11. 11.
    Han SJ, Kim MJ, Han A et al (2002) Magnetic resonance cholangiography for the diagnosis of biliary atresia. J Pediatr Surg 37: 599–604PubMedCrossRefGoogle Scholar
  12. 12.
    Haber BA, Russo P (2003) Biliary atresia. Gastroenterol Clin N Am 32: 891–911CrossRefGoogle Scholar
  13. 13.
    Estevez E, Neto EC, Neto MO et al (2002) Laparoscopic Kasai portoenterostomy for biliary atresia. Pediatr Surg Int 28: 737–740Google Scholar
  14. 14.
    De Carvalho E, Pontes Ivantes CA, Bezerra J (2007) Extrahepatic biliary atresia: current concepts and future directions. J Pediatr 83: 105–120Google Scholar
  15. 15.
    Iwasaki J, Yoshifumi O, Shunichi N et al (2008) Familial recurrence of congenital bile duct dilatation. World J Gastroenterol 14: 941–943PubMedCrossRefGoogle Scholar
  16. 16.
    Benjamin IS (2003) Biliary cystic disease: the risk of cancer. J Hepatobiliary Pancreat Surg 10: 335–339PubMedCrossRefGoogle Scholar
  17. 17.
    Takaya J, Muneyuki M, Tokuhara D et al (2003) Congenital dilatation of the bile duct: changes in diagnostic tools over the past 19 years. Pediatr Int 45: 383–387PubMedCrossRefGoogle Scholar
  18. 18.
    Fitoz S, Erden A, Boruban S (2007) Magnetic resonance cholangiopancreatography of biliary system abnormalities in children. Clin Imag 31: 93–101CrossRefGoogle Scholar
  19. 19.
    Li L, Feng W, Jing-Bo F et al (2004) Laparoscopic-assisted total cyst excision of choledochal cyst and Roux-en-Y hepatoenterostomy. J Pediatr Surg 39: 1663–1666PubMedCrossRefGoogle Scholar
  20. 20.
    Aspelung G, Ling SC, Ng V, Kim PCW (2007) A role for laparoscopic approach in the treatment of biliary atresia and choledochal cysts. J Pediatr Surg 42: 869–872CrossRefGoogle Scholar
  21. 21.
    Berg C, Baschat AA, Geipel A et al (2002) First-trimester diagnosis of fetal hepatic cyst. Ultrasound Obstet Gynecol 19: 287–289PubMedCrossRefGoogle Scholar
  22. 22.
    Rogers TN, Woodley H, Ramsden W et al (2007) Solitary liver cysts in children: not always so simple. J Pediatr Surg 42: 333–339PubMedCrossRefGoogle Scholar
  23. 23.
    Howard ER (2002) Cysts. In: Howard ER, Stringer MD, Colombani PM (eds) Surgery of the liver, bile ducts and pancreas in children, 2nd edn. Oxford University Press, London, p 239Google Scholar
  24. 24.
    Charlesworth P, Ade-Ajayi N, Davenport M (2007) Natural history and long term follow-up of antenatally detected liver cysts. J Pediatr Surg 42: 494–499PubMedCrossRefGoogle Scholar
  25. 25.
    Shankar SR, Parelkar SV, Das SA et al (2000) An antenatally diagnosed solitary, nonparasitic hepatic cyst with duodenal obstruction. Pediatr Surg Int 16: 214–215PubMedCrossRefGoogle Scholar
  26. 26.
    Soyer T, Karnak I, Senokar ME (2007) Congenital solitary intrahepatic biliary cist in a newborn: report of a case. Surg Today 37: 521–524PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2012

Authors and Affiliations

  • George Ekema
  • Pierluigi Pedersini
    • 1
  1. 1.Pediatric Surgery UnitUniversity of BresciaBresciaItaly

Personalised recommendations