Choledochal cyst in infancy: A follow-up study

From January 1980 to February 1997, 19 cases, 8 males and 11 females, of choledochal cyst were diagnosed before one year old. The majority of patients were diagnosed by ultrasonography before 6 months old (15/19: 79%), including two diagnosed prenatally. According to Todani's classification, type I(o) was the most common (74%), followed by type I(o) (26%). Fourteen patients underwent Roux-en-Y choledocho-or hepatico-jejunostomy and cyst excision, 3 patients underwent Kasai operation, and 1 patient underwent external biliary drainage only. The remaining one patient with Trisomy 18 anomaly refused operation. Four of the 10 patients in whom liver histologic examinations were performed, had liver cirrhosis. The follow-up period of these patients ranged from 6 months to 9 years, with a mean of 4.1 years. We divided these 19 cases into 2 groups, according to the presence or absence of biliary atresia. In the 7 infants with biliary atresia (37%), all presented with jaundice and acholic stool. Two patients died due to delayed presentation and surgery, both had liver cirrhosis. One patient is living with liver cirrhosis. Another patient was lost to follow-up, but frequent cholangitis was noted till 8 months old. The remaining 3 patients are living and well. In the 12 without biliary atresia, 9 patients are living and well. Two patients died, one due to Trisomy 18 anomaly and the other with delayed surgery and liver cirrhosis. One case was lost to follow-up. In summary: 1) a possibility of the association of biliary atresia in infants with choledochal cyst should be carefully searched and considered as a unique group; 2) ultrasonography is a good diagnostic tool in choledochal cyst during prenatal or infancy period; 3) the mortality cases were characterized by prolonged bile stasis, biliary cirrhosis, delayed surgery, or multiple anomalies; 4) surgery should be performed as early as possible for those with persistent jaundice and light colored stools.