Eur J Pediatr Surg 2010; 20(2): 128-130
DOI: 10.1055/s-0029-1224193
Case Gallery

© Georg Thieme Verlag KG Stuttgart · New York

The Evolution of Biliary Atresia in Early Life

A. Toubi 1 , I. Sukhotnik 2 , J. Bejar 3 , J. Mogilner 4 , R. Shaoul 5
  • 1Bnai Zion Medical Center, Radiology, Haifa, Israel
  • 2Bnai Zion Medical Center, Pediatric Surgery, Haifa, Israel
  • 3Bnai Zion Medical Center, Pathology, Haifa, Israel
  • 4Bnai Zion Medical Center, Department of Pediatric Surgery, Haifa, Israel
  • 5Bnai Zion Medical Center, Pediatrics, Haifa, Israel
Further Information

Publication History

Publication Date:
22 June 2009 (online)

It is well known that biliary atresia (BA) is a progressive process, leading to fibrosis of the bilary tree and the eventual requirement of a hepatoportoenterostomy. The etiology and timing of the onset of this condition remains an enigma. We present the case of an infant admitted to hospital at the age of 11 days with cholestatic jaundice and subsequently diagnosed with biliary atresia. We tracked his fetal ultrasound (US) image which showed a normal gallbladder at the gestational age of 24 weeks. The gallbladder was absent on US when he presented with jaundice. No other liver abnormalities were noted. During the short follow-up, fibrosis around the intrahepatic bile ducts was noted. We therefore conclude that the atretic process started either late in pregnancy or during the perinatal period. We believe that this case report adds to our understanding of the timing of this condition.

References

  • 1 Sokol RJ, Mack C, Narkewicz MR. et al . Pathogenesis and outcome of biliary atresia: current concepts.  J Pediatr Gastroenterol Nutr. 2003;  37 4-21
  • 2 Schreiber RA, Barker CC, Roberts EA. et al . Biliary atresia: the Canadian experience.  J Pediatr. 2007;  151 659-665
  • 3 Haber BA, Russo P. Biliary atresia.  Gastroenterol Clin North Am. 2003;  32 891-911
  • 4 Petersen C. Pathogenesis and treatment opportunities for biliary atresia.  Clin Liver Dis. 2006;  10 73-88 vi
  • 5 Schreiber RA, Kleinman RE. Biliary atresia.  J Pediatr Gastroenterol Nutr. 2002;  35 (Suppl 1) S11-S16
  • 6 Davenport M, Savage M, Mowat AP. et al . Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup.  Surgery. 1993;  113 662-668
  • 7 Mack CL, Sokol RJ. Unraveling the pathogenesis and etiology of biliary atresia.  Pediatr Res. 2005;  57 (5 Pt 2) 87R-94R
  • 8 Landing BH. Considerations of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst--the concept of infantile obstructive cholangiopathy.  Prog Pediatr Surg. 1974;  6 113-139
  • 9 Ohi R, Chiba T, Endo N. Morphologic studies of the liver and bile ducts in biliary atresia.  Acta Paediatr Jpn. 1987;  29 584-589
  • 10 Schreiber RA, Kleinman RE, Barksdale  Jr  EM. et al . Rejection of murine congenic bile ducts: a model for immune-mediated bile duct disease.  Gastroenterology. 1992;  102 924-930
  • 11 Hinds R, Davenport M, Mieli-Vergani G. et al . Antenatal presentation of biliary atresia.  J Pediatr. 2004;  144 43-46
  • 12 Tsuchida Y, Kawarasaki H, Iwanaka T. et al . Antenatal diagnosis of biliary atresia (type I cyst) at 19 weeks’ gestation: differential diagnosis and etiologic implications.  J Pediatr Surg. 1995;  30 697-699

Correspondence

Dr. MD. R. Shaoul

Bnai Zion Medical Center Pediatrics

47 Golomb st

31048 Haifa

Israel

Phone: +97/24/83 59 662

Fax: +97/24/83/59 724

Email: shaoul_r@012.net.il