Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy.

Introduction

Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy.

Methods

We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia.

Conclusion

The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.