Intrahepatic cystic lesions after hepatic portoenterostomy for biliary atresia with bile lake and dilated bile ducts.
BACKGROUND
Intrahepatic cystic lesions develop after hepatic portoenterostomy in some patients with biliary atresia and have been regarded as an indication of poor prognosis; however, there is confusion about the terminology and concepts of the lesions. We investigated whether the cystic lesions constituted a single entity.


METHODS
From 1980 to 2005, we encountered 80 patients with biliary atresia. Abdominal ultrasonography disclosed intrahepatic cystic lesions in 20 (25%) patients after hepatic portoenterostomy. The cystic lesions were analyzed morphologically with radiologic imaging studies and histologically in 13 patients who underwent liver transplantation.


RESULTS
Fifteen patients had solitary simple cystic lesions, and 5 patients had multiple continuous oval or beaded lesions. Solitary lesions had a fibrotic cyst wall and lacked epithelia. Continuous lesions had a cyst wall covered with biliary epithelia. Thirteen patients with solitary cysts died or required liver transplantation. In 2 patients with continuous lesions, surgical reboring of the porta hepatis could eliminate cystic lesions and jaundice.


CONCLUSIONS
Intrahepatic cystic lesions include 2 different conditions. Solitary cysts are retention pseudocysts, which should be referred to as a bile lake, and are associated with poor prognosis. Continuous beaded cysts are dilated bile ducts, which may be reversed. This distinction is important when considering the treatment strategy.
Tainaka
Takahisa
T
Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan. tainaka@med.nagoya-u.ac.jp
Kaneko
Kenitiro
K
Seo
Takahiko
T
Ono
Yasuyuki
Y
Sumida
Wataru
W
Ando
Hisami
H
D001649:Bile Duct Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003560:Cysts / Q000000981:diagnostic imaging / Q000209:etiology*; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000009:adverse effects*; D011379:Prognosis; D014463:Ultrasonography