Choledochal cyst type IVa

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

History of abdominal pain at home. Now with sudden onset of jaundice.

Patient Data

Age: 19 months
Gender: Female
ultrasound

There are dilated proximal left intrahepatic duct with a dilated, irregular, and tortuous appearing CBD measuring up to 1 cm in diameter. There is a distended and minimally thick-walled gallbladder most consistent with inspissated bile, without evidence for calculi or pericholecystic fluid.

MRCP

mri

There is significant cystic dilatation of the main right and left biliary ducts with branching of the left duct versus distortion/displacement of the right hepatic duct due to a dilated right posterior duct. There is a focal area of dilatation in the suspected right posterior duct, measuring up to 1.6 cm. There appears to be a "waist"/area of narrowing just proximal to the "branching of the left ducts", with the left-sided ducts measuring 6.6 and 7.6 mm. The common bile duct is tortuous and dilated measuring 1.5 cm and tapers towards the ampulla with an intermediate-decreased signal in the distal CBD, most consistent with debris/sludge. There appears to be a dilated tortuous cystic duct measuring 1.1 cm just proximal to entering the CBD. The gallbladder is distended with high signal debris within the distal portion of the gallbladder with intermediate-decreased signal within the remaining gallbladder, consistent with the patient known inspissated bile/debris. There is visualisation of a slightly serpiginous, mildly dilated main pancreatic duct, measuring 2.2-2.4 mm; without dilatation of the branching ducts.

Intraoperative fluoroscopy

x-ray

Fluoroscopic image obtained following contrast injection into the biliary system demonstrate significantly dilated intra- and extrahepatic biliary ducts. There is a dilated and tortuous pancreatic duct.

Case Discussion

This is a choledochal cyst type IVa (cystic dilation of the intrahepatic and extrahepatic ducts). The patient underwent a cholecystectomy and jejunal-hepatic anastomosis.

Co-author:
Travis Bevington, MD

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