Management Conference

Young girl with RUQ massand pain

Raika Jamali MDRaika Jamali MDDigestive Disease Research CenterDigestive Disease Research Center

Tehran University of Medical SciencesTehran University of Medical Sciences

A 11 years girl with A 11 years girl with abdominal painabdominal pain, , pruritispruritis and and icterusicterus from 3 months ago. from 3 months ago.

A A massmass in RUQ with continuous RUQ pain in RUQ with continuous RUQ pain but no obvious radiation or association but no obvious radiation or association with eating. with eating.

Abdominal pain from 1 year ago without Abdominal pain from 1 year ago without icterus or fever.icterus or fever.

Detection of cyst in RUQ not obviously Detection of cyst in RUQ not obviously localized by sonography from one month localized by sonography from one month before this admission, suggested as before this admission, suggested as hydatid cyst.hydatid cyst.

Treatment with Albendazole one month Treatment with Albendazole one month before admission.before admission.

Physical ExamPhysical Exam A well being girl with no distress. Vital signs were A well being girl with no distress. Vital signs were

stable. No fever.stable. No fever. Sclera wasSclera was icteric icteric with mild with mild anemiaanemia. . Chest & heart were normal.Chest & heart were normal. There was a There was a mass in RUQmass in RUQ in inspection. in inspection. Tenderness in RUQTenderness in RUQ without morphy sign. without morphy sign. An ill defined mass was detected in RUQ in An ill defined mass was detected in RUQ in

palpation which does not move with respiration.palpation which does not move with respiration. It was palpated about 5 cm below right costal It was palpated about 5 cm below right costal

margin.margin. Spleen was normal .No ascitis was detected.Spleen was normal .No ascitis was detected.

LAB DATALAB DATA

WBC=4500 ( 2% Eos)WBC=4500 ( 2% Eos) Hb=11.1 Hb=11.1 Ferritin=60Ferritin=60 MCV=75 Serum Iron=115 MCV=75 Serum Iron=115 MCH=24 TIBC=208 MCH=24 TIBC=208 MCHC=33MCHC=33 Plt=249000Plt=249000

BUN=9 BUN=9 AST=252AST=252 Cr=0.7 Cr=0.7 ALT=228ALT=228 Ca=7.5 Ca=7.5 ALP=1214ALP=1214 P=4 P=4 Bili direct =3.5Bili direct =3.5 Na=142 Na=142 Bili direct =5.5Bili direct =5.5 K=4 K=4 Total protein=6.5Total protein=6.5 FBS=108 FBS=108 Albumin=4 Albumin=4 ESR=6ESR=6 PT=13 PT=13 TG=62 LDH=505 TG=62 LDH=505 Cholesterol=94 Amylase= NL Cholesterol=94 Amylase= NL

Anti HBs Ag=negAnti HBs Ag=neg Anti HBs Ab=negAnti HBs Ab=neg Anti HBc Ab=negAnti HBc Ab=neg Anti HCV Ab=negAnti HCV Ab=neg

Anti Ecinococcus granolosus Ab=negAnti Ecinococcus granolosus Ab=neg

Abdominal SonographyAbdominal Sonography

A SUBHEPATIC A SUBHEPATIC MASSMASS MEASURING MEASURING 140 X100 MM WHICH CAUSED 140 X100 MM WHICH CAUSED MARKED DILATATION OF INTRA & MARKED DILATATION OF INTRA & EXTRA BILIARY DUCTS.EXTRA BILIARY DUCTS.

Abdominal CT ScanAbdominal CT Scan

A regular A regular lobulated cystic masslobulated cystic mass in right in right lobe of liver (120 x 120) without lobe of liver (120 x 120) without calcification causing dilatation of intra & calcification causing dilatation of intra & extra biliary ducts.extra biliary ducts.

MRCP & MRIMRCP & MRI Huge thicked wall cystic mass (140 x120 mm), Huge thicked wall cystic mass (140 x120 mm),

at the portahepatis that seems connected to at the portahepatis that seems connected to biliary tree and gall bladder, resulted in severe biliary tree and gall bladder, resulted in severe dilatation of intra hepatic bile ducts and dilatation of intra hepatic bile ducts and displaced right kidney posteriorly.displaced right kidney posteriorly.

Spleen, kidneys, bowel loops and abdominal Spleen, kidneys, bowel loops and abdominal wall are normal.wall are normal.

Finding could be due to congenital anomalies Finding could be due to congenital anomalies like choledocal cyst, Duplication cyst, like choledocal cyst, Duplication cyst, mesenteric cyst and hydatid cyst are in DDx.mesenteric cyst and hydatid cyst are in DDx.

Surgery reportSurgery report There was a There was a massmass(10 x 20 cm) in hilum of (10 x 20 cm) in hilum of

liver which had liver which had compressed the duodenumcompressed the duodenum anteroinferiorly. It was anteroinferiorly. It was not resectablenot resectable because of extension and adhesion to the because of extension and adhesion to the mesocolon & stomach.mesocolon & stomach.

Gall bladder was bilobulatedGall bladder was bilobulated.. Stomach ,intestine & uterus were normal.Stomach ,intestine & uterus were normal. Cyst was draineged which was full of bile.Cyst was draineged which was full of bile. Cystodoudenostomy to D2Cystodoudenostomy to D2 was done. was done.

PathologyPathology Microscopic Description:Microscopic Description:

Sections show Sections show fibrotic cyst wallfibrotic cyst wall with with columnar epithelial lining, inflammation columnar epithelial lining, inflammation and ulceration area.and ulceration area.

Diagnosis:Diagnosis:

Choledocal cyst wall, resection:Choledocal cyst wall, resection:

- Cyst wall with - Cyst wall with fibrosisfibrosis, , hemorrhagehemorrhage, , acute acute & chronic inflammation& chronic inflammation and and ulcerationulceration..

Biliary cystsBiliary cysts

Biliary cysts are cystic dilatations, which Biliary cysts are cystic dilatations, which may occur singly or in multiples may occur singly or in multiples throughout the bile ducts. throughout the bile ducts.

They were originally termed choledochal They were originally termed choledochal cysts (involving the extrahepatic bile cysts (involving the extrahepatic bile duct) but the clinical classification was duct) but the clinical classification was revised in 1977 to include intrahepatic revised in 1977 to include intrahepatic cysts. cysts.

The incidence of biliary cysts has been The incidence of biliary cysts has been estimated to be estimated to be 1:100,000 to 150,0001:100,000 to 150,000. .

The incidence is higher in some The incidence is higher in some Asian Asian countries (Japan) . countries (Japan) .

The female to male ratio is about 3:1.The female to male ratio is about 3:1.

In the past, the majority of cases were In the past, the majority of cases were reported in children, although more recent reported in children, although more recent series report equal numbers in adults and series report equal numbers in adults and children . children .

Pathologic features of choledochal cysts Pathologic features of choledochal cysts are variable, ranging from normal bile duct are variable, ranging from normal bile duct mucosa to carcinoma . mucosa to carcinoma .

More commonly in children there is a More commonly in children there is a densely fibrotic cyst wall with evidence of densely fibrotic cyst wall with evidence of chronic and acute inflammation .chronic and acute inflammation .

In adults, there are frequently In adults, there are frequently

inflammatory changes, erosions, sparse inflammatory changes, erosions, sparse distribution of mucin glands, and not distribution of mucin glands, and not infrequently metaplasia . infrequently metaplasia .

In the case of In the case of type IIItype III cysts (see below), the cyst cysts (see below), the cyst is most often lined by is most often lined by duodenal mucosaduodenal mucosa, and , and less commonly by bile duct epithelium.less commonly by bile duct epithelium.

Cysts may be congenital or acquired and Cysts may be congenital or acquired and have been associated with a variety of have been associated with a variety of anatomic abnormalities.anatomic abnormalities.

Familial occurrence of cysts has been Familial occurrence of cysts has been

described .described .

Congenital biliary cysts may be diagnosed Congenital biliary cysts may be diagnosed prenatallyprenatally and can be associated with and can be associated with biliary atresiabiliary atresia . .

Fetal viral infection may also have a role; Fetal viral infection may also have a role; reovirusreovirus RNA has been isolated from RNA has been isolated from biliary tissue of neonates with infantile biliary tissue of neonates with infantile biliary obstruction and choledochal cysts.biliary obstruction and choledochal cysts.

Cyst formation may be the result of Cyst formation may be the result of ductal ductal

obstructionobstruction or distension during the or distension during the prenatal or neonatal period. prenatal or neonatal period.

Abnormal pancreaticobiliary Abnormal pancreaticobiliary junctionjunction

An abnormal pancreaticobiliary junction (APBJ) An abnormal pancreaticobiliary junction (APBJ) is present in about is present in about 70 percent70 percent of patients with of patients with biliary cysts and may be a significant biliary cysts and may be a significant risk factor risk factor for the development of malignancyfor the development of malignancy in the biliary in the biliary cyst .cyst .

APBJ is characterized by a APBJ is characterized by a long common long common channelchannel (usually over 2 cm in length) and may (usually over 2 cm in length) and may represent represent failure of the embryological ducts to failure of the embryological ducts to migrate fully into the duodenummigrate fully into the duodenum. In support of . In support of this hypothesis is the observation that the this hypothesis is the observation that the ampulla of Vater is diminutive or flatampulla of Vater is diminutive or flat in patients in patients with APBJ. with APBJ.

ERCP demonstrating an anomalous ERCP demonstrating an anomalous pancreaticobiliary junction in a child with pancreaticobiliary junction in a child with recurrent abdominal pain and pancreatitis. recurrent abdominal pain and pancreatitis. Note the long, dilated common channel (thick Note the long, dilated common channel (thick arrow) containing a stone (thin arrow). The arrow) containing a stone (thin arrow). The patient also has pancreas divisum.patient also has pancreas divisum.

ERCP in an adult with obstructive jaundice ERCP in an adult with obstructive jaundice demonstrates an anomalous demonstrates an anomalous pancreaticobiliary junction with a malignant pancreaticobiliary junction with a malignant biliary stricture replacing the cystic duct biliary stricture replacing the cystic duct insertion. There is no evidence of a biliary insertion. There is no evidence of a biliary

cystcyst..

A long common channel may predispose A long common channel may predispose to to reflux of pancreatic juice into the biliary reflux of pancreatic juice into the biliary treetree, since the ductal junction lies outside , since the ductal junction lies outside of the sphincter of Oddi.of the sphincter of Oddi.

This can result in This can result in increased amylase increased amylase levels in bilelevels in bile , intraductal activation of , intraductal activation of proteolytic enzymes, alterations in bile proteolytic enzymes, alterations in bile composition, and theoretically biliary composition, and theoretically biliary epithelial damage, inflammation, ductal epithelial damage, inflammation, ductal distension, and cyst formation. distension, and cyst formation.

Elevated sphincter of Oddi pressuresElevated sphincter of Oddi pressures have have been documented in APBJ and could been documented in APBJ and could promote pancreaticobiliary reflux. promote pancreaticobiliary reflux.

APBJ may be usefully subdivided based upon: APBJ may be usefully subdivided based upon:

the presence of an the presence of an acute or right angleacute or right angle at the at the bile duct/pancreatic duct junction, bile duct/pancreatic duct junction,

the presence or absence of a the presence or absence of a dilated common dilated common channelchannel,,

and the presence or absence of a and the presence or absence of a dominant dominant dorsal pancreatic ductdorsal pancreatic duct . .

This classification system reflects anatomic This classification system reflects anatomic issues of importance to the surgeon during cyst issues of importance to the surgeon during cyst resection. resection.

Proteinaceous plugs or stones within a Proteinaceous plugs or stones within a dilated common channel ( dilated common channel ( figure 1) may ) may cause ductal obstruction and pancreatitis, cause ductal obstruction and pancreatitis, and and cyst resectioncyst resection alone may not resolve alone may not resolve the patient's symptoms. the patient's symptoms.

Surgical sphincteroplastySurgical sphincteroplasty or or endoscopic endoscopic sphincterotomysphincterotomy may be required in the may be required in the treatment of such patients. treatment of such patients.

(IA) common type; (IB) segmental dilatation; (IC) diffuse (IA) common type; (IB) segmental dilatation; (IC) diffuse dilatation; (II) diverticulum; (III) choledochocoele; (IVA) dilatation; (II) diverticulum; (III) choledochocoele; (IVA) multiple cysts (intra- and extrahepatic); (IVB) multiple multiple cysts (intra- and extrahepatic); (IVB) multiple cysts (extrahepatic); (V) single or multiple dilatations of cysts (extrahepatic); (V) single or multiple dilatations of

the intrahepatic ductsthe intrahepatic ducts. .

CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS

Infants:Infants: conjugated conjugated hyperbilirubinemiahyperbilirubinemia (80 %), (80 %), failure to thrivefailure to thrive, or an , or an abdominal massabdominal mass (30 (30

to 60 %). to 60 %). The triad of The triad of painpain, , jaundicejaundice, and , and abdominal abdominal

massmass is found in (11 to 63 %) . is found in (11 to 63 %) .

patients older than twopatients older than two:: chronic and intermittent chronic and intermittent abdominal painabdominal pain

(50 to 96 %).(50 to 96 %). Intermittent jaundiceIntermittent jaundice and and recurrent recurrent

cholangitischolangitis (34 to 55%). (34 to 55%). Abdominal massAbdominal mass is less common(10 to is less common(10 to

20%). 20%). PancreatitisPancreatitis (20%) (20%) Biliary lithiasisBiliary lithiasis (8%) (8%)

Rarely, Rarely, intraperitoneal ruptureintraperitoneal rupture, , bleedingbleeding due to erosion into adjacent vessels, or due to erosion into adjacent vessels, or portal hypertensionportal hypertension and and cirrhosis cirrhosis ..

DIAGNOSISDIAGNOSIS A diagnosis of biliary cyst should be A diagnosis of biliary cyst should be

considered in adults when a considered in adults when a dilated portion dilated portion of the bile ducts or ampullaof the bile ducts or ampulla is identified, is identified, especially in the absence of overt especially in the absence of overt obstruction. obstruction.

A high level of suspicion is required for A high level of suspicion is required for diagnosis, particularly for Type I cysts, diagnosis, particularly for Type I cysts, which may go undiagnosed unless which may go undiagnosed unless considered in the differential diagnosis of considered in the differential diagnosis of patients found to have ductal dilation. patients found to have ductal dilation.

Acute or chronic Acute or chronic biliary obstructionbiliary obstruction may may cause marked biliary dilation that cause marked biliary dilation that mimics a mimics a Type I cyst. Type I cyst.

Such patients usually present with Such patients usually present with jaundice or markedly jaundice or markedly elevated serum elevated serum alkaline phosphatasealkaline phosphatase, have a readily , have a readily identifiable obstructing lesionidentifiable obstructing lesion such as a such as a stone or stricture, and their biliary dilation stone or stricture, and their biliary dilation often often improves after appropriate treatmentimproves after appropriate treatment..

Careful evaluation for APBJ may help with Careful evaluation for APBJ may help with diagnosis in indeterminate casesdiagnosis in indeterminate cases. .

UtrasoundUtrasound or or CTCT may suggest the may suggest the presence of a biliary cyst.presence of a biliary cyst.

Direct cholangiographyDirect cholangiography (whether (whether intraoperativeintraoperative, , percutaneouspercutaneous, or , or endoscopicendoscopic) has long been considered the ) has long been considered the best test for diagnosis and evaluation. best test for diagnosis and evaluation.

Cholangiography demonstrates areas of Cholangiography demonstrates areas of cystic dilationcystic dilation, , excludes overt obstructionexcludes overt obstruction of the bile duct, and delineates the of the bile duct, and delineates the presence of an presence of an APBJAPBJ. It may also . It may also demonstrate demonstrate stonesstones or or malignancymalignancy in the in the cyst. cyst.

MRCPMRCP also appears to be useful for also appears to be useful for diagnosis. It accurately demonstrates diagnosis. It accurately demonstrates cystically dilated segments of the biliary cystically dilated segments of the biliary tree, and tree, and identifies APBJ in over 75identifies APBJ in over 75 percent of cases . percent of cases .

However, However, MR is less sensitive than direct MR is less sensitive than direct cholangiography for excluding obstruction. cholangiography for excluding obstruction.

Endoscopic ultrasound (EUS)Endoscopic ultrasound (EUS) can also can also demonstrate extrahepatic biliary cysts and demonstrate extrahepatic biliary cysts and provide detailed images of the cyst wall provide detailed images of the cyst wall and and pancreaticobiliary junctionpancreaticobiliary junction..

Intraductal ultrasound (IDUS)Intraductal ultrasound (IDUS) has been has been used for diagnosis of early used for diagnosis of early malignant malignant changes in a biliary cystchanges in a biliary cyst . .

This technique is likely to be more This technique is likely to be more sensitive than direct cholangiography for sensitive than direct cholangiography for detection of early malignancy in the cyst detection of early malignancy in the cyst wall. wall.

CANCER RISKCANCER RISK Biliary cysts are associated with an Biliary cysts are associated with an

increased risk of cancer, particularly increased risk of cancer, particularly cholangiocarcinoma. cholangiocarcinoma.

The incidence of The incidence of malignancy increases with malignancy increases with age. age.

0.70.7 percent in patients under 10 years of percent in patients under 10 years of age, age,

6.86.8 percent in patients 11 to 20 years of age, percent in patients 11 to 20 years of age, 14.314.3 percent in patients over 20 years of age percent in patients over 20 years of age 5050 percent has been reported in older percent has been reported in older

patients.patients.

The risk of malignancy is best studied The risk of malignancy is best studied in Type I and IV cysts, in Type I and IV cysts,

although Type V cysts (Caroli's although Type V cysts (Caroli's disease) have also been associated disease) have also been associated with a 7 percent risk of malignancy .with a 7 percent risk of malignancy .

Cancer is an uncommon complication Cancer is an uncommon complication of Type III cystsof Type III cysts, and may be limited to , and may be limited to those choledochoceles lined by biliary those choledochoceles lined by biliary rather than duodenal epithelium. rather than duodenal epithelium.

The cited statistics likely The cited statistics likely overestimate the risk of overestimate the risk of cancer in biliary cystscancer in biliary cysts, because most series , because most series include only symptomatic patients presenting include only symptomatic patients presenting with complications of their cyst, including with complications of their cyst, including malignancy. malignancy.

To calculate the true risk of malignant To calculate the true risk of malignant degeneration, the incidence of asymptomatic degeneration, the incidence of asymptomatic biliary cysts in the population should be used as biliary cysts in the population should be used as the denominator, a parameter that is unknownthe denominator, a parameter that is unknown..

If patients who developed malignancy at least If patients who developed malignancy at least two years after initial diagnosis of their cyst are two years after initial diagnosis of their cyst are studied, the incidence in adults appears lower studied, the incidence in adults appears lower (4.5 percent rather than 14.3 percent) . (4.5 percent rather than 14.3 percent) .

Evidence clearly points to a markedly Evidence clearly points to a markedly increased risk of cholangiocarcinoma in increased risk of cholangiocarcinoma in biliary cystsbiliary cysts compared to the general compared to the general population.population.

This evidence includes the occurrence of This evidence includes the occurrence of cholangiocarcinoma in patients as young cholangiocarcinoma in patients as young as 10 years of ageas 10 years of age , the occurrence of , the occurrence of synchronous and metachronous biliary synchronous and metachronous biliary cancers, and the subsequent development cancers, and the subsequent development of cancer in patients with incompletely of cancer in patients with incompletely resected cysts . resected cysts .

The The possibility of cancer should always be possibility of cancer should always be considered in a newly diagnosed adult considered in a newly diagnosed adult with a biliary cyst.with a biliary cyst.

Patients previously Patients previously treated with biliary treated with biliary bypass rather than cyst resection have a bypass rather than cyst resection have a risk of subsequent malignancy that seems risk of subsequent malignancy that seems higher than the risk in unoperated patientshigher than the risk in unoperated patients..

Patients who have previously undergone Patients who have previously undergone cyst resection continue to have an cyst resection continue to have an increased risk of carcinomaincreased risk of carcinoma. .

Malignancy may develop in portions of Malignancy may develop in portions of cysts that were left behind at surgery, at cysts that were left behind at surgery, at the the anastomotic siteanastomotic site, or in the , or in the pancreaspancreas. .

In one study, the In one study, the increased incidence of increased incidence of cholangiocarcinoma in biliary cysts was confined cholangiocarcinoma in biliary cysts was confined to patients with an APBJto patients with an APBJ . .

APBJ appears to increase the risk of biliary and APBJ appears to increase the risk of biliary and pancreatic malignancy even in patients without a pancreatic malignancy even in patients without a biliary cystbiliary cyst or ductal dilation . or ductal dilation .

K-rasK-ras mutations and mutations and p53p53 overexpression have overexpression have been demonstrated in the biliary mucosa of such been demonstrated in the biliary mucosa of such patients . patients .

Gallbladder cancer is the most common Gallbladder cancer is the most common malignancy seen in patients with APBJ and no malignancy seen in patients with APBJ and no bile duct cyst. bile duct cyst.

Thus, Thus, prophylactic cholecystectomy in patients prophylactic cholecystectomy in patients with APBJ has been advisedwith APBJ has been advised . .

MANAGEMENTMANAGEMENT Because of the risk of malignant Because of the risk of malignant

degeneration of the cyst, the current degeneration of the cyst, the current standard is standard is surgical excisionsurgical excision..

In addition to decreasing the risk of In addition to decreasing the risk of malignant degeneration, excision of cysts malignant degeneration, excision of cysts can reduce can reduce complications such as complications such as recurrent cholangitis, choledocholithiasis, recurrent cholangitis, choledocholithiasis, and pancreatitis, which were frequently and pancreatitis, which were frequently seen following the older surgical strategy seen following the older surgical strategy of cyst drainage by of cyst drainage by choledochojejunostomy, without resectioncholedochojejunostomy, without resection. .

In the case of extrahepatic cysts, resection is usually In the case of extrahepatic cysts, resection is usually followed by followed by hepaticojejunostomyhepaticojejunostomy for for reconstruction . reconstruction .

Some surgeons have advocated leaving the Some surgeons have advocated leaving the

posterior cyst wall intact and performing posterior cyst wall intact and performing mucosectomymucosectomy if dissection of an extrahepatic cyst if dissection of an extrahepatic cyst from the portal vein or hepatic artery is technically from the portal vein or hepatic artery is technically difficult . difficult .

The intrapancreatic portion of Type I cysts can The intrapancreatic portion of Type I cysts can generally be treated with an intramural dissection generally be treated with an intramural dissection down to the pancreaticobiliary junction, without down to the pancreaticobiliary junction, without pancreatic head resection . pancreatic head resection .

If left in place, the intrapancreatic portion of a Type I If left in place, the intrapancreatic portion of a Type I cyst can be associated with subsequent malignancy cyst can be associated with subsequent malignancy or stone formation in the cyst remnant . or stone formation in the cyst remnant .

A A partial hepatectomypartial hepatectomy may be indicated if may be indicated if intrahepatic cysts are present and resectableintrahepatic cysts are present and resectable. .

Surgical resection effectively treats Surgical resection effectively treats symptoms and appears to decrease, symptoms and appears to decrease, although not eliminate, cancer riskalthough not eliminate, cancer risk . .

The most frequent long-term The most frequent long-term complication complication of hepaticojejunostomy is stenosisof hepaticojejunostomy is stenosis of the of the biliary-enteric anastomosis leading to biliary-enteric anastomosis leading to cholangitis, jaundice, or cirrhosis. (25%)cholangitis, jaundice, or cirrhosis. (25%)

After surgery, patients should have yearly After surgery, patients should have yearly serum liver tests looking for biochemical serum liver tests looking for biochemical evidence of partial biliary obstruction. evidence of partial biliary obstruction.

Significant Significant elevations of the serum alkaline elevations of the serum alkaline phosphatase merit investigation and phosphatase merit investigation and treatmenttreatment, even in asymptomatic patients, , even in asymptomatic patients, to prevent secondary biliary cirrhosis. to prevent secondary biliary cirrhosis.

When an When an APBJAPBJ is present with a dilated is present with a dilated common channel, common channel, surgical or endoscopic surgical or endoscopic removal of stonesremoval of stones or protein plugs from or protein plugs from the common channel may be necessary in the common channel may be necessary in patients with recurrent pain or pancreatitis.patients with recurrent pain or pancreatitis.

Cholecystectomy should be considered in Cholecystectomy should be considered in patients with APBJpatients with APBJ and no bile duct cyst and no bile duct cyst because of the increased risk of because of the increased risk of gallbladder cancer. gallbladder cancer.

Patients with Patients with Caroli's diseaseCaroli's disease may may eventually require eventually require liver transplantationliver transplantation. .

Type IIIA cysts (Type IIIA cysts (choledochocelescholedochoceles) are often ) are often amenable to amenable to endoscopic sphincterotomyendoscopic sphincterotomy. .

Type IIIB cysts (diverticular choledochoceles) Type IIIB cysts (diverticular choledochoceles) may be resected surgically or endoscopically . may be resected surgically or endoscopically .

In patients with a biliary cyst who refuse In patients with a biliary cyst who refuse resective surgery or are resective surgery or are poor surgical poor surgical candidatescandidates, lesser interventions (such as , lesser interventions (such as laparoscopic laparoscopic cholecystectomy and ERCPcholecystectomy and ERCP) may ) may treat symptoms caused by gallstones or sludge. treat symptoms caused by gallstones or sludge.

If If screeningscreening is attempted, is attempted, intraductal ultrasoundintraductal ultrasound is probably the most sensitive available test for is probably the most sensitive available test for early malignancy in the cyst wallearly malignancy in the cyst wall