Early and Peri-operative Prognostic Indicators in Infants Undergoing Hepatic Portoenterostomy for Biliary Atresia: a Review
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Purpose of Review
Biliary atresia is the most common indication for liver transplantation among children. In recent years, prospective, multi-centre collaboration has been underway with the aim of providing high-quality data on the natural history of the condition, prior to and following hepatic portoenterostomy.
There is increasing evidence that specific histological findings, and age, at the time of portoenterostomy have relevance as prognostic indicators. Recent data suggest that the sub-type of biliary atresia, its co-existence with other anomalies and concurrent infection may also be important variables.
This review provides a comprehensive summary of the histological predictors of outcome following portoenterostomy. Later age at portoenterostomy, advanced degree of hepatic fibrosis and co-existence with other congenital anomalies are strongly correlated with poor outcomes following portoenterostomy. There is increasing evidence to suggest that common serological indices and the presence or absence of cytomegalovirus (CMV) co-infection may have utility as early prognostic indicators.
KeywordsAtresia Biliary Histology Kasai Outcome Predictors
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
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- 2.• Jimenez-Rivera C, Jolin-Dahel KS, Fortinsky KJ, Gozdyra P, Benchimol EI. International incidence and outcomes of biliary atresia. J Pediatr Gastroenterol Nutr. 2013;56(4):344–54. Provides global epidemiological data on the course and outcomes in infants with biliary atresia.CrossRefPubMedGoogle Scholar
- 3.Kasai M, Sawaguchi S, Akiyama H. A proposal of new classification of extrahepatic biliary atresia. J Jpn Soc Pediatr Surg. 1976;12:327–31.Google Scholar
- 4.• Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, et al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011;254(4):577–85. A multi-centre, prospective study with large numbers which showed that the sub-type of biliary atresia and association with other congenital anomalies were important prognostic variables.CrossRefPubMedPubMedCentralGoogle Scholar
- 5.Nightingale S, Stormon MO, O’Loughlin EV, Shun A, Thomas G, Benchimol EI, et al. Early Post-Hepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia. J Pediatr Gastroenterol Nutr. 2016.Google Scholar
- 7.Kasai M, Suzuki SA. A new operation for "non-correctable" biliary atresia—portoenterostomy. Shijitsu. 1959;13:733–9.Google Scholar
- 8.Kasai M. Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modification. Baltimore: University Park Press; 1974.Google Scholar
- 17.Webb NL, Jiwane A, Ooi CY, Nightingale S, Adams SE, Krishnan U. Clinical significance of liver histology on outcomes in biliary atresia. J Paediatr Child Health. 2016. doi: 10.1111/jpc.13371
- 31.• Qiao G, Li L, Cheng W, Zhang Z, Ge J, Wang C. Conditional probability of survival in patients with biliary atresia after Kasai portoenterostomy: a Chinese population-based study. J Pediatr Surg. 2015;50(8):1310–5. A study with large numbers which showed that age at Kasai portoenterostomy of greater than 90 days was associated with poor native liver survival.CrossRefPubMedGoogle Scholar
- 47.Trivedi P, Cheeseman P, Portmann B, Mowat AP. Serum type III procollagen peptide as a non-invasive marker of liver damage during infancy and childhood in extrahepatic biliary atresia, idiopathic hepatitis of infancy and α1 antitrypsin deficiency. Clin Chim Acta. 1986;161:137–46.CrossRefPubMedGoogle Scholar