James L. Buxbaum1 and Shelly C. Lu2 1 University of Southern California, Los Angeles, CA, USA, 2 Cedars‐Sinai Medical Center, Los Angeles, CA, USA Biliary cysts are cystic dilations that occur anywhere in the biliary system. They mainly afflict children and young adults. Most biliary cysts are congenital. Todani et al. proposed the most widely used classification of biliary cysts. Most common biliary cysts are type I choledochal cysts. Type II are diverticulum cysts found anywhere in the extrahepatic ducts, type III are choledochocele cysts, type IV are multiple cysts in the intrahepatic and extrahepatic ducts, and type V are intrahepatic bile duct cyst (single or multiple). Clinical presentation of biliary cysts depends on the patient’s age. In infancy, jaundice, vomiting, failure to thrive, and hepatomegaly are often found. The classic clinical triad (pain, jaundice, and a palpable abdominal mass) has been reported in 11–63% of large series. In patients older than 2 years of age, the most common presentation is chronic and intermittent pain. Recurrent pancreatitis, cholangitis, and ductal stones are also frequently encountered. Older patients may present with carcinoma of the biliary tract, the most dire complication of biliary cysts (Figure 45.1). Diagnosis of biliary cysts requires a high index of suspicion. Percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP) provide the most detailed exams (Figures 45.2, 45.3a, 45.4, 45.5a). In infants, ultrasonography and hepatobiliary scintigraphy provide a sound basis for diagnosis (Figures 45.3b, 45.6). Ultrasonography is an excellent screening tool but provides little anatomical or functional information (see Figure 45.6). Hepatobiliary scintigraphy provides information about excretory patterns (see Figure 45.3b) and is excellent for postoperative patient follow‐up. Computed tomography is superior to ultrasonography in older patients but does not always demonstrate the relationship of the cyst to the biliary tree (Figure 45.7a). Magnetic resonance cholangiopancreatography (MRCP) is an attractive alternative to ERCP or PTC and shows the relationship of the cyst to the surrounding ductal system (Figures 45.7c, 45.8). An anomalous pancreaticobiliary ductal union (see Figure 45.4), in which the common bile duct enters the pancreatic duct at a right angle abnormally far from the ampulla of Vater, occurs commonly in association with choledochal cysts. This abnormal anatomy impairs normal sphincteric function at the pancreaticobiliary junction, which may lead to reflux of pancreatic juice into the bile duct, causing injury and cystic malformation. Cholangiography is the best means of diagnosis (see Figure 45.5a). Typically, the distal common bile duct appears “clubbed.” Emptying of contrast material is often delayed. Choledochoceles are easily distinguished from duodenal diverticuli and duodenal duplication cysts by filling during cholangiography but not during upper gastrointestinal contrast studies (see Figure 45.5b). Sarris and Tsang proposed a further anatomical classification of choledochoceles (Figure 45.9) which is useful for guiding therapy. Choledochoceles may be amenable to endoscopic sphincterotomy. The treatment of choice for the remainder of choledochal cysts is operative excision. For intrahepatic cyst (i.e., type IVA, V), treatment depends on the degree of involvement. When segmental cystic disease is confined to one lobe (more often the left lobe), then lobectomy is usually curative. Ultimately, if attacks of cholangitis are frequent and quality of life poor, hepatic transplantation may be the best therapeutic option. Histology of the cyst wall typically demonstrates extensive fibrous tissue with a lack of muscle and biliary epithelium (Figure 45.10). Type V is Caroli disease (see Figure 45.7
CHAPTER 45
Cystic diseases of the liver and biliary tract
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