Evaluating patients' outcome post-Kasai operation: a 19-year experience with modification of the hepatic portoenterostomy and applying a novel steroid therapy regimen.
BACKGROUND
We reported a modification of the hepatic portoenterostomy (HPE) for biliary atresia with favorable results. HPE is associated with a risk of hepatic impairment, so we adopted a novel steroid therapy regimen well suited to our procedure. This paper reports the results of our experience.


METHODS
Between 1991 and 2009, 53 patients (18 boys, 35 girls) underwent modified HPE with novel steroid therapy, which consisted of administering hydrocortisone immediately after surgery, followed by intravenous administration of prednisolone. The number of patients who became normal total bilirubin (TB) levels, frequency of early onset cholangitis and other postoperative complications, and outcomes were retrospectively studied.


RESULTS
The TB levels in 43 of the 53 patients became normal. Cholangitis was observed in seven, but all of them recovered. Other postoperative complications were noted in eight, but with no fatal cases. Of the 11 patients who underwent living-donor liver transplantation, 3 died after the transplant. Of the 53 patients, 39 are alive without liver transplantation and 34 have normal TB (range of observation period: 18 years and 9-2 months).


CONCLUSIONS
The novel steroid regimen may have contributed to the outcome and appears to be well suited to the modified HPE.
Suzuki
Tatsuya
T
Division of Pediatric Surgery and Transplant Surgery, Nagoya City University Hospital, Mizuho-ku, Nagoya, Aichi, Japan. tsuzuki@med.nagoya-cu.ac.jp
Hashimoto
Takashi
T
Kondo
Satoshi
S
Sato
Yoko
Y
Hussein
Mohamed Hamed
MH
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001656:Biliary Atresia / Q000188:drug therapy* / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D011183:Postoperative Complications / Q000453:epidemiology; D011239:Prednisolone / Q000627:therapeutic use*; D012189:Retrospective Studies; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D011239:Prednisolone; D006854:Hydrocortisone