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Esophageal atresia


Definition:

Esophageal atresia is a disorder of the digestive system in which the esophagus does not develop properly. The esophagus is the tube that normally carries food from the mouth to the stomach.

Alternative Names:

Tracheoesophageal fistula

Causes:

Esophageal atresia is a congenital defect, which means it occurs before birth. There are several types. In most cases, the upper esophagus ends and does not connect with the lower esophagus and stomach. The top end of the lower esophagus connects to the windpipe. This connection is called a tracheoesophageal fistula (TEF). Some babies with TEF will also have other problems, such as heart or other digestive tract disorders.

Other types of esophageal atresia involve narrowing of the esophagus, and may also be associated with other birth defects.

Esophageal atresia occurs in about 1 out of 4,000 births.

Symptoms:
  • Bluish coloration to the skin (cyanosis) with attempted feedings
  • Coughing, gagging, and choking with attempted feeding
  • Drooling
  • Poor feeding
Exams and Tests:

Before birth, an ultrasound performed on the pregnant mother may show too much amniotic fluid, which can be a sign of esophageal atresia or other blockage of the digestive tract.

The disorder is usually detected shortly after birth when feeding is attempted and the infant coughs, chokes, and turns blue. As soon as the diagnosis is suspected, an attempt to pass a small feeding tube through the mouth or nose into the stomach should be made by a health care professional. The feeding tube will not be able to pass all the way to the stomach in a baby with esophageal atresia.

An x-ray of the esophagus shows an air-filled pouch and air in the stomach and intestine. If a feeding tube has been inserted, it will appear coiled up in the upper esophagus.

Treatment:

Esophageal atresia is considered a surgical emergency. Surgery to repair the esophagus should be done quickly after the baby is stabilized so that the lungs are not damaged and the baby can be fed.

Before the surgery, the baby is not fed by mouth. Care is taken to prevent the baby from breathing secretions into the lungs.

Outlook (Prognosis):

An early diagnosis gives a better chance of a good outcome.

Possible Complications:

The infant may breathe saliva and other fluids into the lungs, causing aspiration pneumonia, choking, and possibly death.

Other complications may include:

  • Feeding problems
  • Reflux (the repeated bringing up of food from the stomach) after surgery
  • Narrowing (stricture) of the esophagus due to scarring from surgery

Prematurity may complicate the condition.

When to Contact a Medical Professional:

This disorder is usually diagnosed shortly after birth.

Call your baby's health care provider if the baby vomits repeatedly after feedings, or if the baby develops breathing difficulties.

References:

Long JD, Orlando RC. Anatomy, histology, embryology, and developmental anomalies of the esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 41.

Khan S, Orenstein SR. Congenital anomalies. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 311.

Orenstein S, Peters J, Khan S, Youssef N, Hussain SZ. Congenital anomalies: esophageal atresia and tracheoesophageal fistula. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 316.


Review Date: 9/1/2013
Reviewed By: Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School; Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

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