Choledochal cyst: review of 74 pediatric cases

Seventy-four cases of CDC were treated at the Children's Hospital from 1977 to 1995. Female to male ratio was 5:1. Forty per cent of the patients developed symptoms within one year after birth and 75 per cent within 5 years of age. About one third of the cases were treated surgically within one year of age. Only 5 patients or 6.8 per cent had all the clinical triads of jaundice, abdominal pain and mass. Jaundice was the most common symptom in infants below one year of age but abdominal pain was the most common symptom in older children. Three newborn infants had associated biliary atresia. Established liver cirrhosis was noted during surgery in 9 patients who were operated upon within one year of age but none of the older children. All but one had either type I or type IV CDC, while the remaining one had Caroli's disease or type V CDC. Excision was the preferred treatment. Overall operative mortality rate after excision was 6.5 per cent. None of the patients who had internal drainage procedures succumbed. Infants below one year of age appeared to have high morbidity and mortality rates after surgery. Internal drainage procedure may be considered in high risk patients with advanced cirrhosis. Available information suggests that the occurrence of this disease in Thailand is probably more common than in Western countries and etiology of CDC in infants may be different from that in older children or adults.