Congenital anomalies of the biliary system: choledochal cysts. Case reports

Abstract

Background. The incidence of congenital bile duct anomalies is 1:100,000-150,000. Biliary tree cystic dilatations can affect intra- and extrahepatic biliary ducts. Treatment of choice is surgery, typically Roux-en-Y hepaticoenteroanastomosis is performed. Prognosis after this operation usually is perfect, nevertheless, patient`s needs further follow-up because of the increased risk of cholangiocarcinoma. Aim. 1. To describe usual clinical symptoms and to demonstrate typical magnetic resonance imaging (MRI) findings of biliary system congenital anomalies. 2. To present four clinical cases of biliary system congenital anomalies. Materials and Methods. This pictorial review demonstrates the spectrum of congenital biliary duct system anomalies. Presentation provides basic morphological classification of congenital bile duct anomalies, the diagnostic value of MRCP, as well as its advantages and disadvantages. In addition, four clinical cases are analyzed, where congenital biliary system anomalies were diagnosed. Results. Bile duct dilatation is usually caused by biliary tree obstruction or non-obstructive diseases. Furthermore, non-obstructive diseases are divided into congenital and conditions caused by unknown etiology, which affects biliary tree. Non-obstructive congenital diseases or main bile duct cysts are rare and they may be discovered at any age, however, mostly in younger children (10 years and less). Main bile duct cysts classically present with the symptoms triad: right upper quadrant abdominal pain, jaundice and palpable abdominal mass. According to Todani classification, these cysts are divided into five main types with several subtypes. Diagnosis of this disease can be made after excluding other conditions which also cause bile duct strictures or dilatation. All the patients in the analyzed clinical cases complained of abdominal pain. Treatment in all of these cases was surgery. Conclusions. Main bile duct cysts are rare pathology. It is important