What is a Choledochal Cyst?

It means congenital cystic dilatation of the bile duct and is very rare.

Are There Types of Choledochal Cyst?

Yes, there are. Many classifications have been made until today, the most commonly used is the Todani classification.

Type I: There is a saccular or fusiform diffuse dilatation of the extrahepatic bile ducts. Type 1 is seen in approximately 90-95% of all choledochal cysts.

Type II: Diverticular dilatation of the extrahepatic bile ducts

Type III Choledochococele

Type IV: Multiple cysts in the intra- or extrahepatic (sometimes in combination) bile ducts

Type V: Hepatic fibrosis with single or multiple intrahepatic cysts, also known as CAROLI disease.

Are Choledochal Cysts in Children Familial or Genetic?

It is 3-4 times more common in girls. 65% of reported cases are Japanese. Similar histologic findings are present in all cysts except type III. The cyst wall is thick and there is supportive tissue including smooth muscle, whereas the choledochocele is lined with duodenal mucosa. Familial cases have been reported in the literature, but there is no information showing a definite familial or genetic inheritance.

What Causes Choledochal Cyst in Children?

There is no definite cause yet, but there are theories that have been proposed as possible causes.

These are

a-Congenital wall weakness

b-Proliferation during the development of the biliary tract - defect in the formation of ducts

c- Intrauterine reflux of pancreatic enzymes into the biliary tract

d-Distal duct obstruction. The last two theories are more accepted.

What are the Clinical Findings of Choledochal Cyst in Children?

The disease is clinically evaluated in two forms, infantile and adult.

Infantile type gives clinical findings in infants aged 1-3 months. Jaundice, acholic stool and hepatomegaly are the main clinical findings. Differentiation from biliary atresia may be difficult.

Adult type presents clinical findings after 2 years of age. It has three classic clinical findings; abdominal pain, abdominal mass and jaundice. This triad is seen in only 50% of patients. Jaundice is intermittent. The history includes occasional fever, abdominal pain and jaundice, especially in the eyes.

How is Choledochal Cyst Diagnosed in Children?

The disease is diagnosed by history and physical examination. The findings described above are detected in the history and physical examination. In recent years, the presence of a cyst can also be detected in the womb due to the development of monitoring of the baby in the womb. The disease should be confirmed by imaging methods and laboratory tests.

Imaging methods are performed after blood tests. Ultrasonography is the first imaging that should be done. Then MR Cholangiography should be performed, this should be done with contrast. These two imaging tests are usually sufficient. However, sometimes CT and Scintigraphy, Endoscopic Retrograde Cholangiography, Pancreticography, Percutaneous Transhepatic Cholangiography may be required.

What should be considered in the differential diagnosis of choledochal cyst in children?

Biliary atresia, Alagille syndrome, neonatal hepatitis, biliary hypoplasia, congenital hepatic fibrosis, primary sclerosing cholangitis, congenital stenosis of the biliary tract are the main diseases that should be considered in the differential diagnosis.

How is choledochal cyst treated in children?

The treatment of the disease is surgical. Surgical method is selected according to the type of cyst. The cyst should be removed completely and primary anastomosis or CASAI surgery should be performed. The surgery can be performed by open or closed (laparoscopic) method by experienced people.

How can you tell if the KASAİ Surgery was successful?

When the surgery is performed by experienced people with both methods, the success rate is complete or close to complete.

Does Laparoscopic (Closed) Surgery Have Advantages Compared to Open Surgery?

Yes, the main advantages are listed below;

• Less pain
• Less bleeding
• Less infection
• Less scarring or even no scarring
• Shorter hospitalization
• Faster and easier recovery
• Faster return to daily activity

How is laparoscopic (closed) CASAI surgery performed?

The abdomen is inflated by giving carbon dioxide (CO2) gas into the abdomen to create a large area in the abdomen through tiny holes in the abdomen. New tubes are inserted by making 3 0.3-1 cm wide incisions in the abdomen, one of which is in the belly button, in appropriate places on the skin. “Long surgical instruments” are inserted through these newly inserted tubes and the planned surgery is performed.

However, it should not be forgotten that Laparoscopy can cause more serious complications when performed by ineffective people.

What is done before KASAİ Surgery?

First of all, a good history should be taken and a general physical examination should be performed. Then the family (mother and father) should be well informed about the process, i.e. before, during and after the operation. If the child is older, the child is also included. A number of tests are performed, these tests are not general and are determined according to the patient. The patient is also seen by the anesthesiologist and the family and child are informed about the anesthesia process. The patient is fasted for 4 hours before surgery.

What is the Recovery Process After KASAİ Surgery?

Generally, in both methods, the patient is fed 3-4 days after the operation in accordance with the doctor and / or nurse directives. The patient is hospitalized for about 5-6 days and discharged.

When the patient is discharged, a dressing is applied, then there is no need for dressing, the stitches dissolve spontaneously and do not need to be removed. The patient continues his/her normal active life after surgery in line with the doctor's recommendations.

What are the Complications of Choledochal Cyst Surgery in Children?

In the early postoperative period, respiratory problems, anastomotic leakage and cholangitis attacks may occur.

In the Late Period; gastrointestinal system bleeding, cholangitis, choledocholithiasis, Portal Hypertension may be seen. However, Surgery is usually successful if the Cyst is completely removed.

*** The information provided here, the content of the website is organized for the purpose of informing the visitor, especially families. No information should be considered as advice by visitors and should not lead to any decision or action. Families should definitely have their patient examined by a pediatric surgeon, consult with him/her and make a decision by consulting his/her knowledge.