Plain language summary
Glucocorticosteroids administered after Kasai surgical procedure for infants with blocked or damaged bile duct
Medications used postoperatively (immediately after surgery) for infants with blocked or damaged bile duct (that is, biliary atresia)
Do medications, called glucocorticosteroids (steroids), have beneficial or harmful effects in the health of infants with biliary atresia operated by the Kasai surgical procedure (that is, portoenterostomy)? We reviewed if there was any difference in death, need for a liver transplant, postoperative jaundice (yellowish or greenish pigmentation of the skin and whites of the eyes), and harmful effects.
Biliary atresia is a rare condition that may occur once in 30,000 births. In biliary atresia, the common bile duct is blocked or damaged; as the bile cannot leave the liver, the liver becomes damaged. An operation called 'Kasai portoenterostomy' is used to replace the damaged bile ducts with a piece of the infant's intestine. This allows the bile to drain directly from the small bile ducts at the edge of the infant's liver, straight into the intestine. Medications called glucocorticosteroids have historically been used in the treatment of biliary atresia after surgery. Two benefits of glucocorticosteroids may be that they are anti-inflammatory, and they increase bile flow. Several studies have been carried out comparing infants taking glucocorticosteroids postoperatively to those who have been given a placebo (an inactive substance that can be made to resemble an active medication or therapy). These studies try to identify if there is any measurable difference in the clearance of jaundice, survival, and need for transplantation. To organise randomised clinical trials large enough to be able to detect differences is, however, challenging.
We performed a search which included studies up to 20 December 2017. We identified two randomised clinical trials (where participants are divided by chance into the trial groups) which met the requirements for our review and followed-up the participants for at least two years. We identified 19 further observational studies from which we were able to report some findings on harms in a narrative form. The randomised trials included 107 infants who were given glucocorticosteroids and 104 who were given placebo. Trials were funded by charities, public organisations, and received support from private sector companies, all of which did not seem to have any interest in the outcome of the respective trials.
The included trials outlined their sources of funding, and the review authors deemed that there were no conflicts of interest. Review authors did not receive funding to carry out this review.
We did not find any differences between the groups of infants treated with glucocorticosteroids compared with placebo in terms of mortality, adverse events, ability to clear jaundice, or need for a liver transplant.
Quality of the evidence
We assessed the two trials as having low risk of bias (we had no concerns that their design and reporting may deviate from the truth), but they were at high risk of imprecision (inexact evaluations of outcomes). They used different categories for adverse events, and we were unable to combine the data from the trials. We could not include enough infants in our analyses (only two published trials) in order to detect small differences between the two intervention groups. The certainty of the evidence was low for mortality, adverse events, ability to clear jaundice, or need for a liver transplant outcomes. One further ongoing trial was identified, with no currently available results.
We need further randomised clinical trials that compare glucocorticosteroids with placebo in order to find out if glucocorticosteroids are of benefit in the postoperative management of infants with biliary atresia. Such trials need to be conducted at different clinical centres.