Biliary cysts: etiology, diagnosis and management

Abstract

Biliary cysts (BC) are rare dilatations of different parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. BC occur the most frequently in Asian and female populations. They are an important problem for pediatricians, gastroenterologists, radiologists and surgeons. Clinical presentation and management depend on the BC type. Cholangiocarcinoma is the most serious and dangerous BC complication. The other complications associated with BC involve cholelithiasis and hepatolithiasis, cholangitis, acute and chronic pancreatitis, portal hypertension, liver fibrosis and secondary liver cirrhosis and spontaneous cyst perforation. Different BC classifications have been described in the literature. Todani classification dividing BC into five types is the most useful in clinical practice. The early diagnosis and proper treatment are very important, because BC are associated with a risk of carcinogenesis. A malignancy risk increases with the age. Radiological investigations (ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography) play an important role in BC diagnostics. Currently, prenatal diagnosis using ultrasonography is possible. It allows to differentiate biliary disorders in fetals and to perform the early surgical treatment that improves results. In most patients, total cyst excision with Roux-Y hepaticojejunostomy is the treatment of choice. Surgical treatment of BC is associated with high success rate and low morbidity and mortality. The early treatment is associated with a lower number of complications. Patients following BC surgery require permanent and careful postoperative observation using laboratory and imaging investigations because of possibility of biliary anastomosis stricture and biliary cancer in tissue remnant.

Keywords: Biliary cysts; Biliary drainage; Hepaticojejunostomy.

Figure 1

Types of biliary cysts according to Todani classification. I: Solitary extrahepatic cyst (40%-85%): IA: Common type; IB: Segmental extrahepatic duct dilatation; IC: Diffuse extrahepatic dilatation; II: Extrahepatic diverticulum (2%-3%); III: Choledochocele (1.4%-5.6%); IVA: Multiple extra- and intrahepatic cysts; IVB: Multiple extrahepatic cysts (18%-20%); V: Multiple intrahepatic cysts (Caroli’s disease) (rare).