Tracheoesophageal fistula connects the trachea to the esophagus, causing symptoms such as coughing fits and food aspiration. The phenomenon is usually congenital and in this case is usually associated with malformation of the trachea and esophagus. Treatment is surgical.
What is a tracheoesophageal fistula?
Fistulas are tubular connections between hollow organs or the body surface and an organ. These connections correspond to pathologic ducts that are surrounded by or lined with tissue. In principle, fistulas can form in a wide variety of locations. The tracheoesophageal fistula corresponds to a fistula connection between the trachea and esophagus, i.e., between the trachea and esophagus. Between these two structures, fistula connections may be present in congenital or acquired form. Depending on the anatomical course, medicine distinguishes between different forms of tracheoesophageal fistula. One of them is the tracheoesophageal fistula at the blind sac of esophageal atresia, which leads into the trachea and causes constant swallowing. H-fistulas, on the other hand, are called connections between the esophagus and trachea that do not affect the passage of the esophagus. Depending on the width, this congenital fistula at best leads to aspiration of fluid during drinking.A tracheoesophageal fistula of the tracheal system leading into the lower blind sac of esophageal atresia is usually associated with reflux of gastric contents and usually causes the most severe symptoms.
Causes
Fistulas between the esophagus and trachea, like all other fistulas, often occur after surgical complications. Infiltrative growth of malignant tumors may also be involved in its development. In principle, acquired fistula tracts between the esophagus and trachea are a rather rare phenomenon. In less than one percent of those affected, a previous tracheostomy is the cause of fistula tract formation. In about five percent of cases, fistula tract formation is preceded by malignant esophageal tumors. Less than one percent of patients suffer from primary lung tumors. The causes mentioned so far refer exclusively to the acquired form of tracheoesophageal fistula. Depending on the cause, the acquired forms show a clinically diverse symptom picture. In the vast majority of all cases, tracheoesophageal fistulas are congenital. Such congenital anomalies are usually related to malformations of the esophagus or trachea and occur rather rarely as isolated phenomena. A congenital fistula between the esophagus and trachea is present in about one or two newborns among 2000 to 4000 live births. The additional malformations affect up to 70 percent of patients. In the context of higher-level syndromes, the fistulas are part of Feingold syndrome or beta-blocker embryopathy, for example.
Symptoms, complaints, and signs
Patients with a tracheoesophageal fistula present with different symptoms depending on the location and cause of the fistula formation. For causes such as esophageal atresia, clinical symptoms are determined by the atresia. If the fistula tract corresponds to an isolated fistula, cough attacks occur as leading symptoms, which are associated with chronic recurrent aspiration pneumonia and meteorism. Patients thus suffer from inflammatory reactions of the lungs as gastric contents reach the lungs via their trachea. In addition, sufferers often exhibit high amounts of air in the digestive tract because the connection between the esophagus and trachea promotes air swallowing. In addition to these symptoms, fistula tracts between the two anatomical structures may also manifest in refusal to drink and blue discoloration during an attempt to drink. Upper lobe atelectasis may occur in addition to recurrent aspiration of fluid and food.
Diagnosis and Course of the Disease
The diagnosis of a tracheoesophageal fistula is made by imaging. In congenital fistulas in this location, the physician usually initiates imaging in response to refusal to drink or constant coughing episodes. H fistulas are localized starting from the trachea especially from level HWK six to BWK two. Thus, these types of fistulas are much higher than fistulas of esophageal atresia. In all cases, the fistula is detected by means of radiographs, which are performed under fluoroscopy with contrast medium.The exact localization determines the classification into one of the subtypes. The prognosis of patients with tracheoesophageal fistulas depends on the exact fistula location and the primary cause of the duct formation.
Complications
First and foremost, those affected with this condition suffer from a very severe and especially uncomfortable cough. This results in coughing attacks, which can significantly restrict the daily life of the affected person. Inflammations and infections in the lungs also occur and have a negative effect on the patient’s quality of life. Many sufferers swallow, which causes the air to enter the digestive tract. This leads to flatulence and bloating. In the worst case, aspiration can also lead to the death of the patient. Especially in children, swallowing can be fatal. Since there is no self-healing in this disease, patients are always dependent on treatment by a physician. As a rule, the treatment can be carried out by a surgical intervention. This takes place without complications and relieves the discomfort enormously. No further discomfort occurs during this procedure. Inflammations and infections are treated with the help of medication. As a rule, successful treatment does not reduce the patient’s life expectancy. However, surgical treatment takes place only after the inflammations have been treated.
When should you go to the doctor?
If there are repeated episodes of coughing or an increase in coughing, clarification of the cause should take place. These are warning signals of the organism, the cause of which should be determined. If food repeatedly enters the trachea, if the affected person frequently swallows, or if involuntary vomiting occurs, a medical examination is needed. Refusal to eat and intake of fluids are considered worrisome. A physician must be consulted as a life-threatening condition may result. An increased body temperature, inner restlessness as well as irritability indicate a health impairment. If breathing noises, problems with air supply or anxiety occur, a doctor must be consulted. Disturbances of the digestive tract, flatulence or swelling in the abdomen are further signs of a present illness. If the affected person suffers from swallowing air, a decrease in physical resilience as well as sleep disturbances, extensive medical examinations should be initiated. Medical attention is indicated to prevent further impairment of quality of life. In acute situations, there is a danger to life. Therefore, emergency medical services should be alerted if respiratory distress, a state of unconsciousness or a panic attack occurs. The affected person is threatened with premature death by suffocation. Bystanders must administer first aid in these cases.
Treatment and therapy
Treatment of a tracheoesophageal fistula depends on the primary cause. Symptomatic treatment of the fistula itself is equivalent to invasive surgery. During this procedure, the fistula tract is obstructed. The connection between the esophagus and trachea is surgically severed and the two systems are made completely separate. In addition to the actual treatment of the fistula, causal therapy is performed to address the root cause. In esophageal atresia, this causal therapy corresponds to surgery, prior to which the accumulated secretions are drained through a tube. Surgical correction consists of removal of the upper part of the esophagus. The loose esophageal parts are joined together after removal. If there is too much distance between the individual parts, an alternative therapy is performed. This therapy usually corresponds to a lengthening treatment of the esophagus, which lasts for several days or even weeks. After the lengthening treatment, the distance between the two parts is ideally short enough to connect the ends. If the lengthening treatment does not produce an adequate result, the surgeon relocates stomach or intestinal parts to the chest area to replace the missing esophageal piece. The existing connections to the trachea or lungs are cut and tightly closed. All treatments for tracheoesophageal fistula can take place only when there is no longer active pneumonia.
Prevention
Tracheoesophageal fistula can be prevented only to the extent that estrophaguatresia and other primary causes can be prevented.
Follow-up
After successful treatment of a noncongenital tracheoesophageal fistula, regular follow-up examinations are necessary because the likelihood of recurrence of a tracheoesophageal fistula is increased in patients who have already had it. For this purpose, regular X-ray and, if necessary, MRI examinations of the esophagus and trachea should be performed. In addition, a physician should be consulted immediately if severe heartburn, reflux (the regurgitation of stomach contents), frequent swallowing, especially when drinking, or breathing problems occur, as these may be signs of the recurrence of a tracheoesophageal fistula. If the fistula occurred as a complication of surgery, no further follow-up is necessary beyond these checks. If a tumor was the cause of the fistula development, it is important to additionally check the blood for tumor markers on a regular basis in order to detect the recurrence of a tumor at an early stage. If the tracheoesophageal fistula was congenital, regular examinations of the esophagus and trachea should also take place during the development of the affected child, since in rare cases the fistula can form again during adolescence. Furthermore, in the case of a congenital tracheoesophageal fistula, any underlying genetic diseases that may lead to malformations (Feingold syndrome, VACTERL association) should be treated for life. Appropriate therapies should be discussed individually with the treating physician.
What you can do yourself
A tracheoesophageal fistula must be surgically removed. The patient can support the treatment with some measures and remedies from home and nature. First, the wound must be carefully cared for and observed after surgery so that complications can be detected early. If pain, bleeding or other unusual symptoms occur, the doctor must be informed. In addition, if there are signs of recurrence, the physician should be consulted. The physician can suggest suitable preparations or, in severe cases, prescribe prescription ointments. Accompanying this, the cause of the development of the fistula must be determined. If the fistula has occurred after an operation, comprehensive tumor screening is also necessary in any case. Especially in the case of malignant esophageal tumors and primary lung tumors, the risk of tumor disease is relatively high. Lastly, rest and sparing apply, as the operation and removal of the fistula place a greater strain on the body. The guideline of the German Society for Pediatric Surgery provides affected patients with further tips and information with which tracheoesophageal fistula can now be treated well.
