Management of a massive choledochal cyst in a 12 year-old girl: Which imaging modalities should be performed preoperatively?

• Determining the anatomy and subtype of massive choledochal cysts is challenging.
• Multiple imaging modalities can be used to assess these lesions.
• Ultrasound, magnetic resonance imaging, and/or scintigraphy are often sufficient.
• Endoscopic and/or percutaneous cholangiography is usually not necessary.

A previously well 12 year-old girl presented to the emergency department with a three-day history of fatigue, pruritus, and epigastric discomfort. Abdominal ultrasound revealed a cystic mass in the right upper quadrant measuring 17.0 × 13.2 × 11.7 cm. Magnetic resonance imaging (MRI) confirmed the diagnosis of a choledochal cyst and hepatobiliary iminodiacetic acid (HIDA) scintigraphy demonstrated a communication between the cyst and biliary tree. Percutaneous cholangiography was also performed but did not provide additional diagnostic information. The patient was brought to the operating room a few days later for open resection of the choledochal cyst, cholecystectomy, and Roux-en-Y hepaticojejunostomy. Frozen sections were obtained to ensure complete excision of cyst mucosa. The patient continues to do well more than 18 months post-operatively with no signs of recurrent inflammation or malignancy. Multiple imaging modalities may be used preoperatively to assess the anatomy and subtype of choledochal cysts. The role of invasive options, such as percutaneous cholangiography or endoscopic retrograde cholangiopancreatograpy (ERCP), remains limited and unclear. Here, we present a potential imaging algorithm to assist with preoperative workup and avoid invasive diagnostic procedures whenever possible.