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Tracheoesophageal Fistula and Esophageal Atresia

Tracheoesophageal fistula and esophageal atresia are among the most serious congenital anomalies in neonates. They may develop separately but usually occur together. In tracheoesophageal fistula, an abnormal connection develops between the trachea and the esophagus. In esophageal atresia, the esophagus is closed off at some point.

Both disorders are surgical emergencies, requiring immediate diagnosis and correction. Sometimes they coexist with other serious anomalies, such as congenital heart disease, imperforate anus, genitourinary abnormalities, and intestinal atresia.

Congenital malformations of the esophagus occur in about 1 in 4,000 live births. These malformations have many anatomic variations. They're classified in the following ways:

  • In type C tracheoesophageal fistula with esophageal atresia - by far the most common esophageal malformation - the upper section of the esophagus terminates in a blind pouch (which has no fistula). The lower section ascends from the stomach and connects with the trachea by a short fistulous tract.
  • In type A atresia, both esophageal segments are blind pouches, and neither has a fistulous connection to the airway.
  • In type E (also known as type H or tracheoesophageal fistula without atresia), the fistula may occur anywhere between the level of the cricoid cartilage and the mid-esophagus. The fistula, which may be as small as a pinpoint, is usually higher in the trachea than in the esophagus.
  • In types B and D, the upper portion of the esophagus opens into the trachea. (In type D, an additional fistula connects the trachea and the esophagus at G lower level) Infants with either anomaly may experience life-threatening aspiration of saliva or food.

Causes

We don't really know what causes these problems. When the esophagus and the trachea grow in the embryo, they start from the same bit of tissue. Sometimes the tubes don't develop right. We don't think these problems are inherited.

Signs and Symptoms

The symptoms of TE fistula or esophageal atresia are usually noted very soon after birth. The following are the most common symptoms of TE fistula or esophageal atresia. However, each child may experience symptoms differently. Symptoms may include the following:

  • frothy white bubbles in the mouth
  • coughing or choking when feeding
  • vomiting
  • blue color of the skin, especially when the baby is feeding
  • difficulty breathing
  • very round, full abdomen

Diagnostic tests

Tests used to help confirm and classify tracheoesophageal fistula and esophageal atresia include a radiopaque #8 or # 10 French catheter passed through the nose, which confirms the presence of a blind pouch in the proximal esophagus if it meets an obstruction between 4" and 5" (10 and 13 cm) distal to the nostrils.

Chest X-ray demonstrates the catheter position in the esophagus and can also show a dilated, air-filled upper esophageal pouch; pneumonia in the right upper lobe of the lung; or bilateral pneumonitis. Both pneumonia and pneumonitis suggest aspiration.

Abdominal X-ray shows gas in the bowel in a distal fistula (type C) but none in a proximal fistula (type B) or in atresia without fistula (type A).

Cinefluorography allows visualization on a fluoroscopic screen. After a # 10 or # 12 French catheter is passed through the patient's nostril into the esophagus, a small amount of contrast medium is instilled to define the tip of the upper pouch. Findings help differentiate between overflow aspiration from a blind end (atresia) and aspiration due to passage of liquids through a tracheoesophageal fistula.

Bronchoscopy with telescopic endoscopy also can confirm the diagnosis in most patients.

Treatment

Tracheoesophageal fistula and esophageal atresia require surgical correction and are usually emergencies. The type of surgery and when it's performed depend on several factors: the nature of the anomaly, the patient's general condition, and the presence of coexisting congenital defects.

Depending on what type of anomaly the child has, a sump tube may be placed in the esophageal pouch until surgery is performed. This procedure is done to remove accumulated secretions, decreasing the passibility of aspiration. A gastrostomy tube may be placed to decompress the stomach. The child's respiratory status must be closely monitored.

Both before and after surgery, positioning varies according to the doctor's preferences and the child's anatomy: The child may be placed in a supine position, with his head low to aid drainage or elevated to prevent aspiration.

To correct gastroesophageal reflux and esophageal atresia, a thoracotomy is performed and the fistula is ligated, after which the upper and lower segments the esophagus are anastomosed. In patients who are poor surgical risks, such as those born prematurely or those with other congenital defects, correction of combined tracheoesophageal fistula and esophageal atresia is done in two stages. The first stage consists of gastrostomy (for gastric decompression, prevention of reflux, and feeding) and closure of the fistula One to 2 months later, the esophagus is anastomosed.

Correction of esophageal atresia alone requires anastomosis of the proximal and distal esophageal segments in one or two stages. End-to-end anastomosis often produces postoperative stricture; end-to-side anastomosis is less likely to do so. If the esophagea: ends are widely separated, treatment may include a colonic interposition (grafting a piece of the colon) or elongation of the proximal segment of the esophagus by bougienage. About 10 days after surgery, and again 1 month and 3 months later, X-rays are required to evaluate the effectiveness of surgical repair.



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