Choledochal Cysts and other Biliary Malformations
What are choledochal cysts?
Choledochal cysts are a rare congenital anomaly usually caused by an abnormal union of the bile duct and the pancreatic duct (long common channel). Choledochal cysts are characterized by a dilation of the ducts that transport bile, and can occur anywhere between the liver, where bile is produced, and the first part of the intestine (duodenum), where it empties. They can occur in the ducts either inside or outside the liver. Most choledochal cysts (approximately two thirds) are diagnosed before your child reaches 10 years of age.
The incidence is only about 1 in 100,000–150,000 live births in developed societies and the cysts occur more often in females than males.
Signs and symptoms
Children with a choledochal cyst typically present with symptoms such as:
- Abdominal mass
Choledochal cysts are most commonly diagnosed by ultrasound. In rare cases the cysts may be diagnosed before birth on a prenatal ultrasound. Other imaging tests that may be used in the diagnosis of a choledochal cyst include CT scans (computerized tomography scan), magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP).
Choledochal cysts are typically diagnosed as one of several types:
- Type I: Cyst of the extrahepatic (outside the liver) biliary duct. Most common type, accounting for approximately half of all choledochal cysts.
- Type II: Pouching or sac on the common bile duct.
- Type III: Cyst within the wall of the duodenum or pancreas.
- Type IV: A type I cyst with extension of the dilation into the intrahepatic (inside the liver) biliary ducts.
- Type V: Multiple intrahepatic cystic dilations of the biliary ducts, also known as Caroli’s Disease.
If left untreated, complications of choledochal cysts may include:
- Gallstones and sludge in the bile duct and gallbladder
- Gastrointestinal (GI) obstruction
- Malignancy, or the development of bile duct cancer (cholangiocarcinoma)
Choledochal cysts are typically treated surgically, either by an open procedure or laparoscopically. Both procedures are performed in the operating room under general anesthesia. In both types of surgery for choledochal cysts, the cyst is removed and a reconstruction of the biliary system is frequently required using a section of intestine.
Laparoscopic excision of choledochal cyst
Laparoscopy, a minimally invasive surgical technique, may be used to remove your child’s choledochal cyst. The laparoscopic excision of a choledochal cyst begins with the surgeon making several small incisions in your child’s abdomen. In one incision, a small scope (or camera) is placed to allow the surgeon to see all of the abdominal organs. Small surgical tools are placed in 2 to 3 other incisions. The abdomen is filled with gas during the procedure to allow the surgeon to see easily.
The surgeon uses these tools to remove the cyst through a small incision inside your child’s belly button. The biliary system is then reconstructed using a section of the intestine called the duodenum (this procedure is called a hepaticoduodenostomy) or by a section of the intestine called the jejunum (called a Roux-en-Y hepaticojejunostomy). DERMABOND (skin glue) will cover the incisions.
Open excision of choledochal cyst
The surgeon will make an incision in your child’s right upper abdomen. The surgeon then removes the cyst and reconstructs the biliary system using either the duodenum (hepaticoduodenostomy) or a section of the intestine called the jejunum (Roux-en-Y hepaticojejunostomy). Your child’s incision will be closed with dissolvable stitches under the skin edges. A strip of tape or DERMABOND (skin glue) will cover the incision.
After the operation, your child will go to the recovery room until he is awake and comfortable. He will then go to a hospital room. Your child will receive IV fluids until he is able to drink clear liquids without nausea or vomiting, and will slowly advance to a regular diet. We may give your child antibiotics through an IV to prevent infection. Pain medication will also go through the IV at first. When your child is eating, he can take pain medication by mouth.
Soon after surgery your child will need to get out of bed and begin to walk and move around. This helps to relieve gas pain that is common after surgery. It also helps your child heal faster. If the procedure was done laparoscopically, your child will go home in approximately 3 to 5 days. If your child had an open excision of a choledochal cyst, he will remain hospitalized a few days longer to ensure full recovery.
Your child’s surgeon and care team will discuss his recovery process with your family, including potential risks and complications to watch for:
- Bile leak
- Strictures (narrowing of the bile duct)
- Wound infection
- Intrahepatic biliary stone formation
While cyst removal will reduce the risk of bile duct cancer (cholangiocarcinoma), your child will still be at higher risk of developing cancer in the remaining bile ducts inside the liver compared to the general population. There is also risk of narrowing in the reconstructed bile duct. Continued yearly follow up with a gastroenterologist or your pediatric surgeon is recommended to monitor for these complications. Your child may require ongoing blood work and imaging at regular intervals.
When to call your doctor
Please call the Division of Pediatric General, Thoracic and Fetal Surgery at 215-590-2730 if your child has any of the following symptoms in the days following surgery:
- Fever (a temperature of 101.5 degrees or higher)
- Increase in pain
- Any signs of infection, including redness, swelling and/or drainage at incision site(s)
- Any further questions or concerns
Once home, your child can gradually return to his normal activities as tolerated.