Abstract
Background
Biliary atresia (BA) with a hilar cyst is an uncommon variant of BA that may be misunderstood to have a relatively favorable prognosis.
Methods
A single-center retrospective review of patients that matched BA with a hilar cyst (n=27) with BA without a cyst (n=27) over a 5-years period was done. All patients were diagnosed as type III BA by histologic examination and cholangiograms.
Results
There were no significant intergroup differences between baseline characteristics and outcomes after Kasai portoenterostomy surgery in two groups. The Kaplan–Meier survival curves showed no significant difference in cumulative native liver survival rate of the two groups (p = 0.584). Of the 27 BA patients with a hilar cyst, 8 were showed the cyst communicated with the gallbladder on cholangiograms, and Kaplan-Meier survival curves indicated that the cumulative native liver survival rate was significantly shorter (P=0.045) in them than those who the cyst was not communicated with the gallbladder.
Conclusions
Type III BA with a hilar cyst had no better prognosis compared with Type III BA without a cyst. A cyst communicated with the gallbladder may be associated with a poor eventual outcome for Type III BA with a hilar cyst.
Quantitative Liver Fibrosis Using Collagen Hybridizing Peptide to Predict Native Liver Survival in Biliary Atresia
