Ductal Plate Malformation-Like Arrays in Early Explants after a Kasai Procedure Are Independent of Splenic Malformation Complex (Heterotaxy)

Abstract

Biliary atresia has at least 2 proposed forms, the common perinatal and the less common embryonic subtype with earlier onset and/or extrahepatic developmental anomalies. Histologic evidence of ductal plate malformation (DPM)–like change in liver has been proposed both as a marker for the embryonic type and as a predictor of poor outcome after Kasai portoenterostomy. We investigated the prevalence of DPM-like change in liver explants in usual biliary atresia (BA) and in BA with splenic malformation syndrome (BASM). Liver sections from 8 patients with BA and 6 with BASM, all of whom had a Kasai procedure followed by explant before age 2 years, were analyzed using hematoxylin and eosin, trichrome, CK7, and AE1/AE3 stains. Each block was scored for inflammation and fibrosis. We estimated the number of portal areas per block and counted the number of definite and possible examples of DPM-like change, defined as a circumferential duct complex arranged around a fibrovascular core. We assessed whether the frequency per portal area was related to low and high scores for either inflammation or fibrosis. Definite and possible examples of DPM-like arrays were present in about 10% of portal areas in both patient groups, but these were unevenly distributed. There was no statistical difference between BA and BASM in terms of the number of examples per portal area. No correlation existed between degree of fibrosis and the intensity of portal inflammation and the number of DPM-like arrays. Ductal plate malformation–like arrays do not distinguish perinatal BA from BA associated with heterotaxy in liver explants after a failed Kasai procedure.