Choledochal cyst

Choledochal CystMethas Arunnart MD.

Department of surgerySongkhla hospital

Introduction

•Cyst dilatation thoughout the billiary tree•Approximately 1 in 100,000 - 1 in 150,000 •More common in some asian

countries(1:1000)•More common in women (3-4 times)•Frequently diagnosed in infancy or

childhood•Associated with billary cancer 10-30%

[~16%] (particularly cholangioCA-varies on age and type)

Anatomy

classificationFurther refined in 2003

Classification• Type I - Cystic or fusiform dilatation of the

CBD• Type II - Diverticulum of the CBD• Type III - Cystic dilatation limited in

intraduodenal portion of distal CBD• Type IV - Multiple cystic dilatation of of extra

±intrahepatic bile ducts(IVb)• Type V - Cystic dilatation of the intrahepatic

bile ducts with normal extrahepatic bile duct (referred to as Caroli disease)

PATHOGENESIS may be congenital or acquired

Congenital cysts may result from an unequal proliferation of embryologic biliary epithelial cells before bile duct cannulation is complete.•Fetal viral infection may also have a role •cyst formation may be the result of ductal

obstruction or distension during the prenatal or neonatal period.

Acquired cysts may be the result of an abnormal pancreaticobiliary junction (APBJ).•APBJ is a rare congenital anomaly, with a

prevalence of 0.03%•It is present in about 70 percent of

patients with biliary cysts and may be a significant risk factor for malignancy with the cyst.

•In addition, patients with APBJ without biliary cysts appear to be at a markedly increased risk for gallbladder cancer

Abnormal pancreaticobiliary junction

Clinical Presentation

•Jaundice •Pain in RUQ•Abdominal mass

CLINICAL MANIFESTATIONS 

•The majority of patients with biliary cysts will present before the age of 10 years.

•Infants with biliary cysts commonly present with▫conjugated hyperbilirubinemia (80

percent),▫failure to thrive▫an abdominal mass (30 to 60 percent).

• The triad of pain, jaundice, and an abdominal mass is found in 11 to 63 percent of infants.

In contrast, in patients older than two,the most common presenting symptom is

•Chronic abdominal pain(50 to 96 %) •recurrent cholangitis (34 to 55 %). •Abdominal mass (10 to 20 %)•Pancreatitis (20 %)•Biliary lithiasis (8 %)

Rarely, biliary cysts present with

• intraperitoneal rupture•bleeding due to erosion into adjacent

vessels• portal hypertension •secondary biliary cirrhosis due to

prolonged biliary obstruction and recurrent cholangitis.

• In addition, type III cysts can case gastric outlet obstruction due to the obstruction of the duodenal lumen or intussusception.

Investigation• Transabdominal ultrasound• Computed tomography• CT cholangiogarphy• Endoscopic ultrasound• Intraductal ultrasound• Endoscopic retrograde

cholangiopancreatography• Magnetic resonance cholangiopancreatography

Transabdominal ultrasound

•First imaging modality used for the evaluation

•Not detect type III and type V cysts. •sensitivity of 71 to 97 %

•Factors that may limit the usefulness of an ultrasound include the patient's body habitus, the presence of bowel gas, and limited visualization due to overlying structures.

Computed tomography•CT can detect all types of biliary cysts.•Can evaluate for the presence of

malignancy. It is also useful for determining the extent of intrahepatic disease in patients with type IVA or V cysts.

CT cholangiography

has high sensitivities for visualizing the •biliary tree (93%), •biliary cysts (90%),• intraductal stones (93%)

• However, its sensitivity is lower for imaging the pancreatic duct (64 %)

CT cholangiography

Endoscopic ultrasound(EUS) •EUS can demonstrate extrahepatic biliary

cysts and provide detailed images of the cyst wall and pancreaticobiliary junction.

•unlike transabdominal U/S, it is not limited by body habitus, bowel gas, or overlying structures.

Endoscopic ultrasound

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

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

Intraductal ultrasound (IDUS)

Hepatobiliary scintigraphy• using radio-labeled dyes : technetium-99m-

labeled hepatic iminodiacetic acid (HIDA), which is selectively taken-up by hepatocytes and excreted into the bile.

• HIDA scanning is useful for extrahepatic cysts, with a sensitivity up to 100% for type I cysts. However, it is inadequate at visualizing the intrahepatic bile ducts

• HIDA scanning may also be useful in cases of cyst rupture

HIDA SCAN

Cholangiography • Direct cholangiography (whether intraoperative,

percutaneous, or endoscopic) has a sensitivity of up to 100 percent for diagnosing biliary cysts and previously was a commonly obtained test.

• can identify abnormal pancreatobiliary junction, and filling defects due to stones or malignancy.

• Increase risk of cholangitis and pancreatitis. [ Patients with cystic disease are greater risk for these complications ]

Radiology

Magnetic resonance cholangiopancreatography [MRCP]•Does not have the risks of cholangitis and

pancreatitis as direct cholangiography

•Sensitivity 73 - 100 %.•less sensitive than direct cholangiography

for excluding obstruction. •The data are variable with regard to its

ability to diagnose an abnormal pancreatobiliary junction. [46-75%]

MRCP

Management

• In the past, some patients were treated with internal drainage via a cystenterostomy

•Because of these complications, patients requiring treatment now generally undergo

cyst excision with hepaticoenterostomy.•In patients with ascending cholangitis

require treatment with antibiotics and drainage. Drainage can often be obtained via ERCP or percutaneous transhepatic cholangiography.

TreatmentFor types I, II, and IV – • Excision of the extrahepatic biliary tree -

including cholecystectomy, with a Roux-en-Y hepaticojejunostomy are ideal.

• In some difficult case, some surgeons advocate leaving posterior cyst wall intact with mucosectomy

• In type IV, additional segmental resection of the liver may be appropriate

For type III • can offen be managed with endoscopic

sphincterotomy or endoscopic resection

For type V — As with type IVA cysts, •some patients with type V cysts will

eventually require liver transplantation.

Alternatives to surgery • In patients who refuse surgical resection or who

are poor surgical candidates, lesser interventions (such as LC or ERCP) may treat symptoms caused by gallstones or sludge.

• No proven effective method of screening biliary cysts for dysplasia or intramucosal cancer. If screening is attempted, an intraductal ultrasound is probably the most sensitive test for detecting early malignancy in the cyst wall.

Cystic Duct

Gallbladder

Choledochal Cyst

Hepaticojejunostomy Roux-en-Y

Complications

•Cholangitis•Biliary stone formation•Anastomotic stricture•Residual debris in the intrahepatic bile

ducts•Intrahepatic bile duct dilatation•Malignancy

Thank you for attention