A review of choledochal cyst in pediatric and adult patients

Abstract

A review of 80 pediatric and 13 adult patients with choledochal cyst disease occurring in a multiracial population is presented to define the incidence, clinical pattern, surgical techniques, and complications seen over periods of 30 years (pediatric) and 15 years (adult patients). In pediatric patients, most cases are now being diagnosed at a much earlier age, usually in infancy or early childhood, presumably due to the ready availability of ultrasound. Adult patients, by contrast, present a complex picture of heptobiliary disease, and delay in diagnosis is not uncommon. Jaundice appears to be the predominant symptom and the classical triad (pain, jaundice, and mass) is seldom seen. The surgical technique emphasises complete excision of the cyst, including the distal end, as the critical factor in the prevention of late complications. Avoidance of anastomotic stricture depends on the apposition of gut to healthy duct wall and not on the size of the anastomosis alone. Postoperative results have been excellent with no immediate mortality and no evidence of cholangitis in patients with extrahepatic cysts. The morbidity is related to patients with persistent intrahepatic biliary dilatation, Caroli's disease, and carcinoma. While endoscopic intervention, hepatic resection, and liver transplantation have been utilized in the management of cystic disease in the liver, the outlook for patients with cholangiocarcinoma associated with choledochal cyst remains extremely poor, with no survivors in this series.

Treatment of choledochal cyst in a pediatric population. A single institution experience of 15-years. Case series

“…We found this triad only in two patients, which makes up for 14.2%. Jaundice and abdominal pain are the most dominant clinical features in children [3] , [17] , [20] , [21] , these symptoms were found in our series in 78.5% and 85.7% respectively ( Table 2 ), and together were found in the 64.2% (n = 9, Table 1 ).…”

Intrahepatic cholangiocarcinoma arising 10 years after the excision of congenital extrahepatic biliary dilation

“…A MEDLINE search of all pertinent articles yielded reports of 12 patients in whom bile duct cancer developed after the primary resection of congenital biliary dilation (Table 1). [1][2][3][4][5][6][7][8][9] The 13 patients, including our own, consisted of 3 men and 10 women, and they ranged in age from 16 to 61 years. The mean period between the primary operation and the development of cancer was 8.2 years, ranging from 1.8 to 19.5.…”

“…However, some patients have been reported to develop biliary cancer long after a "separation-operation". [1][2][3][4][5][6][7][8][9] Most of the development in these patients occurs in the dilated bile duct (for instance, in the remnant choledochal cyst) in the hepatic duct at the anastomotic site, and in the intrahepatic bile duct, in Todani's type IV-A classification of congenital biliary dilation. 10 The development of intrahepatic cholangiocarcinoma after the "separation-operation" has not been reported in patients without dilation of the intrahepatic bile duct (such as those with Todani's type I congential biliary dilation).…”

Clinical problems in the surgical treatment of pancreaticobiliary maljunction

“…In the treatment of pancreaticobiliary maljunction, resection of the extrahepatic bile duct and biliary reconstruction is necessary 1. Treatment of this disease is sometimes more difficult in adults than in children, because the association with cancer and the incidence of postoperative cholangitis are higher in adults than in children . In this study, we compared these features and the therapeutic results of surgery for this condition in an adult and a pediatric group.…”