Pancreas Anulare: Causes, Symptoms & Treatment

Pancreas anulare is a malformation of the pancreas (medical name pancreas). The condition is characterized by incomplete fusion of the anterior and posterior parts of the pancreas. Pancreas anulare is a very rare condition.

What is pancreas anulare?

In pancreas anulare disease, the pancreas encircles the duodenum in a ring shape. A special part of the pancreas includes the duodenum adjacent to the upper end. This results in a defective organ location of the duodenum. Depending on the extent of compression, significant obstructions to the passage of the pancreas may occur. Sometimes pancreas anulare is not considered a malformation but, depending on its extent, a normal variant. Depending on the severity of the resulting passage obstruction, the malformation may appear in the womb or in the affected newborn. In addition, pancreas anulare may not appear until the third or fourth decade of life or may remain completely asymptomatic.

Causes

The causes of pancreas anulare disease lie in malformations during the development of the embryo in the womb. A certain part of the pancreas arises from the duodenum. Due to different growth rates during the rotation of the duodenum, the excretory duct of the pancreas merges with a special part of the bile ducts (medical name choledochal duct). As the process continues, the production of hormones begins, although the glandular part remains without function for the time being during the fetal period. In medical research, it is believed that the hormone insulin plays a significant role in growth regulation. This is where the formation of the pancreas anulare comes in. As part of the formation of the anterior pancreas, two lobes may form with an orientation toward the anterior side. With additional development toward the posterior side and fixation of the posterior part, formation of a ring around a specific part of the duodenum may occur. This may narrow its lumen and give rise to pancreas anulare.

Symptoms, complaints, and signs

Various symptoms may occur in the course of pancreas anulare, depending on the manifestation of the disease and its severity. It is possible for pancreas anulare disease to be completely asymptomatic. In this case, there are no symptoms at all, which means that the affected persons do not notice the disease, so that it cannot be diagnosed and treated. In the event that particularly severe pressure is exerted on the duodenum in the course of pancreas anulare, vomiting after ingestion of food is the most common symptom of the disease. Bilious vomiting may also occur in this context. In general, asymptomatic forms of pancreas anulare may become symptomatic during life. As a result, pancreas anulare is not discovered until adulthood in some cases.

Diagnosis and disease progression

Various methods of investigation are available for the diagnosis of pancreas anulare, the use of which is decided by the attending physician depending on the symptoms shown and the severity of the disease. By means of prenatal diagnostics, the double-bubble phenomenon can give an important indication of the presence of pancreas anulare. In the case of clinical suspicion, sonography of the abdomen is usually performed, as well as a plain abdominal radiograph. Sonography can also be used to perform a differential diagnosis. This is particularly relevant in view of the fact that pancreas anulare may show symptoms similar to those of other, sometimes more serious diseases. In the context of this differentiation, hypertrophic pyloric stenosis and duodenal atresia must be excluded first and foremost. Functional images are taken, and in addition, if pancreas anulare is present, the so-called double-bubble phenomenon is seen in the abdominal empty image. To differentiate from functionally relevant stenosis, the gastrointestinal passage can be examined with special water-soluble contrast agents. Other options for the diagnosis of pancreas anulare include the performance of special computed tomography scans.

Complications

A wide variety of complications and symptoms can occur with this disease.In some cases, however, the disease runs completely without symptoms, so that sometimes no treatment is necessary. However, the disease can lead to discomfort when taking food. Patients often suffer from nausea and vomiting. In many cases, it cannot be properly diagnosed where these complaints come from, so that direct treatment is not possible in the first place. Persistent vomiting can lead to severe psychological symptoms and depression, and can significantly reduce the quality of life of those affected. Children can also suffer from developmental disorders if the disease is not treated properly. As a rule, the malformation in pancreas anulare can be removed relatively well with the help of a surgical procedure. Complications usually do not occur. However, scars or fistulas may still occur. It is also possible that the patient will require treatment again at a later date. The life expectancy of the affected person is not affected if the treatment is successful.

When should you see a doctor?

In a large number of patients, pancreas anulare does not present with any symptoms for the rest of their lives. Therefore, the malformation often goes unnoticed and is not diagnosed. A visit to the doctor is not necessary. If there are no symptoms at all, a check-up is not necessary. Newborns who show abnormalities in feeding must be examined by a doctor. If the fed breast milk or infant formula is immediately vomited, a doctor must be consulted. If there is weight loss, apathy, or flaccid muscles, action is needed. If there is no body tension, this should be considered a warning sign. Normally, care is provided by medically trained staff during the first few days after delivery. Tips and advice are given on the correct handling of the newborn. If irregularities become apparent, the nursing staff or nurses in most cases take over the organization of further steps. A doctor is asked by them for help and a thorough examination of the infant is initiated. If discoloration is observed in the vomit, this information is to be forwarded to the attending physician. If the irregularities appear in the further development and growth process of the child, a visit to the doctor is also necessary. Underweight, deficiency symptoms, an internal weakness as well as apathy must be presented to a doctor. A feeling of pressure inside the body is another sign of a health disorder.

Treatment and therapy

Only a limited selection of therapeutic methods is available for the treatment of pancreas anulare. In the event that symptomatic forms of pancreas anulare disease are present, affected patients undergo surgery. During the surgical procedure, a bypass is placed in the affected stenosed bowel segment. The operation is usually performed under general anesthesia. Another therapeutic measure is to split the laced ring of the pancreas. However, this method is outdated because it involves various risks. These include, for example, fistula formation, gait injury, and scar shrinkage, which may result in recompression.

Outlook and prognosis

The prognosis of pancreatic anulare should be evaluated according to the individual characteristics of the health disorder. Basically, it is a congenital disease, the intensity of which is documented differently in each affected individual, and the outlook is favorable. There are a large number of patients who are symptom-free for life. Nevertheless, they should be examined by a specialist at regular intervals so that immediate action can be taken in the event of changes. In most cases, surgery is recommended. The aim of this is to achieve lifelong freedom from symptoms. If the operation proceeds without further complications, the patient is usually discharged from treatment after a few weeks as recovered. It is also possible that the course of the disease is determined by permanent medication. This depends on the health condition of the patient. Further diseases may develop as a result of the symptoms. The risk of an additional psychological disorder is increased in affected patients. The reason for this is the emotional burden of the disease and its symptoms.There may be a severe restriction of the quality of life. A relapse of the complaints is not to be expected after a surgical intervention. Nevertheless, secondary diseases can occur if not everything went optimally during the treatment. In the event of undesirable scarring, it should be checked whether the use of further therapies is indicated.

Prevention

In most cases, pancreas anulare disease forms in the embryo. For this reason, no effective methods exist to prevent the disease. It is of all the more relevance to consult a physician at the first signs and symptoms of pancreas anulare and to have further diagnostic investigations initiated. If the disease already appears in childhood, an immediate medical examination is required. If pancreas anulare is asymptomatic and symptoms do not develop until adulthood, these should also be clarified for differential diagnosis. If pancreas anulare is diagnosed, the attending physician will give the affected patient individualized advice, for example, regarding the choice of food, in order to positively influence the further course of the disease and prevent complications.

Follow-up

In most cases of pancreas anulare, only limited and also only few measures of aftercare are available to the affected person. For this reason, the affected person should ideally see a doctor at the first symptoms and signs of this disease, so that it does not lead to complications or other complaints in the affected person in the further course. The sooner a doctor is consulted, the better is usually the further course of the disease, so that a doctor should be contacted at the first signs and symptoms of the disease. Most of the affected persons have to undergo a surgical intervention. After such an operation, the patient should rest and take care of his body, keeping a strict bed rest and avoiding stressful activities. In general, regular check-ups by a doctor are also very important after the operation in order to detect and treat further damage at an early stage. Further measures of an aftercare are not available to the affected person in case of pancreas anulare. In most cases, this disease does not reduce the life expectancy of the affected person.

Here’s what you can do yourself

Because pancreas anulare can be quite individual, the symptoms of affected patients are also quite different. They may have no problems, while in others only surgical intervention will help. If vomiting is one of the symptoms, it is important for patients to eat particularly conscientiously to avoid deficiencies. This is especially true for children, whose development could otherwise be disturbed. Several small meals a day are recommended, which should be easily digestible, but at the same time rich in vitamins, minerals and fiber. A conscious diet not only has a positive influence on the symptoms, but at the same time can also compensate for any existing tendency to depression. This is because constant nausea and vomiting trigger feelings of helplessness and powerlessness in some sufferers. Patients are also advised to attend psychotherapeutic sessions. But also various relaxation techniques help to live with this rare disease. These include gentle sports such as yoga, qigong and tai chi. Reiki, music therapies or the tapping therapy EFT have also proven to be relieving. Easy to learn and very effective is Progressive Muscle Relaxation according to Jacobson, which is even taught in adult education centers. Meditations and breathing exercises are also good ways to relieve stress caused by illness. Sports, especially outdoor exercise, also provide balance.