ABSTRACT
Congenital biliary dilatation (CBD), or choledochal cyst, is a
cystic or fusiform dilatation of the common bile duct that is
uncommon in Caucasians. There is little doubt that CBD is a
congenital lesion with a strong hereditary component, which
may explain the higher incidence seen in Asia, and its familial
occurrence in siblings and twins.13 Traditionally, approxi-
mately half become symptomatic in infancy, and neonatal
cases have been uncommon. However, with advances in
diagnostic imaging techniques, its incidence is increasing,
particularly in neonates.411 In our series, about 20% of
patients were detected either neonatally or antenatally, and
interestingly, the ratio of cystic to fusiform-type CBD
neonatally or antenatally is 20:1, in contrast to an overall
ratio of 5:3.12
The treatment of CBD in early infancy has unique aspects
that must be considered in relation to the risks of surgery
itself and the size and physiological/immunological imma-
turity of the patient. Because CBD is commonly associated
with pancreaticobiliary malunion (PBMU) involving con-
current anomalies of the common channel, pancreatic duct,
and intrahepatic bile duct (IHBD), the importance of
cholangiography both preoperatively and intraoperatively
cannot be overemphasized. If these anomalies go unnoticed
by surgeons, they may be injured during surgery and cause
serious postoperative morbidity. Primary cyst excision (CE)
with biliary reconstruction to avoid two-way reflux of bile
and pancreatic secretions is now the standard procedure of
choice.
