Choledochal Cysts

Practice Essentials

Choledochal cysts are congenital bile duct anomalies (see the image below). These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both.

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Computed tomography (CT) scan of a choledochal cyst involving the common hepatic duct.

Signs and symptoms

Infants with choledochal cysts can present dramatically with the following:

• Jaundice and acholic stools: In early infancy, may prompt workup for biliary atresia
• Palpable mass in the right upper quadrant of the abdomen, with hepatomegaly

The clinical manifestations in older children and adults are variable. Children diagnosed with choledochal cysts after infancy typically present with intermittent biliary obstruction or recurrent bouts of pancreatitis with the following features:

• Biliary obstructive pattern: Palpable right upper quadrant mass and jaundice
• Primary manifestation of pancreatitis: May pose diagnostic difficulty; patients frequently have only intermittent attacks of colicky abdominal pain (elevated amylase and lipase concentrations lead to the proper diagnostic workup)

The classic triad in adults with choledochal cysts is abdominal pain, jaundice, and palpable right upper quadrant abdominal mass. However, this triad is found in only 10-20% of patients.

Adults may present with the following:

• Abdominal pain: Most common symptom; there may be vague epigastric or right upper quadrant pain
• Jaundice or cholangitis
• One or more severe complications (eg, hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, cholelithiasis)

See Presentation for more detail.

Diagnosis

Testing

No laboratory studies are specific for the diagnosis of a choledochal cyst, but some may be used to narrow the differential diagnosis. The following tests may be helpful:

• Complete blood count with differential: Elevated white blood cell count with increased numbers of neutrophils and immature neutrophil forms may be noted in the presence of cholangitis
• Liver function studies: Elevated hepatocellular enzymes and alkaline phosphatase levels are nonspecific for choledochal cysts
• Serum amylase and lipase levels: Both may be elevated in the presence of pancreatitis, but they can also be elevated in the presence of biliary obstruction and cholangitis
• Serum chemistry levels: Results may be abnormal if the patient is vomiting (hypochloremic, hypokalemic metabolic alkalosis)

Imaging studies

The following imaging studies may be used to assess patients with suspected choledochal cysts:

• Abdominal ultrasonography: Test of choice for the diagnosis of a choledochal cyst; can be useful for antenatal diagnosis^{[1, 2]}
• Abdominal computed tomography (CT) scanning and magnetic resonance imaging (MRI): Help to delineate the anatomy of the lesion and the surrounding structures; can also assist in defining the presence and extent of intrahepatic ductal involvement
• Magnetic resonance cholangiopancreatography (MRCP): Useful for defining anomalous pancreatobiliary junctions^[3] and pancreatobiliary anomalies^[4]

Procedures

Percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP) are used to supplement the above noninvasive imaging studies when those studies fail to sufficiently delineate the relevant anatomy.

See Workup for more detail.

Management

Surgery

The treatment of choice for choledochal cysts is complete excision with construction of a biliary-enteric anastomosis to restore continuity with the gastrointestinal tract.^{[5, 6]}

The surgical management for each choledochal cyst type is described as follows:

• Type I: Treatment of choice is complete excision of the involved portion of the extrahepatic bile duct; a Roux-en-Y hepaticojejunostomy is performed to restore biliary-enteric continuity^[7]
• Type II: Complete excision of the dilated diverticulum comprising a type II choledochal cyst; the resultant defect in the common bile duct is closed over a T-tube
• Type III (choledochocele): Therapeutic choice depends on the size of the cyst; choledochoceles measuring 3 cm or less can be treated effectively with endoscopic sphincterotomy, whereas lesions larger than 3 cm (which typically produce some degree of duodenal obstruction) are excised surgically via a transduodenal approach—if the pancreatic duct enters the choledochocele, reimplantation into the duodenum may be required following excision of the cyst
• Type IV: Complete excision of the dilated extrahepatic duct, followed by a Roux-en-Y hepaticojejunostomy to restore continuity; intrahepatic ductal disease does not require dedicated therapy unless hepatolithiasis, intrahepatic ductal strictures, and hepatic abscesses are present (in such instances, resection of the affected hepatic segment or lobe is performed)
• Type V (Caroli disease): Hepatic lobectomy for disease limited to one hepatic lobe (left lobe usually affected); however, one should carefully examine the hepatic functional reserve before committing to such therapy; patients with bilobar disease manifesting signs of liver failure, biliary cirrhosis, or portal hypertension may require liver transplantation
• Lilly technique: When the cyst adheres densely to the portal vein secondary to long-standing inflammatory reaction, it may not be possible to perform a complete, full-thickness excision of the cyst; the Lilly technique allows the serosal surface of the duct to be left adhering to the portal vein, while the mucosa of the cyst wall is obliterated by curettage or cautery—theoretically, this removes the risk of malignant transformation in that segment of the duct

Supportive measures

No medical therapy specifically targets the etiology of choledochal cysts, nor is any drug or any type of nonsurgical modality curative. Patients with choledochal cysts who present at a late stage (ie, after the development of advanced cirrhosis and portal hypertension) may not be good candidates for surgery because of the prohibitive morbidity and mortality associated with these comorbid conditions.

Patients who present with cholangitis should be treated with broad-spectrum antibiotic therapy directed against common biliary pathogens, such as Escherichia coli and Klebsiella species, in addition to other supportive measures, such as volume resuscitation.

See Treatment for more detail.

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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. They may occur as a single cyst or in multiples within the biliary tree. In 1723, Vater and Ezler published the anatomic 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.^[8] The patient died a month after an attempt was made at percutaneous drainage of the mass. (See image below.)

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Operative specimen of a 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.

Based on findings from a retrospective analysis of 32 children and 47 adults with choledochal cysts, Shah et al concluded that because of differences with regard to the presentation, management, and histopathology of, as well as the outcomes related to, these lesions, choledochal cysts in children should be considered separate entities from those in adults.^[9] The authors reported the following findings^[9] :

• 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.
• 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.

For patient education resources, see the Digestive Disorders Center, as well as Gallstones, Pancreatitis, Cirrhosis, and Abdominal Pain in Adults.

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Pathophysiology

No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably multifactorial.^[10] 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.^[11] 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 the 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).

Type I cysts

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. They do not involve the intrahepatic bile ducts.

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Type I choledochal cyst.

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 cysts

Type II choledochal cysts (see image below) appear as an isolated true diverticulum protruding from the wall of the common bile duct. The cyst may be joined to the common bile duct by a narrow stalk.

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Type II choledochal cyst.

Type III cysts

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.

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Type III choledochal cyst (choledochocele).

Type IVA cysts

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.

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Type IV choledochal cyst (extrahepatic and intrahepatic disease).

Type V cysts

Type V (Caroli disease) cysts (see image below) consist of multiple dilatations limited to the intrahepatic bile ducts.^[11]

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Type V choledochal cyst (intrahepatic, Caroli disease).

Choledochal cyst of the proximal cystic duct

Rarely, a patient may present with an isolated choledochal cyst involving the proximal cystic duct.^[12] At present, this type of cyst may be categorized as either a type II or type VI cyst.^[12]

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Epidemiology

United States statistics

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 statistics

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).^[11]

Sex- and age-related demographics

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

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.^[9]

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Prognosis

The prognosis after excision of a choledochal cyst is usually excellent, but it is influenced by several factors, including patient age, cyst type, histologic features, and site.^[13] Moreover, patients need lifelong follow-up because of the increased risk of cholangiocarcinoma and gallbladder carcinoma, even after complete excision of the cyst. In adults, there appears to be a 6-30% risk of malignancy associated with choledochal cyst.^[13]

Morbidity/mortality

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, cholangitis, 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.^[14] The bile from the patients with choledochal cysts produced significantly more mitogenic activity in the cancer cell line than the bile from the controls.

Complications

Complications of choledochal cysts include the following:

• Patients undergoing excision of a choledochal cyst are subject to the usual complications associated with surgery, including hemorrhage, wound infection, bowel obstruction, and thrombotic complications.
• Postoperatively, patients are at risk of developing pancreatitis and ascending cholangitis.
• Late postoperative complications include development of intrahepatic bile duct stones and cholangiocarcinoma.
• Adult patients with long-standing subclinical ductal inflammation and biliary stasis may develop one or more of the following complications: hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis.

Other potential complications include cyst rupture, secondary biliary cirrhosis, bleeding, and obstructive jaundice.

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History

The patient 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.

Infants

Infants frequently present with obstructive jaundice and acholic stools. In early infancy, this may prompt a workup for biliary atresia. In addition, infants with choledochal cysts often have a palpable mass in the right upper quadrant of the abdomen, accompanied with hepatomegaly.

Children

Children diagnosed after infancy typically have a clinical picture of intermittent biliary obstruction or recurrent bouts of pancreatitis. Those with a biliary obstructive pattern can still present with a palpable right upper quadrant mass and jaundice.

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

Adults with choledochal cysts can present with one or more severe complications. Frequently, adults with choledochal cysts complain of vague epigastric or right upper quadrant pain and can develop jaundice or cholangitis.

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. The majority of patients only have one or two symptoms of the triad.

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Physical Examination

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.

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Nuclear medicine scan of a choledochal cyst.

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Nuclear medicine scan of a choledochal cyst.

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Nuclear medicine scan of a choledochal cyst.

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Complications

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Laboratory Studies

No laboratory studies are specific for the diagnosis of a choledochal cyst.

Complete blood cell count

An elevated white blood cell count with increased numbers of neutrophils and immature neutrophil forms may be observed in the presence of cholangitis.

Liver function studies

Liver function tests may be useful in narrowing the differential diagnosis. Hepatocellular enzyme and alkaline phosphatase levels may be elevated. None of these tests are specific for the diagnosis of a choledochal cyst.

Serum amylase and lipase concentrations may be increased in the presence of pancreatitis. Serum amylase concentrations also may be elevated in biliary obstruction and cholangitis.

Chemistry panel

Results of serum chemistry may be abnormal if the patient is vomiting. One might expect to see a hypochloremic, hypokalemic metabolic alkalosis in this clinical picture.

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Imaging Studies

Ultrasonography

Abdominal ultrasonography is the test of choice for the diagnosis of a choledochal cyst. Ultrasonography is useful in the antenatal period as well and can demonstrate a choledochal cyst in a fetus as early as the beginning of the second trimester.^{[1, 15]} Caroli disease has also been detected antenatally with ultrasound by Sgro and colleagues.^[2] (See image below.)

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Diagnostic ultrasonogram demonstrating a type I choledochal cyst in a 4-month-old child presenting with hyperbilirubinemia and transaminase elevations....

Endoscopic ultrasonography (EUS) appears to have the potential to differentiate between choledochal and pancreatic cysts, particularly in patients with type II choledochal cysts.^[16] It is not limited by bowel gas, intraabdominal structures, or body habitus compared to abdominal ultrasonography. In a retrospective review (2010-2014} of 4 women with either type II cysts, equivocal for choledochal cyst on magnetic resonance imaging (MRI), or possibility of branch-duct intraductal papillary mucinous neoplasm of the pancreas on computed tomography (CT) scan, EUS was able to demonstrate no communication in all cases between the choledochal structure and the common bile duct.^[16] Moreover, EUS-guided fluid aspiration could be used for further testing to differentiate between biliary cysts and other cystic structures.^[16]

CT scanning and MRI

Abdominal CT scanning and MRI help to delineate the anatomy of the lesion and the surrounding structures. These tests also can assist in defining the presence and extent of intrahepatic ductal involvement. Yu and associates published a series of 64 patients in whom magnetic resonance cholangiopancreatography (MRCP) was particularly valuable in defining anomalous pancreaticobiliary junctions.^[3] (See the images below.)

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Computed tomography (CT) scan of a choledochal cyst demonstrating intrahepatic extension involving the main left hepatic duct.

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Computed tomography (CT) scan of a choledochal cyst involving the common hepatic duct.

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Computed tomography (CT) scan demonstrating a large choledochal cyst and the adjacent gall bladder.

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Computed tomography (CT) scan of a large, saccular type I choledochal cyst.

Magnetic resonance cholangiopancreatography

Fitoz and colleagues described the use of MRCP in 17 children with pancreatobiliary anomalies.^[4] The reported diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) in choledochal cysts in this series was 100%.

Hepatobiliary scintigraphy

Hepatic iminodiacetic acid (HIDA) scintigraphy is a nuclear medicine examination that uses a radiolabeled bile salt (technetium-99m) that is given intravenously and is then taken up by the hepatocytes and excreted in the bile.

Invasive diagnostic studies

When noninvasive measures (eg, ultrasonography, CT scanning, MRI) fail to sufficiently delineate the anatomy, they should be supplemented by the addition of percutaneous transhepatic cholangiography (PTC) or endoscopic retrograde cholangiopancreatography (ERCP) which may have a sensitivity up to 100%. As reported by Nagi and coworkers, these studies are particularly helpful in demonstrating the presence of an anomalous pancreatobiliary junction and in delineating associated extrahepatic or intrahepatic strictures and stones.^[17]

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Histologic Findings

Evidence of chronic inflammation is typically observed in the cyst wall. The cyst wall is thin, fibrous, and frequently devoid of a true epithelial surface, although it can be lined by a low columnar epithelium. Note that infants can develop complete obstruction of the distal common bile duct secondary to acute and chronic inflammatory changes. In the liver, ductal fibrosis and portal edema may be present. Changes consistent with biliary cirrhosis may be observed in adults with long-standing disease. The most feared histologic abnormality is the presence of cholangiocarcinoma.

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Approach Considerations

The treatment of choice for choledochal cysts is complete excision.^[18] Patients with type I, II, or IV cysts are recommended for surgical excision due to the risk of malignancy, if they are deemed good surgical candidates. Type III cysts may be managed with endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy or endoscopic resection. Primary care physicians who encounter a patient with a choledochal cyst should consult a surgeon.

A retrospective (1996-2015) analysis of 36 adult patients with congenital biliary dilatation suggests that laparoscopic surgery may be effective and provide acceptable outcomes.^[19] A different retrospective study (2011-2016) of 20 adults with choledochal cysts (type I, n = 19; type IV, n = 1) who underwent laparoscopic choledochal cyst excision reached similar findings but noted there was one fatality involving bilioenteric anastomosis leak with pseudoaneurysm that led to uncontrolled intraabdominal hemorrhage.^[20]

It remains unclear what the optimal therapy is for the rare isolated choledochal cyst involving the proximal cystic duct.^[12] Treatment options that have been considered include a bilioenteric reconstruction (because of the wide cystic duct-bile duct junction), as well as cholecystectomy, in combination with examination of the specimen and frozen section (to rule out any abnormalities), with close follow-up owing to the risk of malignancy.^[12]

Pregnant women with choledochal cysts are managed on the basis of whether or not cholangitis is present.^[21] In those with cysts refractory to medical management, biliary tree decompression is indicated, followed by postpregnancy definitive treatment.^[21]

Contributing factors for recurrent biliary obstructions after primary laparoscopic hepaticojejunostomy in children with choledochal cyst appear to include the presence of an aberrant hepatic artery, unresolved hepatic duct strictures, and a poor anastomotic technique.^[22] Early surgical correction is important for minimizing liver injury.

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Medical Care

Appropriate antibiotic therapy and supportive care should be given to patients presenting with cholangitis. Patients who present at a late stage, after the development of advanced cirrhosis and portal hypertension, may not be good candidates for surgery because of the prohibitive morbidity and mortality associated with these comorbid conditions.

Note the following:

• No medical therapy specifically targets the etiology of choledochal cysts, nor is any drug or any type of nonsurgical modality curative.
• Patients who present with cholangitis should be treated with broad-spectrum antibiotic therapy directed against common biliary pathogens, such as Escherichia coli and Klebsiella species, in addition to other supportive measures, such as volume resuscitation.
• Again, it must be emphasized that these means are supportive and that surgery is the only currently available definitive therapy.

Patients with choledochal cysts require lifelong follow-up because of an increased risk of cholangiocarcinoma and gallbladder carcinoma, even after complete excision of the cyst.^[13]

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Surgical Care

The treatment of choice for choledochal cysts is complete excision of the cyst with construction of a biliary-enteric anastomosis to restore continuity with the gastrointestinal tract.^{[5, 6, 18, 23]} According to Jordan and associates, both partial resection of the cyst and internal drainage procedures expose patients to increased risks of cholangitis, pancreatitis, and cholangiocarcinoma.^{[24, 25]}

The positive results of proper surgical treatment were reinforced by Visser and colleagues.^[26] These investigators reported a series of 39 adult patients with choledochal cysts. Cholangiocarcinomas or gallbladder cancers were noted in 8 patients (21%) at the initial operation performed by the authors. Seven of these patients had previously undergone a partial cyst excision, drainage procedure, or expectant management. No cancer was noted during the follow-up care of the patients who underwent complete cyst excision.

Shimotakahara and coworkers compared Roux-en-Y hepaticojejunostomy to hepaticoduodenostomy for biliary reconstruction following choledochal cyst excision and concluded that hepaticojejunostomy was a better choice because of an unacceptably high rate of duodenogastric bile reflux (33.3%) in the hepaticoduodenostomy group.^[27] See a review of 79 cases by Mukhopadhyay et al.^[28]

In a report of three cases of laparoscopic choledochal cyst excision and Roux-en-Y reconstruction in children, one was converted to open operation owing to the involvement of the confluence of the lobar hepatic ducts.^[29] All three children did well postoperatively. In a more recent study of 110 patients (55 children, 55 adults) who underwent laparoscopic cyst excision and biliary-enteric reconstruction for type I choledochal cysts and IVA cysts (intrahepatic and extrahepatic fusiform cysts), investigators found an overall complication rate of 10% (including 3 of 6 patients who developed cholangitis that required intervention for anastomotic stricture), with a 2% reexploration rate; there was 1 postoperative death.^[18] Children had a significantly lower rate of blood transfusions and shorter operative time; 3 adults required conversion to open laparotomy.

Laparoscopic surgical management of choledochal cysts in a series of 12 adult patients (mean age, 37.3 y) was successful in all patients via complete cyst excision and reconstruction via Roux-en-Y hepaticojejunostomy.^[30] No mortalities and no anastomotic complications occurred. The mean operative time was 228 minutes. Patients were discharged from the hospital after an average stay of 5.8 days.

Robotically-assisted laparoscopic resection of choledochal cysts is a relatively recent technique. This technique was used successfully in the management of a 5-year-old child with a type I choledochal cyst.^[31] The total robotic operative time was 390 minutes, and the time for the entire procedure was 440 minutes. No complications occurred. The patient was reported well after 6 months of follow-up.

Choledochal cyst excision in 198 children early in the neonatal period has been reported to have a lower complication rate and less hepatic fibrosis, particularly in neonates who underwent excision of a choledochal cyst within the first 30 days of life.^[32]

No mortalities occurred in a study of 32 patients, 84% of whom underwent an initial operation with complete cyst excision and Roux-en-Y hepaticojejunostomy and the remaining 16% had revisional surgery for incompletely resected cysts with hepaticojejunostomy.^[33] The overall morbidity rate was 44%, with wound infection being the most common (19%). Malignancy was found in only one specimen (3%). The authors emphasized the importance of treating cyst-associated complications, such as pancreatitis and sepsis, before attempting to define cyst anatomy with ERCP or MRCP. This aids in delineating the extent of involvement of the biliary tree and the exact type of choledochal cyst. Furthermore, they reiterated the importance of complete cyst excision and reconstruction with Roux-en-Y hepaticojejunostomy.^[33]

Surgical management by choledochal cyst type

Type I

The treatment of choice is complete excision of the involved portion of the extrahepatic bile duct. A Roux-en-Y hepaticojejunostomy is performed to restore biliary-enteric continuity.^[7] (See images below.)

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Intraoperative cholangiogram of a type I choledochal cyst.

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Intraoperative image of a Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of a choledochal cyst.

Type II

The dilated diverticulum comprising a type II choledochal cyst is excised in its entirety. The resultant defect in the common bile duct is closed over a T-tube.

Type III (choledochocele)

The choice of therapy depends upon the size the cyst. Choledochoceles measuring 3 cm or less can be treated effectively with endoscopic sphincterotomy. Lesions larger than 3 cm typically produce some degree of duodenal obstruction. These lesions are excised surgically through a transduodenal approach. If the pancreatic duct enters the choledochocele, it may have to be reimplanted into the duodenum following excision of the cyst.

Type IV

The dilated extrahepatic duct is completely excised and a Roux-en-Y hepaticojejunostomy is performed to restore continuity. Intrahepatic ductal disease does not require dedicated therapy unless hepatolithiasis, intrahepatic ductal strictures, and hepatic abscesses are present. In such instances, the affected segment or lobe of the liver is resected.

Type V (Caroli disease)

Disease limited to one hepatic lobe is amenable to treatment by hepatic lobectomy. When this occurs, the left lobe usually is affected. Hepatic functional reserve should be examined carefully in all patients before committing to such therapy. Patients with bilobar disease who begin to manifest signs of liver failure, biliary cirrhosis, or portal hypertension may be candidates for liver transplantation.

Lilly technique

Occasionally, the cyst adheres densely to the portal vein secondary to long-standing inflammatory reaction. In this situation, a complete, full-thickness excision of the cyst may not be possible. In the Lilly technique, the serosal surface of the duct is left adhering to the portal vein, while the mucosa of the cyst wall is obliterated by curettage or cautery. Theoretically, this removes the risk of malignant transformation in that segment of the duct.

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