Surgery of Choledochal Cysts

8/2/2019 Surgery of Choledochal Cysts

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Surgery of Choledochal CystsCystic 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 areeaten. 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 duct3. Type III - cystic outpouching of the biliary duct into the duodenum4. Type IV - dilation of the hepatic ducts both inside and outside the liver5. Type V - dilation of the hepatic ducts inside the liver


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Figure 2 - Types of choledochal cysts. See

text. R. Walls

History and Exam

These conditions present with jaundice (yellow skin), abdominal pain, fever associatedwith 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 diagnosis2. Magnetic Resonance Imaging (MRI) demonstrates the cysts3. 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 thehepatic ducts on X-ray. This test has some risk

4. Nuclear scanning. A compound, which is secreted by the liver into the hepaticducts 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 ofthe liver)
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Surgery

1. Regardless of the type of choledochal cyst surgery includes2. An initial exploration of the abdominal contents for other abnormalities3. 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

liver4. 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 theliver 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 thehepatic ducts from the right and left sides of the liver. This junction of ducts is

then anastomosed to a loop of bowel4. 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 isfollowed 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
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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 requireventilator support2. Temperature monitoring3. 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


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Answer to Case of the Week: Jan 7-Jan 14, 2010

2 different patients with same diagnosis


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Fusiform dilatation of common bile duct.

Diagnosis: Choledochal Cyst


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Choledochal cysts are spectrum of

malformations of the extrahepatic and

intrahepatic bile ducts.

One in 100,000-150,000 live births in US versus

1 in 1,000 live births in Japan.

2/3 of all choledochal malformations are

diagnosed before 10 years of age.

More common in females 3 or 4:1

Cholangiocarcinoma is a worrisome long-term

complication, 2-18% and 5-35 increased risk.

Caroli disease and Caroli syndrome risk of

cholangicarcinoma at a rate of 100x that of

general population.

TRIAD:

Jaundice

Right upper quadrant pain


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Palpable mass

Classification:

Todani5 types ( See the figure)

Type I-(A)Segmental or fusiform dilatation of

the common bile duct-most common(75-95%)

Type II- (B) Diverticulum of the duct protrudingfrom the lateral wall

Type III- (C ) Choledochocele

Type IV-( D) Multiple extrahepatic bile ducts

alone(type IV B) or in association with Caroli-type (E)

Type V- (F) Cystic dilatation of intrahepatic bile

ducts equivalent to Caroli disease

Pathophysiology: Anomalous junction of thecommon biliary and pancreatic ducts with

provides conduit for mixing of pancreatic juice

and bile.


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Residents Submitting Correct Diagnosis - Case of the Week

Radiology

Pediatrics

Choledochal cyst (type IV)

Added byWael Nemattallaon November 9, 2009 at 8:18am

View Images
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Previous|Next

Choledochal cysts are congenital conditions associated with benign cystic dilatation of bileducts. They are uncommon in western countries but not as rare in East Asian nations like Japan

and China.

They were classified into 5 types by Todani in 1977.

Classification was based on site of the cyst or dilatation.

Type I: Most common variety involving sacular or fusiform dilatation of a portion or entirecommon bile duct (CBD) with normal intrahepatic duct.

Type II: Isolated diverticulum protruding from the CBD.Type III or Choledochocele: Arise from dilatation of duodenal portion of CBD or where

pancreatic duct meets.
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Type IV: Dilatation of both intrahepatic and extrahepatic biliary duct.

Type V or Caroli's disease: Cystic dilatation of intra hepatic biliary ducts.

Symptoms of Choledochal Cyst

Some newborns with cysts have symptoms right away. Others have good bile flow at first, butthey get symptoms soon after birth. Some children dont have symptoms for a couple of years or

longer. Some people live with a choledochal cyst for many years but dont know about it becausethey dont have symptoms until they are an adult.

Babies or children with a choledochal cyst may have these symptoms:

Yellow color to the eyes and skin (jaundice)

Pain in the upper right belly (upper right quadrant pain)

Soft mass that can be felt in the upper right belly

Pale or clay-colored stools (feces)

Fever, if they have an infection (cholangitis) If they have an inflamed pancreas (pancreatitis): belly pain that may come and go, nausea and

vomiting

Choledochal Cyst Diagnosis

Sometimes doctors can see a choledochal cyst on an ultrasound before a baby is born (prenatalultrasound). If this happens with your baby, doctors will plan the tests and treatment your baby

will need after birth. Its not always possible to see a cyst before birth.

If your childs cyst was not seen but your child has symptoms after they are born, your childs

doctor will ask for a detailed history of your childs illness. The doctor will do a thorough exam.

There is no blood test for choledochal cysts. But the doctor may do blood tests to check whether

your childs bile ducts are inflamed or infected and whether their liver is working well.

The doctor will do an ultrasound of your childs belly (abdomen) to see your childs bile ductsand other organs in the area. Imaging studies like ultrasound help the doctor know whether there

is a cyst, where it is and how it is shaped. These factors help your childs doctor plan surgery to

remove the cyst.

Your child may also need other imaging studies, like these:

Cholangiographyinjecting dye (contrast) into your childs bile ducts and then taking an X-ray.

This may be done in one of two ways. One way is to inject the dye through your childs skin and

liver into their ducts (percutaneous transhepatic cholangiography, PTC). The other way is to

inject the dye through a small tube placed into your childs mouth and down their throat into

their intestine (endoscopic retrograde cholangiopancreatography, ERCP).

Abdominal CT (computed tomography) scan.

Abdominal MRI (magnetic resonance imaging) scan.


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HIDA scan (hepatobiliary iminodiacetic acid scan). A HIDA scan allows doctors to see whether a

special dye that collects in the liver can pass out of the liver through the bile

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Key words

What is a choledochal cyst?

Bilethat is produced in theliverflows through increasingly larger channels (ducts) within theliver and finally into even larger ducts that leave the liver, pass through the substance of the

pancreas, and then empty into theduodenum.

A choledochal cyst is a cyst (hollow outpouching) of the bile ducts. Choledochal cysts have been

classified into several different types depending on where they are located and whether they can

be seen as separate structures from the ducts (diverticulum-like); or whether they can be seen asa localized dilation (enlargement) of the ducts. Choledochal cysts are rare, occurring in less than

1% of individuals. The cause of choledochal cysts is unknown, but they are congenital, that is,

present from birth, and, therefore, represent developmental abnormalities of the bile ducts in thefetus.
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What are the symptoms and complications of choledochal cysts?

In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile.This leads tojaundiceand an enlarged liver. If the obstruction is not relieved, permanent damage

may occur to the liver - scarring and cirrhosis - with the signs ofportal hypertension(obstructionto the flow of blood through the liver) andascites(fluid accumulation in the abdomen). There is

an increased risk ofcancerin the wall of the cyst.

In older individuals, choledochal cysts are more likely to causeabdominal painand intermittent

episodes of jaundice and occasionallycholangitis(inflammation within the bile ducts caused by

the spread of bacteria from the intestine into the bile ducts). Pancreatitisalso may occur. Thecause of these complications may be related to either abnormal flow of bile within the ducts or

the presence ofgallstones.
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Surgery of Choledochal Cysts

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