Purpose: Significant differences in the clinical and pathological presentation of choledochal cysts between infants and older children have been noted. We developed a new management strategy according to these differences.
Methods: Between 1990 and 2001, we managed 34 cases of type I choledochal cyst. Nineteen patients were more than 1 year old. Fifteen patients underwent surgery before the age of 1 year, and eight before the age of 2 months. One patient was diagnosed prenatally. All patients underwent choledochal cyst excision and hepaticojejunostomy. Symptoms and signs, and findings from physical examination, laboratory tests, and pathology were recorded and analysed. Follow-up periods ranged from 6 months to 11 years.
Results: Eight cases who were less than 1 year old suffered from painless jaundice, and choledochal cysts in this group usually ended as a blind pouch. Their cystic amylase and lipase concentrations were within normal limits. Biliary cirrhosis was diagnosed by liver biopsy in three of these patients. In contrast, 17 of the 19 patients who were more than 1 year old had abdominal pain, and 15 of their cysts were connected to the pancreatic duct. Cystic amylase and lipase concentrations were often elevated. All patients had an excellent outcome.
Conclusion: Diseases with different pathogeneses may cause different clinical manifestations and pathology of choledochal cysts in infants and older children. Some patients develop biliary cirrhosis in the early months of life. Our findings show that it is safe and imperative to treat neonates and infants with choledochal cysts as soon as possible, as delayed surgery may cause severe complications.