The innervation of choledochal cysts was studied to see if the distrib
ution of innervating ganglion cells can be utilized as a possible indi
cator of the damage and dilatation process of choledochus. Specimens f
rom 32 patients aged 1 month to 12 years were stained by the immunoper
oxidase method with neural tissue-specific antibodies, the innervating
ganglion cells were counted and their number was correlated with the
clinical severity patient's age-at-surgery, and intraoperative cholang
iographic and/or ERCP findings. In cystic dilatation, the total number
of ganglion cells within the choledochal wall was significantly lower
than in control specimens regardless of the diameter of the choledoch
al cyst, age-at-surgery, or clinical course. In fusiform dilatation, t
he total number of ganglion cells varied according to the severity of
the clinical presentation, and the diameter of the fusiform choledochu
s increased in proportion to the patient's age-at-surgery. The distrib
ution of innervating ganglion cells is thought to correlate quantitati
vely with the damage and dilatation process responsible for the choled
ochal cyst. These results may support a hypothesis that the pathogenes
is of cystic dilatation proceeds antenatally or soon after birth, whil
e that of fusiform dilatation begins after birth.