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After portoenterostomy (PE) for biliary atresia (BA), many patients suffer progressive deterioration of liver function and ultimately require liver transplantation. We retrospectively reviewed a single center's experience with pediatric liver transplantation for BA from 1988 to 2002. Sixty-six patients underwent 69 liver transplants for BA. Forty-two (63%) patients had previously undergone Kasai PE, 11 (17%) biliary appendicoduodenostomy (BAD), and 13 (20%) had no prior biliary drainage (NBD). The BAD procedure offered only short-term biliary drainage--the mean interval between PE and transplant was more than twice that for Kasai patients than for BAD patients (132 versus 49 weeks). The transplants included 11 cadaveric partial, 27 cadaveric whole, and 31 living related transplants. Three patients required retransplant. Prior PE did not increase the incidence of major perioperative complications or unplanned reexploration. After transplant, the 1-, 5-, and 10-year actuarial graft survival rates were 87%, 86%, and 80%, respectively. The 1-, 5-, and 10-year actuarial patient survival rates were 91%, 89%, and 83%. PE remains an important bridge to transplant. In conclusion, transplantation for BA offers excellent long-term graft and patient survival.

HYPOTHESIS/OBJECTIVE:Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. DESIGN/METHODS:Retrospective case series and literature review. SETTING/METHODS:Tertiary care university hospital. PATIENTS/METHODS:Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. MAIN OUTCOME MEASURES/METHODS:Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. RESULTS:Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. CONCLUSIONS:Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entire extrahepatic biliary tree should be removed when CC is diagnosed whether or not symptoms are present. The outcome of that approach was excellent.