Anomalous Drainage of the Common Bile Duct Into the Fourth Portion of the Duodenum

We report two cases of early-childhood anomalous drainage of the common bile duct (CBD) into the fourth portion of the duodenum, which caused recurrent abdominal pain and hyperamylasemia. In both patients, this anomaly was associated with a long common channel between the CBD and the pancreatic duct. The CBD was obstructed by compression from the first portion of the duodenum, which passed anteriorly. Both children required choledochoenteric anastomosis to achieve permanent resolution of the abdominal pain. Elevation of the first portion of the duodenum does not produce permanent relief from symptoms. We believe that these patients represent the first described to have this unusual malformation.