Manganese deposits in patients with biliary atresia after hepatic porto-enterostomy

Purpose: The aim of this study was to determine if there is latent manganese toxicity in patients with biliary atresia. Methods: Fifteen children with biliary atresia were examined postoperatively with regard to whole-blood manganese levels using brain magnetic resonance imaging (MRI) and I-123 iodoamphetamine (IMP) per rectal portal scintigraphy. Results: Nine (60%) of the 15 had high whole-blood manganese levels (mean, 4.1 mu g/dL; range, 1.2 to 9.6; normal, 0.5 to 2.5), and these 9 had hyperintense globus pallidus on T1-weighted images, with no corresponding signal change in T2 sequences, I-123 IMP per rectal portal scintigraphy was done for 13 patients to evaluate portosystemic shunt flow. 12 (92%) of these patients had an increased flow. Mean shunt ratio was estimated to be 41% (range, 0.6 to 98; normal, (<5%). Encephalopathy was evident in only 1 patient. Conclusions: Some patients with biliary atresia in the postoperative period have manganese deposits in globus pallidus on T1-weighted images and high whole-blood manganese levels, possibly caused by increased portsystemic shunt, and a latent or subclinical encephalopathy is also present. Copyright (C) 2000 by W.B. Saunders Company.