A choledochal cyst represents a cyst-like enlargement of the bile ducts. Its cause is largely unknown. Early treatment is necessary because of complications that occur later.
What is a choledochal cyst?
A choledochal cyst manifests as a cyst-like dilatation of the bile ducts. In this case, the bile ducts represent canal-like structures that transport bile from the liver to the duodenum. There are intrahepatic and extrahepatic bile ducts. While intrahepatic bile ducts are located within the liver, extrahepatic bile ducts represent bile transport outside the liver. In the broadest sense, this also includes the gallbladder. A choledochal cyst describes the extrahepatic dilatation in the bile ducts between the liver and duodenum. The cystic dilations can occur in different forms. Thus, there may be localized or diffuse dilatations of the bile duct. However, there are also diverticular cysts. These consist of outpouchings of the choledochal wall. Some cystic outpouchings protrude into the duodenum. The entire choledochal duct may also be dilated. Usually, cysts have been present since birth. Often they are discovered only by chance. However, there are also cases in which the cysts develop only over time. Overall, choledochal cysts occur very rarely. Thus, the probability of their occurrence is between 1:50,000 and 1:150,000. A choledochal cyst always carries the risk of developing a complication.
Causes
The causes of a choledochal cyst are largely unknown. However, because the cyst has usually been present since early childhood, a genetic cause is suspected. Autosomal recessive malformations are thought to be involved. Secondary causes for cyst formation are also discussed. For example, due to a malformation of the excretory duct of the pancreas, in rare exceptional cases this duct forms a common channel with the bile duct for a short distance. Via this common connection, the digestive enzymes of the pancreas partially flow into the ductus choledochus. This results in self-digestion of the bile duct walls. Due to the resulting weakness of the bile duct walls, they can no longer withstand the pressure of the accumulating bile. As a result, they dilate like cysts. However, this explanation is based only on conjecture, which has yet to be proven. However, the cysts apparently arise because of congenital malformations, but these are not uniform.
Symptoms, complaints, and signs
Depending on the extent of the choledochal cyst, there are either no symptoms or abdominal pain, yellowing of the skin, and pale stools occur in early childhood. Bile is stagnated, allowing it to enter the blood. This causes posthepatic jaundice to develop. When the yellow pigment bilirubin then leaks from the blood into the body organs, there is yellowing of the skin. The bile pigment no longer reaches the intestines, so stools look pale. Since the dietary fats can no longer be emulsified by the bile, they are excreted with the stool in the form of so-called fatty stools. Furthermore, patients also suffer from constant flatulence because the pancreatic digestive enzymes no longer work effectively. If the choledochal cyst persists for a long time, there is a risk of developing recurrent gallstones, bilious [peritonitis|peritonitis]] or cholangiocarcinoma. Peritonitis may be caused by rupture of the weakened walls of the bile duct. Chronic inflammatory processes also take place due to the action of the pancreatic digestive juices, which increase the risk of carcinoma.
Diagnosis
Often, a choledochal cyst is detected as an incidental finding during sonographic examinations during pregnancy. However, cysts usually become apparent after pregnancy. Again, they are either incidental findings on sonography or investigative findings on a detailed examination for chronic abdominal symptoms of the child with jaundice. MRI or endoscopy may also be performed to clarify the diagnosis.
Complications
The complications that can occur as a result of a choledochal cyst vary widely. In most cases, there is relatively severe abdominal pain and jaundice. The stools also turn yellowish and are light in color.The patient’s quality of life is greatly reduced by the persistent abdominal pain. Even physically difficult work is hardly possible due to the pain. The patient also suffers from severe flatulence, which can have a negative effect on social contacts and also on the patient’s self-esteem. Often, this also leads to the formation of gallstones or inflammation in the pancreas. If the choledochal cyst is not treated properly and early, life expectancy is usually reduced. In many cases, the choledochal cyst reappears in the patient even after treatment and therefore needs to be removed one more time. The treatment itself aims at the complete removal of the cyst. The removal is performed by means of a surgical procedure. Complications may arise due to certain inflammations before and after the surgery. If the choledochal cyst is diagnosed in children and removed immediately, there are usually no restrictions on physical and psychological development.
When should you go to the doctor?
If the child complains of increased abdominal pain and problems with bowel movements, this is a reason to see a doctor. In addition, if the characteristic yellowing of the skin is noticed, it is best to go directly to the nearest hospital. Gallstones and symptoms of peritonitis indicate that the choledochal cyst has been present for some time. Any complaints should therefore be immediately clarified medically. If the cyst remains untreated, there is a risk of biliary cancer – a medical emergency that must be treated in the hospital. If pancreatitis or a rupture has already occurred, a doctor must also be consulted immediately. A choledochal cyst usually has genetic causes. So, in the best case, a physical examination is performed during childhood, which reveals the disease. Diseases of the pancreas and gallbladder are suspected to favor the development of a choledochal cyst. Anyone who has been or is affected by such a condition should talk to their family doctor if they have the symptoms mentioned. Other contacts are the gastroenterologist or a specialist in internal medicine.
Treatment and therapy
Because of the many complications of choledochal cyst, early therapy is absolutely necessary. For example, studies have shown that rupture can occur in 20 percent of all cases. Another 30 percent of patients develop pancreatitis. In addition, the risk of malignant degeneration is very high. Again, as early as 30 to 35 years of age, 20 to 30 percent of those affected develop choledochal cyst carcinoma. In the past, permanent cyst drainage was performed, but this is no longer recommended today due to the complications that occur. The most important goal of therapy today is the complete removal of the cyst. In doing so, the pancreatic duct must be spared. The operation should be performed as early as possible because the risk of surgical complications increases with age. Any inflammatory process contributes to increase the risk of surgery later. Surgery can be performed either open or minimally invasive. During surgery, the dilated bile ducts are removed and the remaining bile ducts are connected with a loop of small intestine. After the surgery, the children’s development is no longer restricted. They can also be fed normally. Only for adulthood, there is still a higher risk of developing gallstones.
Outlook and prognosis
The choledochal cyst has a good prognosis for most patients. With early diagnosis as well as treatment, the cyst can be completely removed and the patient remains permanently symptom-free. Without treatment, patients experience various complications as the disease progresses. The general state of health is significantly affected. The probability of developing a choledochal cyst carcinoma is 30% of all patients. Since the malignant tumor can lead to a life-threatening condition of the patient, prompt medical attention immediately after diagnosis is life-saving. The choledochal cyst is removed in a routine surgical procedure within a short period of time. If the operation proceeds without obstacles, the patient is usually discharged from treatment after a few days.The risk of recurrence of the choledochal cyst is minimal. Similarly, hepatic parenchymal damage does not subsequently occur with cysts. With good wound care, no further impairment is to be expected. The markedly good healing prospects worsen in people with a coagulation disorder or poor health. In addition to early treatment, high-risk patients are at increased risk of complications during the surgical procedure. The recovery path is additionally delayed in these sufferers. If recovery occurs, it is also permanent in the high-risk patients.
Prevention
No recommendation can be made for prophylaxis of a choledochal cyst. In most cases, the condition is congenital without knowing the exact cause. Even in cases where symptoms develop later, there is usually an unknown malformation of the pancreatic duct associated with the choledochal duct. Once a choledochal cyst has been diagnosed, it should be surgically removed as soon as possible to prevent serious complications.
Follow-up
After surgical treatment of a choledochal cyst, follow-up care begins and usually takes a fairly short time. Patients are usually allowed to return home after only a few days. In general, the risk of recurrence of the disease is very low. Consequential damage is also not to be expected following the operation. If the wound is well taken care of and heals relatively quickly, there are no adverse effects for the patients. However, it is helpful to change one’s diet to counteract the chronic stomach pain and other complaints. In particular, heavy meals and foods that irritate the mucous membranes should be avoided as part of an individual diet. Luxury foods could also promote the complaints. That is why it is advisable to avoid coffee, alcohol and cigarettes. Natural remedies, for example St. John’s wort, chamomile and lemon balm, are suitable as support for a healthy gastrointestinal tract. These remedies are mainly available as tea. Resting and taking it easy will also make patients feel better. Special relaxation exercises or massage sessions are particularly useful for people who have suffered from the disease since childhood. Follow-up examinations also play an important role in optimizing the medication regimen.
What you can do yourself
Depending on the type and severity of the choledochal cyst, various symptoms may occur that require medical treatment. Some of the typical signs of the disease can be treated by the affected person himself. A change in diet helps against chronic abdominal pain and other gastrointestinal complaints. Those affected should avoid heavy and irritating foods and drinks. Also stimulants such as alcohol, coffee and cigarettes should be avoided. In addition, various natural remedies can be used, such as the pain-relieving St. John’s wort or various teas with chamomile or lemon balm. Diet also helps against jaundice. In addition, attention should be paid to sufficient rest and sparing. Patients who have suffered from choledochal cyst since early childhood should consider pain therapy, accompanied by relaxation exercises and massage. Since the condition is long-lasting and can cause various secondary symptoms, close medical monitoring is also always necessary. Affected persons should ensure that their medication is optimally adjusted and that there are no other illnesses or complaints that could interact with the prescribed drugs. Finally, a healthy lifestyle with sufficient exercise and the avoidance of stress is recommended. In combination with an individual diet and comprehensive drug treatment, a choledochal cyst can be treated well.
