Abstract
Purpose: Advances in surgical techniques and perioperative care have improved patients' short-and mid-term postoperative outcomes with Biliary Atresia (BA). However, the long-term results of these patients have not been thoroughly investigated. This systematic review aims to determine the long-term outcomes and the patients' health-related Quality of life (HrQoL) with their native livers or liver transplantation. Methods: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to August 2021. Original studies reporting on BA, Hepatoportoenterostomie, portoenterostomy, Kasai, Liver transplantation, Quality of life, or HrQoL were included. Pooled prevalence has been calculated for cholangitis, secondary liver transplantation, or associated malformations using MetaXL (version 5.3). Subgroup analysis on HrQoL followed surgical treatment after BA was calculated by using RevMan (version 5.4).Results: 12 articles were considered for data synthesis. Nine studies compared biliary atresia patients to an age-matched healthy reference group. 4/9 (n = 338) of these studies indicated lower scores for biliary atresia patients; 5/9 (n = 127) stated similar health status. A Forest plot analysis including all studies with total HrQoL showed a tendency of higher scores towards healthy controls (MD -0.79, 95% CI: -6.00-4.41). Comparing patients after Kasai Hepatoportoenterostomy with healthy controls demonstrated favorable outcomes for the control group (MD -3.22, 95% CI: -7.20-0.75) with no statistical significance (p = 0.11). The pooled estimation of the prevalence of cholangitis, secondary liver transplantation and associated malformations are 0.33 (95% CI: 0.06–0.66), 0.59 (95% CI: 0,42–0.75) and 0.13 (95% CI: 0,01–0.33).Conclusion: Biliary atresia patients have an overall high prevalence of progressive liver-related complications and risk of lower HrQoL compared to their healthy peers. Furthermore, those patients who received liver transplantation appear to have the same Quality of life as those living with their native livers. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research das focuses on the attributes and predictors of the long-term prognosis of patients with biliary atresia are necessary.
Long-term results of splenomegaly after surgery for biliary atresia in the native liver
