Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Abstract

Purpose: Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis.

Methods: A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason.

Results: We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23-12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83-236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70-83.93, p = 0.002, respectively).

Conclusion: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

Keywords: Alagille syndrome; Biliary atresia; Kasai operation; Meta-analysis; Neonatal cholestasis.