The term choledochal cyst refers to cystic* changes / dilatations of the bile ducts that run outside the liver (so-called "extrahepatic bile ducts"). The cause of these dilatations is unknown. One of the theories is that the extrahepatic bile ducts and the pancreatic duct form a common duct over a short distance (which is not normally the case). This could allow secretions from the pancreas to enter the bile ducts and weaken their structure. A build-up of bile would then lead to increased pressure in the bile ducts, their expansion and the formation of cysts*. However, this explanation is very speculative, more of interest to scientists and has no influence on your child's treatment.

Cyst: Greek kystis "bladder" = a tissue cavity closed by an epithelium (membrane)

The diagnosis and surgical treatment of biliary tract and liver diseases in children is a specialization of the Hannover Paediatric Surgery Centre, which is a reference center for paediatric liver and biliary tract diseases. Each new case is examined in close cooperation with the departments that specialize in particular diagnostic or therapeutic measures. These include, for example, the (pediatric) gastroenterologists. Together, the necessary examinations are planned for each patient and an individual treatment plan is drawn up. Thanks to the special focus of our center on this disease, every patient can be treated to a very high standard.

In most cases, the choledochal cyst is reliably diagnosed by ultrasound. Occasionally, the suspected diagnosis is made during prenatal screening. If your child has no other symptoms, we can plan a timely operation together with you. However, if there is any doubt about the correct diagnosis, open questions can be answered with an MRI (under general anesthesia for small children and under sedation (with a "mild sedative") for older children (Fig. 1). Alternatively or additionally, the findings can also be confirmed by endoscopy** (always under general anesthesia). Our center has small endoscopes so that this examination is also routine in our clinic for newborns (Fig. 2).

** with a flexible device that is inserted into the intestine via the mouth and can be used to examine the inside of the intestine (including the opening of the bile duct into the duodenum).

 

 

Choledochal cysts are always treated surgically. The choledochal cyst itself and the affected bile ducts must always be completely removed, as any remaining tissue can degenerate malignantly later in life. As every choledochal cyst carries a high risk of generalized inflammation of the bile ducts (cholangitis), surgery should be planned as early as possible (before the age of 6 months), even in asymptomatic children. This is because any inflammatory process taking place in this tissue makes the operation more difficult and increases the surgical risk. The procedure can be performed open or minimally invasively ("laparoscopically"). The principle of the operation is the same in both cases. The altered bile ducts, which run from the liver to the duodenum, are completely removed, including the gallbladder. In order to drain the bile from the liver back into the digestive tract, a loop of small intestine is connected to the remaining bile ducts (Fig. 3). After the operation, the children can eat normally and are not restricted in terms of their development and quality of life. Possible late effects include an increased risk of developing gallstones in young adulthood.

We will discuss the advantages and disadvantages of each procedure and which surgical method is best in your individual case in a detailed information and explanatory discussion.

Patients usually spend the first night after the operation in the so-called "intermediate care area" ("mixture" of normal ward and intensive care unit) and return to the normal ward the next day. The diet is usually started on the first or second day after the operation and is primarily based on your child's condition and appetite. The same applies to the length of the hospital stay. If the course of the operation is uncomplicated and your child is well again, nothing stands in the way of discharge and outpatient follow-up care.

Follow-up care should always be carried out in close cooperation between our Clinical Department and the treating pediatrician. The liver values should be checked and the duration of the antibiotic infection prophylaxis should be adjusted individually for each patient. We do not recommend dietary restrictions or a special diet. However, long-term monitoring of patients is necessary in any case, as there is an increased risk of gallstones forming. These should be recognized and treated before they cause clinical problems. In addition, there is a slightly increased lifelong risk of developing a malignant disease of the liver or bile ducts in the area of the operated area, so that we recommend annual check-ups until late adulthood. This can also be carried out close to home by (pediatric) gastroenterologists.