CHAPTER 46 - Bile duct cysts in adults

Abstract

First reported by Vater and Ezler in 1723, bile duct cysts represent a rare pathology. They exist most commonly as a pediatric surgical problem.1 The majority of bile duct cysts are diagnosed in infants and children within the first decade of life, and an estimated 25% are detected by 1 year of age with an additional 35% to 55% by the age of 10 years.2–5 Presentation occurs in adulthood in approximately 25% of patients.6,7 Although clinically similar, the presentation and therapeutic strategies for bile duct cysts in adults may differ substantially from those of younger patients.8–10 In contrast to the pediatric experience, adult patients have an increased rate of acute presentation with biliary and/or pancreatic symptoms,11 are more commonly associated with hepatobiliary pathology,12–14 and they are often first seen with complications of previous cyst-related procedures.12,15–18 The surgical management of bile duct cysts in adults is therefore complicated by co-existing hepatobiliary disease and the added technical difficulties of reoperative biliary surgery. Despite the heterogeneity of the disease and the absence of clinical trials, a collective consensus for excision of extrahepatic bile duct cysts has been generally accepted. However, the management of intrahepatic bile duct cysts remains controversial and the method of choice for reestablishing bilioenteric continuity after excision is debatable. This chapter examines the spectrum of hepatobiliary pathology encountered in adults with bile duct cysts and describes the surgical approaches for managing such patients.