Tracheoesophageal fistula is a pathological fistula connecting the lumen of the windpipe with the esophagus. It may be a developmental anomaly or an acquired pathology. Disease is manifested by coughing attacks during meals, which are accompanied by suffocation, cyanosis, and the release of foamy sputum with pieces of food. Aspiration pneumonia often develops. Confirmatory diagnosis is carried out using endoscopic methods (esophagoscopy, tracheobronchoscopy), radiography of the esophagus. Treatment is exclusively operative – closure of the tracheoesophageal fistula, often with simultaneous tracheoplasty, circular resection of the trachea, esophagoplasty.
ICD 10
J95.0 Q39.1 Q39.2
Meaning
Tracheoesophageal fistula is a congenital or acquired defect that supports abnormal communication between the trachea and the esophagus. Congenital tracheoesophageal fistula is diagnosed in 1 out of 3,000 newborns. Isolated fistulas make up no more than 3-4% of all esophageal abnormalities; most often they are located at the level of the VII cervical- I thoracic vertebrae. In most cases, congenital fistulas are accompanied by esophageal atresia. The frequency of acquired pathology has not been reliably determined, however, researchers note that iatrogenic tracheoesophageal messages occur in 0.5-3% of patients and are often combined with cicatricial tracheal stenosis. The problem of prevention and treatment of tracheoesophageal fistulas is relevant for pediatrics, anesthesiology and resuscitation, thoracic surgery.
Causes of tracheoesophageal fistula
Congenital tracheoesophageal fistula is formed by the 4th-6th week of embryogenesis with incomplete separation of the primary intestine and the respiratory tube. At birth, children with this anomaly usually have a body weight of less than 2500 g.
The nature of the origin of acquired tracheoesophageal fistulas may be different. Most often, oncologists have to deal with such pathology, and fistulas themselves arise as a result of far-reaching cancer of the esophagus or trachea, lymphomas, lymphogranulomatosis. The second most common cause is injuries to the digestive and respiratory tubes (including their iatrogenic injuries) received during esophagoscopy, esophageal augmentation, perforation of the esophagus or trachea by foreign bodies, due to chemical burns of the esophagus, with closed and penetrating wounds of the neck and chest. Unintentional damage to the trachea and esophagus may occur during surgical interventions in this area.
In anesthesiological and resuscitation practice, cases of bedsores from a tracheostomy or intubation tube have been noted, leading to the formation of a tracheoesophageal fistula. Among the rare causes include perforation of the diverticulum of the esophagus into the trachea. Disease can be a complication of a nonspecific bacterial infection (pleural empyema, lung abscess, purulent mediastinitis) or specific inflammatory processes (tuberculosis of the intra-thoracic lymph nodes, syphilis, actinomycosis).
Classification
By their origin, tracheoesophageal fistulas can be congenital or acquired in the course of life. There are 5 types of congenital tracheoesophageal fistulas, designated by Roman signs:
- Type I – both ends of the atresized esophagus are blind, the proximal end of the esophagus communicates with the trachea in its upper part;
- Type II – the continuity of the esophagus is preserved, but there is a fistulous communication between the posterior tracheal wall and the anterior part of the esophagus. It accounts for about 5% of cases of all tracheoesophageal fistulas;
- Type IIIA – both ends of the atresized esophagus are blind, the proximal end of the esophagus communicates with the trachea in its lower part;
- Type IIIB – atresia of the proximal segment of the esophagus; the distal end of the esophagus connects to the breathing tube in its lower part. Accounts for the majority of cases of congenital tracheoesophageal fistulas;
- Type IIIC – atresia of the proximal segment of the esophagus; both ends of the esophagus (proximal and distal) connect to the trachea at different levels.
The etiology of acquired tracheoesophageal fistulas can be postoperative, post-inflammatory, posttraumatic, postintubation, tumor. Depending on the duration of their existence, they are divided into acute (up to 1 month) and chronic (over 1 month). Taking into account the length of the fistula, short fistulas (1 cm) are isolated. By the length of the tracheoesophageal defect, small fistulas (1-3 cm) and giant (>3 cm) are distinguished; by the width of the message – narrow (up to 1 cm) and wide (more than 1 cm).
According to the presence/absence of a valve mechanism, tracheoesophageal fistulas are divided into valvular and valveless. With a single defect, they talk about an isolated fistula, when combined with other pathological processes of the trachea or esophagus, about a combined one. The clinical course of tracheoesophageal fistulas can be uncomplicated, smooth or complicated by various conditions (aspiration pneumonia, nutritional insufficiency and cachexia, bacterial destruction of the lungs, pneumogenic sepsis, etc.).
Tracheoesophageal fistula symptoms
The clinical picture, its severity and features depend on the time of occurrence, size, type, direction of the course of the fistula. Most children with this anomaly are born prematurely, and full-term newborns have signs of intrauterine hypotrophy. During gestation, pregnant women have polyhydramnios.
Signs of congenital tracheoesophageal fistula with esophageal atresia appear within a few hours after birth. There is a cough, attacks of suffocation, bloating. During the first feeding, the diagnosis becomes obvious. Each sip of milk or water is accompanied by an attack of coughing, the release of foamy mucus from the nose and mouth of the child. Breathing becomes difficult, sharp cyanosis and arrhythmia develop, abundant large-bubble wheezing is heard in the lungs. Aspiration pneumonia and lung atelectasis join early. The child’s condition is progressively deteriorating.
The listed signs are most pronounced with a wide and short tracheoesophageal fistula. In the presence of a narrow and long fistula course, the anomaly often remains unrecognized for a long time. Occasionally, choking and mild coughing occur during feeding, but changing the feeding position relieves the child from seizures, so parents do not attach due importance to this fact. Such children suffer from frequent pneumonia.
In 40% of newborns with tracheoesophageal fistula, concomitant congenital heart defects, abnormalities of the development of the intestine and urinary tract are detected. The literature describes the VATER syndrome, which includes anomalies in the development of the spine (wedge-shaped vertebrae), atresia of the anus, tracheoesophageal fistula, hypoplasia of the radius and kidney dysplasia.
Acquired tracheoesophageal fistula manifests by coughing, choking, cyanosis associated with eating. In the coughed-up secret, you can find pieces of food. With valvular tracheoesophageal fistula, cough may be absent. Often, patients have chest and epigastric pain, hemoptysis, vomiting with an admixture of blood, shortness of breath, impaired phonation, weight loss. In all cases, tracheoesophageal fistula is accompanied by periodic temperature rises, recurrent lung infections.
Diagnostics of tracheoesophageal fistula
If a tracheoesophageal fistula is suspected, the patient needs to consult a thoracic surgeon, gastroenterologist, pediatric surgeon, endoscopist, radiologist. At the initial stage, probing of the esophagus with a rubber catheter is used: if the resistance of the blind end of the esophagus interferes with the catheter’s progress, and when a liquid stained with methylene blue is injected into the catheter, it is released through the mouth or nose when coughing, the probability of a tracheoesophageal fistula is estimated as very high.
Radiation examination includes performing radioscopy and lung x-ray, esophagography, chest MSCT. Contrast examination of the esophagus is carried out very carefully, using water-soluble or oily substances that are injected through a probe (the use of barium sulfate in these cases is unacceptable). Tracheoesophageal fistula requires a thorough differential diagnosis with isolated atresia and esophageal stenosis, gastroesophageal reflux, achalasia of the cardia, dysphagia of various genesis.
Endoscopic diagnosis of tracheoesophageal fistula remains the main one. Esophagoscopy and tracheobronchoscopy are performed to better visualize the pathological anastomosis and obtain maximum information about its features. In case of critical tracheal stenosis, diagnostic tracheoscopy can be transformed into a therapeutic procedure by endoscopic restoration of the tracheal lumen or stenting of the stenosed area.
Tracheoesophageal fistula treatment
Treatment is carried out surgically, conservative therapy is unpromising and is used only at the stage of preparation for surgery. In order to remove the accumulated secretion, a catheter is inserted into the blindly ending end of the esophagus, sanitization bronchoscopy is performed. As part of the treatment of aspiration pneumonia, oxygen therapy, antibacterial and infusion therapy, UHF on the chest are prescribed. To prevent food from entering the tracheobronchial tree, mouth feeding is completely stopped, gastrostomy, nutritional support, and vitamin therapy are performed.
The options for radical surgical removal of tracheoesophageal fistula may be different. The operation is usually performed from a cervical access or a posterolateral thoracotomy. A long fistula is isolated and crossed between two superimposed ligatures. Defects in the walls of the esophagus and trachea are sutured with immersion sutures. With a short and wide fistula, the trachea is cut off from the esophagus, after which a continuous double-row suture is applied to both defects. Auto tissues (omentum or stomach wall) can be used to close the defect.
In cases where tracheoesophageal fistula is combined with tracheal stenosis or esophageal atresia, the fistula is separated with simultaneous circular resection or tracheal plastic surgery and/or simultaneous esophageal plastic surgery. If there is a large diastasis between the proximal and distal segments of the esophagus, which does not allow an end-to-end anastomosis, the upper part of the esophagus is removed to the neck, the fistula is separated and the operation is completed.
During the interoperative period, the patient is fed through a gastrostomy. At the second stage, the esophagus is plasticized with a gastric or small intestine transplant. In tracheoesophageal fistulas caused by the collapse of a cancerous tumor, palliative gastrostomy is limited. Active anti-pneumonic therapy continues in the postoperative period. Parenteral nutrition is carried out on the first day, then feeding through a probe, which is left for 7-10 days.
Forecast
Postoperative complications may include stenoses in the area of esophageal anastomoses, which are subject to bougie, and relapses of the fistula, requiring repeated surgery. Mortality during surgical treatment of tracheoesophageal fistula is 10-15% and is mainly associated with the initially severe condition of the patient. At the same time, if the operation is refused, the mortality rates increase to 80-90%.
Literature
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Cervical tracheoesophageal fistula. Laryngoscope. 1983 Mar;93(3):364-9. link
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Surgical management of acquired nonmalignant tracheoesophageal and bronchoesophageal fistulae.
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Possibilities and results of surgical treatment of benign tracheoesophageal fistula. Rozhl Chir. 2010 link
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Surgery for intrathoracic tracheoesophageal and bronchoesophageal fistula. Ann Transl Med. 2018 Jun;6(11):210. link
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Management of acquired tracheoesophageal fistula. Chest Surg Clin N Am. 1996 Nov;6(4):819-36.PMID: 8934011 Review. link
