Short-term outcomes and prognostic factors of cholecystojejunocolonic anastomosis for PFIC and Alagille syndrome

Abstract

Objective To observe the short-term outcomes of cholecystojejunocolonic anastomo sis for patients with progressive familiar intrahepatic cholestasis (PFIC) or Alagille syndrome and to determine the possible prognostic factors.Methods Cholecystojejunocolonic anastomosis was performed on 11 patients with PFIC and 2 patients with Alagille syndrome from August 2010 to August 2012.Total bile acids (TBA) and total bilirubin (TB) were measured preoperatively,1 week,1,6 and 12 months postoperatively.Pre-and postoperative TBA,TB,pruritus scale and height Z value were compared.Results TBA decreased by ＞50％ 1 month after operation in 5 patients (group A).In group A,TBA decreased to (27.0 ± 6.4) ％,(24.4 ± 10.2) ％ and (40.4 ± 17.2) ％ and TB decreased to (51.6 ± 31.9) ％,(37.8 ± 36.5) ％ and (49.6 ± 21.5) ％ of the pre-operative concentrations at 1,6 and 12 months after operation.Pruritus scale decreased by (2.4 ± 1.3) and (1.5 ± 1.2) points at 6 and 12 months after operation.Height Z increased in 3 patients.In the rest 8 patients (group B),TBA,TB,pruritus scale and height Z did not improve after operation.Univariate analysis showed age,sex,pre-operative TB and TBA had no differences in group A and B,but only 1 patient in group A had persistent pre-operative jaundice which was significantly different from group B.Conclusions PIBD can relieve pruritus and jaundice for some patients with PIFC/AGS.Persistent pre-operative jaundice and a decrease of ＜50％ 1 month after operation are unfavorable prognostic factors. Key words: Cholestasis, intrahepatic; Alagille syndrome; Anastomosis, surgical; Prognosis