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Background

Choledochal cysts are congenital bile duct anomalies. These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. In 1723, Vater and Ezler published the anatomical description of a choledochal cyst. Douglas wrote the clinical report involving a 17-year-old girl presenting with jaundice, fever, intermittent abdominal pain, and an abdominal mass.1 The patient died a month after an attempt at percutaneous drainage of the mass. (See image below.)

Operative specimen of type I choledochal cyst.

In 1959, Alonzo-Lej produced a systematic analysis of choledochal cysts, reporting on 96 cases. He devised a classification system, dividing choledochal cysts into 3 categories, and outlined therapeutic strategies. Todani has since refined this classification system to include 5 categories. This article reviews the incidence, pathophysiology, diagnosis, and management of choledochal cysts.
Recent research

In a retrospective analysis of 32 children and 47 adults with choledochal cysts, Shah et al investigated the differences between these 2 groups with regard to the presentation, management, and histopathology of, as well as the outcomes related to, these lesions. 2 The following were among the authors' findings:
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A history of biliary surgery, pancreatitis, cholangitis, early postoperative complications, and late postoperative complications occurred, respectively, 5.1, 5.4, 6.4, 2.0, and 3.3 times more frequently in adults than they did in children. The classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass occurred 6.7 times more frequently in children than in adults. Fibrosis of the cyst wall was peculiar to children.

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Signs of inflammation and hyperplasia were primarily seen in adults. Long-term complications occurred in 29.7% of adults but in only 9.3% of children.

The authors concluded that because of such differences, choledochal cysts in children should be considered separate entities from those in adults.
Pathophysiology

No strong unifying etiologic theory exists for choledochal cysts. Th e pathogenesis is probably multifactorial. 3 In many patients with choledochal cysts, an anomalous junction between the common bile duct and the pancreatic duct can be demonstrated. This occurs when the pancreatic duct empties into the common bile duct more than 1 cm proximal to the ampulla. Some series, such as the one published by Miyano and Yamataka in 1997, have documented such anomalous junctions in 90-100% of patients with choledochal cysts. 4 This abnormal union allows pancreatic secretions to reflux into the common bile duct, where the pancreatic proenzymes become activated, damaging and weakening the bile duct wall. Defects in epithelialization and recanalization of the developing bile ducts and congenital weakness of the ductal wall also have been implicated. The result is formation of a choledochal cyst. These anomalies are classified according to the system published by Todani and coworkers. Five major classes of choledochal cysts exist (ie, types I-V), with subclassifications for types I and IV (ie, types IA, IB, IC; types IVA, IVB).
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Type I cysts (see image below) are the most common and represent 80-90% of choledochal cysts. They consist of saccular or fusiform dilatations of the common bile duct, which involve either a segment of the duct or the entire duct.

Type I choledochal cyst.


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Type IA is saccular in configuration and involves either the entire extrahepatic bile duct or the majority of it. Type IB is saccular and involves a limited segment of the bile duct. Type IC is more fusiform in configuration and involves most or all of the extrahepatic bile duct. Type II choledochal cysts (see image below) appear as an isolated diverticulum protruding from the wall of the common bile duct. The cyst may be joined to the common bile duct by a narrow stalk.

Type II choledochal cyst.


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Type III choledochal cysts (see image below) arise from the intraduodenal portion of the common bile duct and are described alternately by the term choledochocele.

Type III choledochal cyst (choledochocele).


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Type IVA cysts (see image below) consist of multiple dilatations of the intrahepatic and extrahepatic bile ducts. Type IVB choledochal cysts are multiple dilatations involving only the extrahepatic bile ducts.

Type IV choledochal cyst (extrahepatic and intrahepatic disease).


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Type V (Caroli disease) cysts (see image below) consist of multiple dilatations limited to the intrahepatic bile ducts. 4

Type V choledochal cyst (intrahepatic, Caroli disease). Frequency United States

Choledochal cysts are relatively rare in Western countries. Reported frequency rates range from 1 case per 100,000-150,000 to 1 case per 2 million live births.
International

Choledochal cysts are much more prevalent in Asia than in Western countries. Approximately 33-50% of reported cases come from Japan, where the frequency in some series approaches 1 case per 1000 population (as described by Miyano and Yamataka). 4
Mortality/Morbidity

The morbidities associated with choledochal cysts depend on the age of the patient at the time of presentation. Infants and children may develop pancreatitis, cholangitis, and histologic evidence of hepatocellular damage. Adults in whom subclinical ductal inflammation and biliary stasis may have been present for years may present with one or more severe complications, such as hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis. Cholangiocarcinoma is the most feared complication of choledochal cysts, with a reported incidence of 9-28%. Wu and colleagues exposed cells from a cholangiocarcinoma cell line to bile from patients with choledochal cysts and from controls with structurally normal biliary systems. 5 The bile from the patients with choledochal cysts produced significantly more mitogenic activity in the cancer cell line than the bile from the controls.

Sex

Choledochal cysts are more prevalent in females than males, with a female-to-male ratio in the range of 3:1 to 4:1.
Age

Most patients with choledochal cysts are diagnosed during infancy or childhood, although the condition may be discovered at any age. Approximately 67% of patients present with signs or symptoms referable to the cyst before the age of 10 years. 2
Clinical History

The history varies according to the age at presentation. Choledochal cysts can present dramatically in infancy. The clinical manifestations in older children and adults are more protean.
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Infants o Infants frequently present with jaundice and acholic stools. In early infancy, this may prompt a workup for biliary atresia. o In addition, infants with choledochal cysts often have a palpable mass in the right upper quadrant of the abdomen, accompanied with hepatomegaly. Children o Children diagnosed after infancy typically have a clinical picture of intermittent biliary obstruction or recurrent bouts of pancreatitis. o Those with a biliary obstructive pattern can still present with a palpable right upper quadrant mass and jaundice. o Children whose primary manifestation is pancreatitis may pose some difficulty in arriving at the correct diagnosis. These patients frequently have only intermittent attacks of colicky abdominal pain. Biochemical testing reveals elevated amylase and lipase concentrations, which lead to the proper diagnostic workup. Adults o Adults with choledochal cysts can present with one or more severe complications. o Frequently, adults with choledochal cysts complain of vague epigastric or right upper quadrant pain and can develop jaundice or cholangitis. o The most common symptom in adults is abdominal pain.

A classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass has been described in adults with choledochal cysts but is found in only 10-20% of patients.

Physical

A right upper quadrant mass may be palpable. This is observed more frequently in infancy and early childhood. Patients who develop pancreatitis present with nonspecific midepigastric or diffuse abdominal pain. The images below depict nuclear medicine scans of choledochal cysts.

Nuclear medicine scan of choledochal cyst.

Nuclear medicine scan of choledochal cyst.

Nuclear medicine scan of choledochal cyst.

Causes

No unifying etiologic theory exists for choledochal cysts. The pathogenesis probably is multifactorial.