CHOLEDOCHAL CYSTS

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CHOLEDOCHAL CYSTS

• Aswad Habeeb Hameed Al-Obeidy
• FICMS GE Hep

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CHOLEDOCHAL CYSTS

• 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

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Classification

• 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

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Etiology

• 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
  transcriptasepolymerase 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

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Pathology

• 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

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Clinical Features

• 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

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Diagnosis

• 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

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Treatment

• 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

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Caroli's Disease

• 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

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Caroli's Disease

• 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