Transcript CHOLEDOCHAL CYSTS

CHOLEDOCHAL CYSTS
Aswad Habeeb Hameed Al-Obeidy
FICMS GE & Hep
CHOLEDOCHAL CYSTS
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Congenital anomalies of the biliary tract that
manifest as cystic dilatation of the extrahepatic and
intrahepatic bile ducts
The incidence rate of choledochal cysts is 1 in
13,000 to 15,000 in Western countries and as high as
1 in 1000 in Japan
These cysts are not familial
Females are more commonly affected than males
Cases have been described in utero and in elderly
patients
Approximately two thirds of patients come to
medical attention before age 10 years
Classification
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The classification proposed by Todani and colleagues
Several varieties of type I cysts, accounting for 80% to 90% of
cases
Exhibit segmental or diffuse fusiform dilatation of the common
bile duct
Ia, common type; Ib, segmental dilatation; Ic, diffuse dilatation
Type II cysts consist of a true choledochal diverticulum
Type III cysts consist of dilatation of the intraduodenal portion
of the common bile duct, or choledochocele
Type IV cysts may be subdivided into type IVa, multiple
intrahepatic and extrahepatic cysts, and type IVb, multiple
extrahepatic cysts
V, or Caroli's disease, which consists of a single or multiple
dilatations of the intrahepatic ductal system, should be viewed
as a form of choledochal cyst is not settled
Etiology
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The cause of choledochal cysts has not been established
Congenital weakness of the bile duct wall, a primary
abnormality of epithelial proliferation during embryologic
ductal development
Congenital obstruction of bile ducts have been suggested
A relationship to other obstructive cholangiopathies, such as
biliary atresia, has been proposed but not proven
Reovirus RNA has been detected by reverse transcriptase–
polymerase chain reaction methodology in hepatic or biliary
tissues of 78%
A high frequency (40%) of an anomalous junction of the
pancreatic and common bile ducts
Pathology
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The cysts are composed of a fibrous wall
There may be no epithelial lining or a low columnar epithelium
Mild chronic inflammation may be present
Complete, in-flammatory obstruction of the terminal portion of
the common bile duct is common in infants who have a
choledochal cyst
Liver histology in the affected neonate shows typical features
of large duct obstruction
Portal tract edema, bile ductular proliferation, and fibrosis may
be prominent
A pattern of biliary cirrhosis may be observed in older patients
with long-standing biliary obstruction
Carcinoma of the cyst wall may occur by adolescence
Clinical Features
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Disease often appears during the first months of life
As many as 80% of patients have cholestatic jaundice and
acholic stools
Vomiting, irritability, and failure to thrive may occur
Physical examination shows hepatomegaly and, in
approximately one half of patients, a palpable abdominal mass
Spontaneous perforation of a choledochal cyst may occur
In older patients, epigastric pain, which may result from
pancreatitis, is the most common symptom
Intermittent jaundice and fever may result from recurrent
episodes of cholangitis
The classic triad, consisting of abdominal pain, jaundice, and a
palpable abdominal mass, is observed in less than 20% of
patients
Diagnosis
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The diagnosis of a choledochal cyst is best established with
ultrasonography
Several reports have demonstrated that antenatal
ultrasonography can be used to detect a choledochal cyst in
the fetus
Sequential ultrasonographic examinations have allowed study
of the evolution of choledochal cysts during pregnancy
In the older child, percutaneous transhepatic cholangiography
or ERCP may help define the anatomic features of the cyst
MRCP is being used increasingly to evaluate the extent of the
cyst and defects within the biliary tree and to detect an
anomalous junction of the pancreaticobiliary ducts
In practice, most pediatric surgeons rely on an operative
cholangiogram to define the extent of intrahepatic and
extrahepatic disease
Treatment
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Preferred treatment for choledochal cyst is surgical excision of
the cyst with reconstruction of the extrahepatic biliary tree
Biliary drainage is usually accomplished by a
choledochojejunostomy with a Roux-en-Y anastomosis
Excision of the cyst reduces bile stasis and the risk of
cholangitis and malignancy
Simple decompression and internal drainage should be done
only when the complicated anatomic characteristics do not
allow complete excision
Long-term follow-up is essential, because recurrent
cholangitis, lithiasis, anastomotic stricture, and pancreatitis
may develop years after the initial operation
Caroli's Disease
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Caroli's disease is a subtype of choledochal cyst characterized by
diffuse intrahepatic dilatation
Classically segmental and saccular and is associated with stone
formation and recurrent bacterial cholangitis
May manifest as cholangitis, abscesses, jaundice, or cirrhosis
A more common type, Caroli's syndrome, is associated with a portal
tract lesion typical of CHF
Renal disease occurs in both forms, renal tubular ectasia occurs with
the simple form, and both conditions can be associated with
autosomal recessive polycystic renal disease
The gene encodes a large protein (4074 amino acids), which has been
called fibrocystin to reflect the main structural abnormalities in liver
and kidney
The intrahepatic cysts are in continuity with the biliary tract and are
lined by epithelium that may be ulcerated and hyperplastic
The cysts may contain inspissated bile, calculi, and purulent material
Caroli's Disease
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During childhood and adolescence because of hepatomegaly and
abdominal pain
The disorder appears in the neonate as renal disease or cholestasis
Stagnation of bile, leading to formation of biliary sludge and
intraductal lithiasis
Fever and intermittent jaundice may occur during episodes of
bacterial cholangitis
Hepatospleno-megaly is found in cases associated with CHF; affected
patients may exhibit bleeding esophageal varices
Ultrasonography, MRC, and computed tomography are of great value
Percutaneous or endoscopic cholangiography usually demonstrates a
normal common duct with segmental, saccular dilatations of the
intrahepatic bile ducts
Hepatic resection is indicated for disease limited to a single lobe
Therapy with ursodeoxycholic acid, 10 to 15 mg/kg/day in individual
doses
Liver transplantation is an option in pati-ents who have extensive
disease and frequent complications