Abstract

Choledochal cysts are rare congenital cystic dilatations of the biliary tree. Choledochal cysts in pregnancy are a rare entity and represent a diagnostic and therapeutic challenge. The authors present a case of two full-term pregnancies in a woman who underwent multiple hepatobiliary operations for a choledochal cyst. The patient had two major problems: a type IV(a) choledochal cyst with atrophy of the left side of the liver and a congenitally dilated pancreatic duct in the head of the gland. She underwent a drainage surgical procedure using a Roux loop of jejunum and a revision surgery in which complete excision of the cyst and hepaticojejunostomy was performed. The surgical management was completed by a left lateral hepatic segmentectomy. During her first pregnancy, she experienced an episode of cholangitis, most probably due to the remaining congenitally dilated pancreatic duct. In succeeding years, the patient presented with recurrent pancreatitis and finally she underwent a Whipple operation. After that, the patient had a second pregnancy without any complications from the biliary tract system. Although choledochal cysts rarely occur in pregnancy, clinicians should be aware of this condition, as delayed or inappropriate therapy may be catastrophic for both the mother and the fetus.

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