A 33 Year-Old Male with a Type IIIA Choledochocele

Abstract

Purpose: Choledochal cysts are congenital biliary tree abnormalities that can involve the intrahepatic biliary ducts, extrahepatic biliary ducts, or both. They are rarely diagnosed, especially in the adult population. A 33 year-old white male with a past medical history notable for recurrent pancreatitis and a cholecystectomy presented to our institution with complaints of light stools, dark urine, and jaundice for several days. He did not take any medications regularly. He smoked 1 pack of cigarettes per day, rarely drank alcohol, and denied any illicit drug use. His family history was notable for heart disease. On physical examination, the patient was afebrile and his vital signs were stable. He displayed scleral icterus and diffuse abdominal tenderness, without any evidence of guarding or rebound. He was not jaundiced. Laboratory evaluation was notable for a total bilirubin of 13.3 mg/dL, direct bilirubin of 8.4 mg/dL, AST of 105 U/L, ALT of 172 U/L, and alkaline phosphatase of 408 U/L. Lipase was within normal limits. White blood count was within normal limits. Viral hepatitis panel was negative. HIV testing was negative. A right upper quadrant ultrasound was performed which showed intrahepatic and extrahepatic biliary duct dilatation and a cystic lesion in the head of the pancreas. An MRCP was then performed which showed a 2.7 cm x 2.7 cm cystic dilation of the pancreatic head, which was inseparable from the common bile duct and the pancreatic duct. It also demonstrated a dilated common bile duct of 1.7 cm, and a choledochal cyst that communicated with the common bile duct. An ERCP was performed with findings that included bulging at the major papilla, congestion at the minor papilla, and cystic dilation of the intraduodenal portion of the common bile duct with the pancreatic duct and common bile duct draining into the cyst. These findings were consistent with a type IIIA choledococele. A sphincterotomy was performed. A biliary stent was placed in the bile duct. Upon contrast injection into the pancreatic duct, diffuse irregularity was seen, consistent with mild chronic pancreatitis. A stent was placed in the ventral pancreatic duct. The patient tolerated the procedure well, displayed symptomatic improvement, and resolution of his abnormal liver function tests prior to discharge. Choledochal cysts were first recognized as biliary tree abnormalities in 1723. While rare, they present an important clinical problem. Diagnosis of these rare anomalies is crucial as they can lead to cholangitis, pancreatitis, and can place patients at risk of developing malignancy.