The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup "o" biliary atresia.

PURPOSE: The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA. METHODS: Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT. RESULTS: All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup "o", in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct. CONCLUSIONS: Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.

The incidence of chylous ascites after liver transplantation and the proposal of a diagnostic and management protocol.

BACKGROUND: No protocol has been established for the diagnosis and management of chylous ascites after liver transplantation (LT). In this study, we retrospectively reviewed our cases of posttransplant chylous ascites (PTCA) and aimed to propose a diagnostic and management protocol. PATIENTS AND METHODS: We retrospectively reviewed the clinical records of 96 LT recipients who underwent LT at our department. The incidence of PTCA and the associated risk factors were analyzed and our protocol for chylous ascites was evaluated. RESULTS: PTCA occurred in 6 (6.3%) patients (mean age: 10.7±11.0years) at a mean of 10.8±3.6days after LT. The primary disease in all of PTCA cases was biliary atresia (BA). The periportal lymphadnopathy was an independent risk factor for PTCA. In all cases PTCA successfully resolved according to our protocol. Octreotide was administered in 4 of our 6 PTCA cases. The mean postoperative hospital stay was 40.2±8.4days, which was similar to that of cases without PTCA. CONCLUSIONS: The incidence of PTCA in LT patients, especially in those with BA, is relatively high. Our diagnostic criteria and our management protocol were helpful for patients with refractory ascites after LT. TYPE OF STUDY: Diagnostic test: Level II. Treatment study: Level III.