Secondary biliary cirrhosis due to a rare choledochal cyst type II complicated with portal hypertension and pancytopenia in a young female patient.

Choledochal cyst type II, is an extremely rare cause of portal hypertension, severe pancytopenia in an adult patient, and a complication of long-standing disease. We present an uncommon cause of portal hypertension in a young female patient secondary to an obstructive choledochal cyst type II provoking massive splenomegaly and severe pancytopenia. A high level of clinical suspicion is important due to its high mortality rate if it remains undiagnosed. To our knowledge there are few publications describing this rare type of choledochal cyst in a Hispanic adult.