Shinichi Shimadera

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up

INTRODUCTION Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC. PATIENTS AND METHODS Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy. RESULTS Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively. CONCLUSIONS Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.

Significance of ductal plate malformation in the postoperative clinical course of biliary atresia

PURPOSE Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance. METHODS Between 1988 and 2005, 31 patients with uncorrectable BA underwent hepatoportoenterostomy and steroid therapy. Immunohistochemistry was used to characterize biliary structures using cytokeratin 19. Specimens were defined as DPM-positive if a concentric cellular arrangement was detected. This retrospective study included comparisons of preoperative characteristics, the postoperative jaundice period, and cumulative steroid doses between patients with and without DPM. RESULTS Of the 31 patients with uncorrectable BA, 25 (80.6%) became jaundice-free. Ductal plate malformation was detected in 11 (35.5%) of the 31. Between the 2 groups, there were no differences in preoperative characteristics or in the postoperative jaundice-free rate. Among those who became anicteric, the postoperative jaundice period was 145.3 +/- 69.9 days in the DPM-positive group (n = 9) and 81.8 +/- 44.7 days in the DPM-negative group (n = 16) (P < .05). The total administered steroids were 149.7 +/- 78.2 and 95.0 +/- 60.2 mg/kg, respectively (P = .09). CONCLUSION Ductal plate malformation may disturb bile flow and require more steroid to improve bile drainage. Therefore, the presence of DPM in the liver predicts poor bile flow after hepatoportoenterostomy in infants with BA.

Clinical Course of Obstructive Jaundice Associated with Operated Meconium Peritonitis in Neonates

BACKGROUND/PURPOSE Meconium peritonitis (MP) may induce prolonged cholestasis after laparotomy. In this study, we investigated the postoperative clinical course of MP retrospectively and discuss the relationship between MP and the development of obstructive jaundice, including biliary atresia (BA). PATIENTS AND METHODS Between 1979 and 2008, 23 infants with MP underwent laparotomy at our institution. Eleven of the 23 infants (47.8%) developed obstructive jaundice postoperatively. The medical charts of these 11 infants were reviewed. RESULTS The causative disease underlying MP included jejunoileal atresia in 10 and cloacal anomaly in 1. Of these 11 infants, 4 had acholic stools. Nine of the 11 improved with conservative management including an expectant approach, choleretic agents, and exchange blood transfusion. To differentiate the diagnosis from BA, open cholangiography was required in 2 cases following negative HIDA scintigraphy and a small gallbladder on ultrasonography. One of these 2 cases was diagnosed as BA and underwent hepatic portoeneterostomy simultaneously, after which the infant became jaundice free. CONCLUSIONS Postoperative cholestasis after MP was a transient condition in most cases. However, ultrasonography and HIDA scintigraphy should be performed to differentiate BA in infants with MP who show prolonged jaundice with acholic stools.