Duodenal stenosis is a narrowing of the small intestine. It is usually congenital, but it can also be acquired.
What is duodenal stenosis?
A stenosis is a narrowing of a hollow organ. In duodenal stenosis, the small intestine, or more specifically the duodenum, is affected by the stenosis. Duodenal stenosis is also often referred to as duodenal atresia, although atresia is only one form of stenosis. Anatomically, the stenosis is subdivided with respect to its location to the papilla duodeni major. In high obstruction, the obstruction is located above the papilla of Vater. The Vater’s papilla (also known as the papilla duodeni major) is located just above the junction of the common bile duct and the pancreatic duct with the duodenum. A stenosis that lies below the papilla paternale is called a deep obstruction. The congenital form of duodenal stenosis occurs in an average of one in 7000 children. Duodenal stenosis is often combined with other malformations. One third of all children with congenital duodenal stenosis also have trisomy 21. Trisomy 21 is also known as Down syndrome.
Causes
Duodenal stenosis can be congenital or acquired. External stenosis is usually caused by compression. The most common cause is malrotation. Malrotation is a disturbance in the rotation of the large and small intestines during embryonic development. The positional anomaly results in compression of the duodenum by the colon. Pancreas anulare can also cause duodenal stenosis. This is a malposition of the pancreas. The dorsal and ventral portions of the pancreas are not completely fused, forming two lobes. This creates a ring around the small intestine. This ring can narrow the lumen of the duodenum. Depending on the extent of the obstruction, the maldevelopment is already noticeable in the womb. Mild narrowing may also not be noticed until the third or fourth decade of life. Another cause of duodenal stenosis is Ladd syndrome. This is a congenital volvulus, a twisting of a section of the digestive tract. Furthermore, brides can narrow the duodenum. Brides are cicatricial strands located at the level of the abdomen. Generally, brides are also called adhesions. They usually develop after surgery. Less commonly, duodenal stenosis is caused by atypical blood vessels. For example, the portal vein may atypically run directly in front of the duodenum. Superior vena mesenteric syndrome is another example of such an anomaly. Other external causes of stenosis include intestinal duplications or diverticula. In intestinal duplication, a portion of intestine is duplicated in the mesentery. Diverticula are sac-like protrusions of the bowel wall. Internal stenoses are caused by a tubular narrow segment. Annular strictures or connective tissue changes of the intestinal wall may also have a slowly increasing stenotic effect. In duodenal atresia, the lumen of the small intestine is absent or inadequately developed because of a malformation of the duodenal canal.
Symptoms, complaints, and signs
Symptoms of duodenal stenosis depend on the extent of the disorder. Mild duodenal stenosis may go unnoticed for a lifetime. Intrauterine polyhydramnios may occur during pregnancy. Severe duodenal stenosis is manifested by massive vomiting in the first days of life. The upper abdomen of affected infants is distended, whereas the lower abdomen is retracted. Periods without symptoms are also possible. The diagnosis may even not be made until school age or adulthood. When the stenosis is located below the papilla of Vater, patients vomit bile. Green vomiting is a typical symptom of duodenal stenosis. Often, the stenosis is also associated with esophageal atresia or anal atresia. It is not uncommon to find a heart defect in addition to the stenosis. The children are also frequently affected by Down syndrome.
Diagnosis and course
In severe duodenal stenosis, the suspected diagnosis may be made on the basis of symptoms alone. If an ultrasound examination of the child’s abdomen in the womb is abnormal, the diagnosis can be confirmed by X-ray after birth. In the ultrasound examination, the so-called double-bubble phenomenon becomes visible. The child’s stomach is filled with fluid and forms the first bubble.The duodenum also contains fluid and thus appears as a second bubble. Since the stomach and duodenum are next to each other in the image, a double-bubble is seen. The same phenomenon is seen in the X-ray image. The upper gastrointestinal tract fills with air after birth. One air bubble forms in the stomach, and the second bubble forms in the duodenum. Since the rest of the gastrointestinal tract is empty, the double-bubble phenomenon is also seen here.
Complications
Duodenal stenosis does not necessarily result in complications. If it is only a very mild manifestation of duodenal stenosis, it may not be noticed by the patient at all. In this case, there are no complaints, limitations or complications. Life is then not affected by duodenal stenosis and life expectancy is not reduced. If duodenal stenosis is more widespread, in many cases there is vomiting and abdominal pain. In many cases, the disease also causes a heart defect. Very often, especially people with Down syndrome are affected by duodenal stenosis, which further limits their lives. As a rule, treatment is possible and in many cases successful. There are no particular complications. Often, the treatment is carried out already in childhood. However, if the child is also affected by other malformations, duodenal stenosis may not be easily treated. The further complications depend strongly on the syndrome with which the child is born. Unfortunately, it is not possible to prevent the symptom.
When should you go to the doctor?
In the case of duodenal stenosis, it is not necessary to see a doctor in every case. If the disorder itself does not cause discomfort or other uncomfortable feelings, there is no need for medical treatment. However, a doctor should be consulted if the affected person suffers from severe and, above all, persistent vomiting and also nausea. A distended abdomen may also indicate duodenal stenosis and should be examined. Especially in case of vomiting of bile, a doctor should be consulted immediately. In many cases, the condition is also associated with a heart defect, so a cardiologist should also be consulted when duodenal stenosis is diagnosed. As a rule, the affected person must then participate in regular examinations to prevent further symptoms. The disease itself can be diagnosed by an internist. In an emergency, an emergency doctor can also be called or the hospital visited. The disease can be treated relatively easily and the affected person does not suffer from a reduced life expectancy.
Treatment and therapy
If duodenal stenosis with a membrane is present, treatment is endoscopic if necessary. Otherwise, surgical intervention is used. One possible procedure is duodenoduodenostomy. Duodenojejunostomy can also be performed. In this procedure, part of the small intestine is removed and the remnants of the duodenum are surgically united with the jejunum. Depending on the extent of the stenosis, a membrane excision may also be performed. The prognosis depends on several factors. First, the severity of the stenosis plays a role. Also, the birth weight and the severity of additional malformations or deformities have a decisive influence on the prognosis. Children with a birth weight of less than 2000 grams or children with a birth weight between 2000 and 2500 grams and with severe malformations have the worst chance of complete recovery.
Outlook and prognosis
Patients with duodenal stenosis usually have a good prognosis. In mild cases, no treatment measures need to be taken. Food intake is adjusted to the needs of the organism so that there are no complications during bowel movements. If symptoms occur, treatment should be carried out so that no consequential damage or life-threatening situations occur. In a surgical procedure, the congenital or acquired narrowing of the small intestine is corrected. After a subsequent wound healing, the patient can be discharged from the treatment free of symptoms. The functional capability of the intestine is permanently restored by the correction. No further measures need to be taken. The healing process depends on the extent of the stenosis. The more extensive the procedure, the longer the recovery. If the wound is not treated optimally, the risk of disease increases.In severe cases, sepsis occurs. In the case of congenital duodenal stenosis, infants with a low birth weight below 2,000 grams in particular have an unfavorable prognosis. The corrective procedure is often not well tolerated by their organism and causes excessive stress. In addition, the prospect of complete recovery is diminished in patients with particularly severe malformations. It is often not possible for doctors to correct all abnormalities. Malformations remain, and they continue to cause discomfort.
Prevention
Duodenal stenosis is congenital in most cases. The exact mechanisms of development are unknown. Thus, prevention of the disease is not possible.
Follow-up
First and foremost, duodenal stenosis always requires medical examination and treatment. There are also only very limited options for aftercare available to the affected person, so that an early diagnosis is very important in the first place. The earlier duodenal stenosis is detected, the better the further course of the disease usually is. A doctor must therefore be consulted at the first sign of the disease. The treatment of the disease is a surgical procedure in which part of the intestine is removed. After this operation, the patient should in any case remain on bed rest and not exert himself unnecessarily. Stressful or physical activities should also be avoided in order not to slow down the healing process. Furthermore, heavy food should not be consumed in order not to overload the stomach and intestines. Patients are dependent on a light and gentle diet, and normal food intake can only be carried out after a successful recovery. However, there is not always a complete cure for duodenal stenosis. In many cases, parents are therefore dependent on psychological treatment, although discussions with friends and with the family can also be very useful.
What you can do yourself
Duodenal stenosis is usually caused by a genetic malformation of the small intestine and is associated with Down syndrome (trisomy 21) in about 30 percent of cases. In less common cases, the narrowing of the duodenum can also be acquired by accident or after surgery. Depending on the type of malposition, the passage of intestinal contents may be only slightly or considerably impeded. The symptoms vary accordingly. They range from not noticeable to frequent severe vomiting and severe abdominal pain already in the first days of life. In principle, this also applies to duodenal stenosis acquired at a later age. In the case of only minor symptoms that do not necessitate immediate surgical intervention, a change to an easily digestible diet with a balanced proportion of indigestible dietary fiber is recommended as an adjustment to everyday life and as a self-help measure. The easily digestible diet supports a rapid intestinal passage, so that the narrowing of the small intestine remains asymptomatic and no further restrictions in everyday life are to be expected. In addition, the food mush that passes directly from the stomach into the small intestine can be diluted a little by a sufficient amount of water, which also supports the speedy passage of the constriction in the small intestine. In cases of severe symptoms, self-help measures are not sufficient. In these cases-especially in newborns-surgical intervention is usually required to prevent secondary damage, some of which may be life-threatening.
