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**Chapter 5**

**Abstract**

*Hasan Özkan Gezer*

Pediatric Choledochal Cysts:

Choledochal cysts (CCs) are congenital cystic dilatation of extrahepatic and/ or intrahepatic bile ducts. CCs are more common in Asian population, the cause is still unknown. Although the etiology is controversial, the main elements in the natural historical emergence of the type I and type IV, which make up the majority of all types, have become clearer. The majority of CCs are diagnosed in childhood. Clinical presentation varies from jaundice in young patients to nonspecific abdominal pain in older, but morbidity increases with complications such as cholangitis, pancreatitis, perforation, hepatitis, liver failure, and malignancy in delayed diagnosed patients. MRCP is considered the current gold standard diagnostic modality that is able to accurately assess biliary anatomy. Although the treatment approach has been formed over the years, it still has not reached the last state. Eventually, the removal of the entire cyst and the reconstruction of the remaining biliary tract to drainage is the current treatment approach. But the dilemma is the way of reconstruction procedure (hepaticoduodenostomy or hepaticojejunostomy). All patients should be followed up for a long period of time, regardless of the surgery method.

**Keywords:** choledochal cyst, children, hepaticoduodenostomy, Roux-en-Y

Choledochal cysts (CCs) are congenital dilatations of extrahepatic and/or intrahepatic bile ducts defined by Vater and Ezler in 1723. It is a rare biliary entity with an estimated incidence of 1:100–150,000 live births in Western countries. In the Asian population, the incidence can be as high as 1:1000 live births. CC is primarily a childhood disease—up to 80% of patients are diagnosed before 10 years of age. The original classification, first described by Alanso-Lej and colleagues in 1959, was changed in 1977 by Todani and colleagues that classified the CCs as five types. In addition, isolated cystic dilatation of the cystic canal was identified subsequently and proposed as type VI, apart from the revised Todani classification. Although the etiology is controversial, the main elements in the natural historical emergence of the type I and type IV, which make up the majority of all types, have become clearer. A common symptom is nonspecific abdominal pain in older children. When the cyst is complicated, the diagnosis is delayed, the treatment becomes complex, and the results are affected. External drainage (ED), internal drainage (ID), total cyst excision (CE) + hepaticoduodenostomy (HD), and total CE + hepaticojejunostomy (HJ) were defined according to historical development of treatment. Resection is considered that is necessary to prevent further complications and long-term sequel.

hepaticojejunostomy, common bile duct

**1. Introduction**

Unknowns are Decreasing
