I. N. Lobeck1, I. P. Lim2, R. Karns3, J. Bezerra3, G. Tiao2 1Wayne State University,General Surgery,Detroit, MI, USA 2Cincinnati Children’s Hospital Medical Center,General And Thoracic Pediatric Surgery,Cincinnati, OH, USA 3Cincinnati Children’s Hospital Medical Center,Division Of Gastroenterology, Hepatology, And Nutrition,Cincinnati, OH, USA
Introduction: Biliary atresia (BA) is a cholangiopathy of infancy which, without intervention, progresses to death in the first two years of life. We report our institutional experience of the pathophysiology and natural history of BA without portoenterostomy.
Methods: After Institutional Review Board approval, a retrospective chart review of patients who underwent primary liver transplant for BA without portoenterostomy during 2003-2015 was performed. Data collected included demographics, laboratory and clinical history at diagnosis, listing and transplantation. Statistical analysis was performed utilizing pairwise correlation analyses, time-series regression modeling and association testing between clinical variables and surgical outcomes.
Results:Sixteen patients were identified (63% male; mean age 165±68 days at diagnosis and 362±223 days at transplant). At presentation, symptoms were predominantly of cholestasis and mild synthetic dysfunction, with normal platelet counts (mean direct bilirubin 8.1mg/dL, INR 1.3, platelets 256,000). At transplantation, direct bilirubin rose to 34 fold and INR 2 fold over normal; mean platelet count decreased to 128,000. 45% (n=5) endured variceal bleeding. Patients with bleeding episodes before transplant tended to have more postoperative complications (p=0.08, R2=0.45). The strongest predictors of postoperative course were platelets, change in INR between diagnosis and transplant, and bilirubin. Each increase of direct bilirubin by 1 unit resulted in one day increase of ICU stay (p=0.002). INR also predicted EBL, with each increase of 1 unit resulting in additional 838ml of blood loss (p=0.022). Platelet count showed a borderline association with postoperative outcomes, with each increase of 50,000 units in platelets linked to decreasing the risk of infection one year postoperatively by half (p=0.058).
Conclusion: At presentation, patients with uncorrected BA have cholestasis and mild synthetic dysfunction. Disease progression entails increasing cholestasis, portal hypertension, and synthetic dysfunction. The greatest predictors of post-transplant outcomes include bilirubin, platelets and INR. Early liver transplantation is warranted once the opportunity for portoenterostomy is missed.