IS Case 552: 1. Esophageal atresia with tracheoesophageal fistula; 2. Imperforate anus
Imaging Sciences URMC 2010
Publication Date: 2010-09-25
A single AP view of the chest and abdomen demonstrated an oropharyngeal tube tip in the thoracic inlet with a suggestion of a surrounding pouch. Additionally, there were dilated loops of distal colon, likely representing the sigmoid colon and proximal rectum, with absence of air in the distal rectum.
Esophageal atresia (EA) occurs in 1/3,000-4,500 live births. Esophageal atresia should be suspected if any one of the following is present: maternal polyhydramnios, excessive oral secretions in the newborn, cyanosis, regurgitation or coughing occurring with the first feeding. Esophageal atresia results from failure of division of the foregut into tracheal and esophageal channels in early embryogenesis. The majority of EAs are accompanied by a fistula between the trachea and the distal esophagus. In rare instances, infants have a tracheoesophageal fistula without an esophageal atresia. This is known as an H-type TEF. The best radiological clue for EA is a dilated pharyngeal pouch, containing the tip of an enteric tube, which is unable to pass secondary to obstruction. An EA with a TEF can be distinguished from an EA without a TEF by the presence of gas in the bowel. Fluoroscopic studies are rarely needed for the diagnosis of EA; however, may be of utility in the diagnosis of H-type TEF.
Imperforate anus occurs in approximately 1/5,000 births. It is a congenital defect in which the rectum is malformed, resulting in an absent opening of the anus. Imperforate anus is often diagnosed on physical exam, with absence of the normal anal dimple. Radiologically the best diagnostic clue is a dilated distal colon, with absence of air in the distal rectum.
One half of infants with esophageal atresia have other associated abnormalities. For example, the VACTER association includes vertebral defects, anal atresia, cardiac anomalies, esophageal atresia with or without tracheoesophageal fistula, limb hypoplasia and renal defects. These non-random associated congenital abnormalities should be considered when one is present.
- Berdon WE, Baker DH. Radiographic findings in esophageal atresia with proximal pouch fistula (type B). Pediatr Radiol. 1975 Mar 20;3(2):70-4.
- Blickman JG, Parker BR, Barnes PD. Pediatric Radiology: The Requisites, 3rd ed. Mosby, 2009.