Further Observations on Cystic Dilatation of the Intrahepatic Biliary System in Biliary
Atresia after Hepatic Portoenterostomy: Report on 10 Cases
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HIDEO KAWARASAKI1,
MITSUHIRO ITOH1,
KOICHI MIZUTA1,
HIDEAKI TANAKA1 and
MASATOSHI MAKUUCHI2
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1Department of Pediatric Surgery and 2The Second Department of
Surgery, Faculty of Medicine, The University of Tokyo, Tokyo 113
This is a report on ten patients with cystic dilatation of the intrahepatic biliary system (CDIB)
after hepatic portoenterostomy. They were five girls and five boys and the diagnosis of CDIB
was made at ages 6 months to 11 years (mean age: 2.8+-±3.3 years). Follow-up ranged from
one month to 15 years (mean: 5.5+-±4.9 years). In order to elucidate the factors which affect
the clinical outcome of such patients, the types of CDIB (Type A: non-communicating
solitary cyst, Type B : communicating solitary cyst, Type C: multi-cystic dilatation), clinical
symptoms at onset of CDIB and the method for the treatment were reviewed in relation to the
outcome. For the purpose of understanding pathogenesis of CDIB, immunohistochemical
study on hepatobiliary system was done with monoclonal antibody for cytokeratin. Outcome
of the patients of Type C was poor, whereas the outcome of patients with type A and B was
good. The outcome of preoperatively jaundiced patients was poor, but jaundice-free patients
showed good outcome. Method of treatment was not related to the outcome. As epithelium of
CDIB was positive for monoclonal antibody of cytokeratin, it was suspected that
pathogenesis of CDIB might be related to peribiliary gland which originated from ductal plate.
Key word(s)---
biliary atresia; dilatation of the intrahepatic bile ducts; Kasai's operation
Tohoku J. Exp. Med., 1997, 181, 175-183
Address for reprints:
Hideo Kawarasaki, M.D., Ph. D., Department of Pediatric Surgery, Faculty of Medicine, The
University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113, Japan.
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