c l i n i c a l f o l i o s : d i s c u s s i o n

Esophageal Atresia and Tracheoesophageal Fistula



Related narrative: Tracheoesophageal Fistula

Background and Epidemiology

Esophageal atresia occurs in 1:3000-5000 live births, and in more than 85% of the cases, a tracheoesophageal fistula (TEF) accompanies the atresia. Occasionally, one of these conditions will occur alone or with other combinations: the second most common is atresia without TEF, and the third most common is the H-type fistula, which is TEF without the esophageal atresia. This last anomaly is the most difficult to diagnose, and patients may go for years with minimal symptoms. The most common variant comprises a blind esophageal pouch with a fistula between the trachea and the distal esophagus. The fistula often enters the trachea close to the carina. Problems with the formation of the paired cranial and single caudal folds in the primitive foregut explain the variations in atresia and fistula formation.

About 50% of children with TEF and atresia have nonhereditary concurrence of three or more defects of various systems including the constellations known as VATER (veterbral or vascular defects, anal anomalies, TEF, esophageal atresia, and radial limb or renal anomalies) (also known as VACTERL [vertebral, anal, cardia, tracheal, esophageal, renal, and limb]) and CHARGE (coloboma, heart defect, atresia choanae, retarded growth, genital anomalies, and ear anomalies). Cardiac anomalies, such as ventricular septal defect, patent ductus arteriosus, and tetralogy of Fallot, are the most common-occurring in about one quarter of infants with esophageal atresia. Gastrointestinal anomalies, like imperforate anus, duodenal atresia, and malrotation, occur in about 16% of the babies.

Diagnosis and Treatment

Maternal polyhydramnios may be an early sign of esophageal atresia. The inability to identify the fetal stomach on a prenatal sonogram makes the diagnosis more probable. Infants with esophageal atresia usually present with excessive, fine, white, frothy, oral secretions and choking, cyanosis, or coughing at attempted feeding. The diagnosis may not be made until the child has aspirated feedings. A nasogastric tube should be passed through the nose to the stomach. In patients with the condition, the tube will stop at 10 to 12 cm instead of the normal 17 cm. Chest film should be taken to confirm the position of the tube. Air in the stomach or bowel will confirm the TEF, unless it is esophageal atresia with proximal TEF, in which case the stomach will be airless. The chest radiograph will also provide information about heart and skeletal malformations and pneumonia.

Before the first successful surgical repair in 1943, this condition was fatal. Prompt diagnosis and appropriate clinical management have had a dramatic effect on survival. In fact, in the absence of other problems, survival rates approach 100%. Infants who are premature or have associated anomalies have a survival rate of about 70%, but infants who are severely premature or have serious congenital anomalies may only have a 10% survival rate.

Treatment in the great majority of cases consists of dividing and oversewing the fistula, and anastomosing the esophageal ends in one stage as in this case. In the case of a long-gap esophageal atresia and TEF, there are various procedures to stretch the proximal and distal esophageal pouches (e.g., proximal circular myotomy and distal circular myotomy) that can help ensure success of the anastomosis. In extreme cases, the fistula is divided and the proximal esophageal pouch brought out on the neck as a cervical esophagostomy. A gastrostomy is created for feeding. Then, a passage from the proximal segment of the esophagus to the stomach can be made from a segment of colon or stomach. In such cases, the infant may be given sham feedings by mouth in conjunction with gastrostomy feeding, to accustom the infant to associate oral feeding with satiety, and decrease later feeding difficulties.

Recurrence of the TEF is associated with an anastomotic leak and is reported in 4-10% of the cases. Recurrent TEFs do not resolve spontaneously, so surgical repair is necessary. Postoperative complications are common and can include atelectasis, pneumonia, esophageal dysmotility, gastroesophageal reflux disease (25-50%), anastomotic stricture (15-30%), and tracheomalacia (8-15%). Because of the esophageal dysmotility issues and complications of GERD, long-term follow up of these patients is important.


Clark, DC. Esophageal atresia and tracheoesophageal fistula. 1999 Am Fam Phys 59(4).

Hilman, BC, Lierl, MB, Constantinescu, M. Congenital lung disease. In: Bone: Pulmonary & Critical Care Medicine, 1998 ed., St. Louis, MO:Mosby-Year Book.

Engum SA, Grosfeld JL. Pediatric surgery. In: Townsend: Sabiston Textbook of Surgery, 16th ed., 2001, Philadelphia, PA:W.B. Saunders Company.

This page was last modified on 24-Jan-2002.