[Choledochal cyst IV A type with primary ductal stricture]

Area of Science:

• Gastroenterology and Hepatology
• Pediatric Surgery
• Medical Imaging

Background:

• Type IV A choledochal cysts (CCCs) are rare congenital anomalies involving cystic dilatation of the entire biliary tree, including intrahepatic ducts.
• This condition often presents with symptoms like jaundice and abdominal pain, necessitating accurate diagnosis and timely intervention.

Observation:

• A 19-year-old female presented with jaundice and abdominal pain.
• Diagnostic imaging, including computerized tomography and magnetic cholangiopancreatography, revealed a large (13.5 cm) Type IV A choledochal cyst with intrahepatic extension.
• A distal ductal stricture within the extrahepatic cyst was identified.

Findings:

• Surgical excision of the extrahepatic cyst component was performed.
• The procedure was combined with a Roux-en-Y hepaticojejunostomy to restore biliary continuity.
• The patient experienced no perioperative or late complications during follow-up.

Implications:

• This case demonstrates the successful surgical treatment of a complex Type IV A choledochal cyst.
• Early diagnosis and surgical intervention are crucial for managing these rare biliary anomalies.
• Roux-en-Y hepaticojejunostomy is an effective reconstructive technique following cyst excision, leading to favorable outcomes.