Introduction: Choledochal cyst is a rare inborn dilatation of biliary pathways associated with anatomical defect in the connection of biliary and pancreatic outlet. The incidence in the western countries is around 1:10,000 live-born children.
If the cyst is not resected there is a high risk of cholangiogenic carcinoma. Method: Retrospective analysis of 42 patients with a choledochal cyst operated on at the Clinic of Child Surgery in the period of 1991-2007.
Results: The mean age of the patients was 4.7 years (1.5 months - 14.5 years). The most frequent clinical symptoms were: abdominal pain 24 times, icterus 16 times, cyst of choledochus in two cases, always found accidentally during US examination of the belly.
Sonography was performed in all 42 patients, ERCP in 30 subjects. According to Todani classification Type I was found in 35 children, type II in two children and type IV in five subjects.
The complete extirpation of the cyst and hepaticojejunoanastomosis (HJA) was performed in 16 children, a partial extirpation and mucosectomy with HJA in 25 children and extirpation of the ductus choledochus cyst in one patient. Frequent complications included sepsis in two cases, cholangeitis is one and in another case with biliary fistula a re-operation was required.
The patients are presently one to 17 years after the surgery in good condition without a medicament therapy. Late complications included one case of obstruction icterus, stricture of hepatic duct, and lithiasis in the remainder of choledochus, and pancreatitis and colic abdominal pains.
Conclusions: The patients having undergone extirpation of choledochal cyst and HJA should be continually observed for long period of time although late complications are infrequent. The authors recommend annual ultrasonographic examinations o the liver and subhepatic region including a laboratory examination and the examination of liver by magnetic resonance after 5 years.