Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East

Choledochal cysts are rare congenital cystic dilations of the extrahepatic or intrahepatic bile ducts that occur in fewer than 1% of individuals. These abnormal dilations were first described by Vater et al. in 1723 [1]; in 1977, Todani et al. proposed an updated classification system in which choledochal cysts were divided into five subtypes according to gallbladder structure and the anatomical location of the lesions [2].

The estimated prevalence of choledochal cysts in Western countries ranges between 1 per 50,000 and 1 per 200,000 [3]; the prevalence is 150-fold higher in East Asia [4], specifically in Japan, Korea, and China [5]. The reason for this geographical difference remains unknown. The cause of choledochal cysts is also unclear, and both congenital and acquired origins have been proposed [6], [7]. It is classically believed to be secondary to an anomalous pancreaticobiliary junction that causes distal common bile duct (CBD) obstruction, which leads to reflux of pancreatic enzymes into the CBD. The ductal wall consequently weakens, and a cyst forms [8]. Results of recent investigations suggest that acquired dysfunction of the sphincter of Oddi leads to pancreatic enzyme reflux and resultant cyst formation [9]. However, many patients exhibit no such abnormalities.

Choledochal cysts tend to occur more frequently in women in a 3:1 sex ratio [10]. In most affected patients, choledochal cysts are diagnosed during infancy or childhood; approximately 67% of patients present before 10 years of age. In children, choledochal anomalies manifest with the classical triad of palpable abdominal mass in the right upper quadrant, intermittent abdominal pain, and jaundice. The condition may, however, be diagnosed at any age. Affected adults present mostly with nonspecific symptoms; as a result, diagnosis is often delayed. In an increasing number of adults, choledochal cysts are diagnosed incidentally through radiologic imaging performed for other reasons [11].

The management of choledochal cysts depends on the morphological subtype, the extent of disease, and the patient’s comorbid state. Early diagnosis is crucial, and surgical resection of the anomaly is generally preferred because of increased risks of developing liver failure or malignancy within the anomalous tissue [12], [13].

Most literature on choledochal anomalies has focused on East Asian populations, especially those in Japan, Korea, and China because of the high disease incidence. Data from Western populations have recently been published [14], but epidemiological data from East Asian or Western populations may not apply to Middle Eastern populations. Most results in Middle Eastern populations have been reported only in small-scale case studies and case series. A few larger scale studies have been published, but the data are older than 10 years [15], [16] or reflect only a short period of time [17]. We report a recent 20-year experience in a single Middle Eastern institution in diagnosing and managing choledochal anomalies.

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