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Surgery of Choledochal Cysts
Cystic enlargement of the bile ducts

Choledochal cysts are cystic enlargements of the bile ducts, which do not occur commonly.

Anatomy and Physiology

  • The gall bladder is a small pear shaped organ located beneath the liver in the right side of the upper abdomen
  • The cystic duct carries bile from the gallbladder and joins the common hepatic duct, which carries bile from the liver) to form the common bile duct. The common bile duct then empties into the duodenum, which is the beginning of the small intestine (Figure 1)
  • The main purpose of the gallbladder is to concentrate and store bile. It releases bile by ejecting it through the common bile duct into the small intestine when fatty foods are eaten. The bile aids in the digestion of fatty foods.
  • The gall bladder may be congenitally absent
Figure 1 - Anatomy of the liver, duodenum, gallbladder, hepatic ducts and common bile duct. © R. Walls

Pathology

  • The cause is not definitely known
  • It is more common in females and has a higher incidence in Japan
  • These cysts typically do not contain a normal biliary epithelium (lining cells of duct)
  • There are five main variants of choledochol cysts (Figure 2):
    1. Type I - fusiform (spindle shaped) dilation of the common bile duct and common hepatic duct
    2. Type II - a sac like duct off the side wall of a biliary duct
    3. Type III - cystic outpouching of the biliary duct into the duodenum
    4. Type IV - dilation of the hepatic ducts both inside and outside the liver
    5. Type V - dilation of the hepatic ducts inside the liver
Figure 2 - Types of choledochal cysts. See text. © R. Walls

History and Exam

  • These conditions present with jaundice (yellow skin), abdominal pain, fever associated with the jaundice and/or an abdominal mass
  • Clinical exam reveals the jaundice and often a tender mass in the upper abdomen on the right side
  • These conditions are not always present in infancy

Tests

  • It may be diagnose before birth by ultrasound over the uterus as early as 20 weeks of gestation
  • Laboratory tests show abnormal liver function
  • Multiple imaging tests help make the diagnosis:
    1. Ultrasound of the abdomen usually makes the diagnosis
    2. Magnetic Resonance Imaging (MRI) demonstrates the cysts
    3. ERCP ( See Endoscopic Retrograde CholangioPancreatography). An endoscope is passed through the stomach into the duodenum and then through the ampulla of Vater into the lower common bile duct. A dye is injected that shows up the hepatic ducts on X-ray. This test has some risk
    4. Nuclear scanning. A compound, which is secreted by the liver into the hepatic ducts and containing a medical nuclear isotope, is injected. Nuclear scanning of the liver and bile ducts is then carried out and an image of the ducts produced

Indications for Surgery

Surgery is indicated to correct the abnormality and prevent progressive liver dysfunction resulting in cholangitis (inflammation of the ducts) and biliary cirrhosis (chronic inflammation of the liver)

  • Surgery
    1. Regardless of the type of choledochal cyst surgery includes
    2. An initial exploration of the abdominal contents for other abnormalities
    3. An operative cholangiogram of the bile ducts, which is carried out by injection of a dye that shows up on X-ray into the gall bladder or a hepatic duct outside the liver
    4. Cholecystectomy (removal of the gall bladder - See Cholecystectomy)
  • The type of defect determines the surgical procedure
    1. Types I & II - In addition to the gallbladder, the hepatic duct cysts outside the liver are removed. This is followed with a hepaticojejunostomy in which the hepatic duct is anastomosed (joined) to the jejunum using a Roux-en-Y jejunal loop and end to side jejunum to jejunum anastomosis (Figure 3)
    2. Type III - The procedure varies with this type. Often there is excision of the cyst along with sphincteroplasty, division of the sphincter of Oddi (muscle fibers at the lower end of the common bile duct) which promotes bile drainage into duodenum
    3. Type IV - This type of choledochal cyst may require total removal the hepatic ducts lying outside the liver and partial removal of liver at the junction of the hepatic ducts from the right and left sides of the liver. This junction of ducts is then anastomosed to a loop of bowel
    4. Type V - If the cysts are confined to a lobe of the liver then partial removal of the involved sites is carried out. Extensive involvement may ultimately require a liver transplant
Figure 3 - The gallbladder and the hepatic duct cysts outside the liver are removed. This is followed with a hepaticojejunostomy in which the hepatic duct is anastomosed to the jejunum using a Roux-en-Y jejunal loop and end-to-side jejunum to jejunum anastomosis. © R. Walls

Complications

  • The complications associated with any major operation should be anticipated
  • Bleeding
  • Respiratory distress
  • Hypothermia (low body temperature)
  • Low urine output
  • Infection in blood, urine, abdomen, lung and/or bile
  • Bowel obstruction
  • Anastomotic leak (fistula) at any anastomotic suture site (bowel, liver)
  • Stricture (narrowing) at bile duct anastomosis
  • Cholangitis - infection of bile ducts

Post Op Care

  • The child is cared for in an intensive care unit with:
    1. Special monitoring to ensure adequate breathing since the child may require ventilator support
    2. Temperature monitoring
    3. Urine output monitoring
  • Fluids are given by vein until food is tolerated
  • A tube is placed through the nose into the stomach to keep the stomach decompressed
  • Antibiotics are given top prevent infection
  • Prolonged intensive care may be required

After Care

  • These children may be hospitalized for long periods and require special follow up
  • The goal is a healthy baby able to eat, urinate, and eliminate stool at the time of discharge without need for respiratory or special nutritional support
  • Follow up is determined by the complexity of the cyst formation
  • Physical activity is limited for 4-6 weeks
  • Follow up with surgeon in 7-10 days
  • Continued monitoring of liver function studies