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Atresia and Transesophageal Fistula- Causes, Classification, Symptoms, Diagnosis, and Treatment

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Atresia and tracheoesophageal fistula are two congenital conditions causing defects in the esophagus. Read this article to learn about it.

Medically reviewed by

Dr. Ghulam Fareed

Published At December 2, 2022
Reviewed AtDecember 2, 2022

Introduction:

The esophagus is the hollow, round muscular tube that helps pass food from the throat to the stomach. The primary function of the esophagus is to carry food which first moves from the mouth to the throat (pharynx) and then to the stomach. Esophageal atresia and tracheoesophageal fistula are congenital disabilities in which the esophagus does not develop normally. They can often occur together and affect the development of the esophagus, trachea, or both. These are life-threatening conditions and should be treated as soon as possible.

During embryological development, the trachea connects the throat to the lungs for passing air) and the esophagus (a tube that connects the mouth to the stomach for providing food) forms as a single tube. In the developing phase, a wall usually forms in the tube, dividing them into two completely separate tubes. If the esophagus and trachea do not divide completely or develop abnormally, esophageal atresia and tracheoesophageal fistula can occur.

What Is Esophagus Atresia?

Esophageal atresia (EA) is a condition of the esophagus that usually occurs in early pregnancy as the baby develops, which is why it is present at birth. The esophagus develops during the first few months of fetal development, and with this defect, the formation of the esophagus gets interrupted. In this condition, two esophageal tubes are formed, one from the throat and the other from the stomach, but they do not unite, which leads to a gap in the tube, and the food or liquid will not be able to pass through that.

What Is a Tracheoesophageal Fistula?

Tracheoesophageal fistula (TEF) is often present in babies with esophagus atresia. In this, an abnormal one or two openings develop between the esophagus and trachea. Due to these openings, saliva can pass through the esophagus into the lungs, leading to lung problems. Moreover, in premature babies with underdeveloped lungs and tracheoesophageal fistula, the air might pass from the trachea into the stomach, causing severe stomach swelling and respiratory distress.

Another condition, tracheomalacia, can also develop in newborns with esophageal atresia and tracheoesophageal fistula. In this condition, the trachea becomes softer or less rigid than usual and causes a barking cough, and its severity varies among children.

What Are the Causes of Esophageal Atresia?

The exact cause of esophageal atresia is unknown. Although, it is thought to be caused by a problem with the development of the esophagus while the baby is in the womb. This condition is more common in babies with mothers who have too much amniotic fluid during pregnancy (polyhydramnios).

It can also be seen in babies with kidney, heart, and spine problems.

What Is the Classification of Esophageal Atresia and Tracheoesophageal Fistula?

Classification of Esophageal Atresia and Tracheoesophageal Fistula

What Are the clinical features of Esophageal Atresia and Tracheoesophageal Fistula?

The symptoms of esophageal atresia and tracheoesophageal fistula vary significantly from one newborn to another. The symptoms include -

  • Coughing or choking during feeding.

  • Frothing or drooling from the mouth.

  • Vomiting.

  • Difficulty breathing while feeding.

  • Blue-tinged skin while feeding.

  • An unusually rounded abdomen.

  • Failure to gain weight.

What Is the Diagnosis of Esophageal Atresia and Tracheoesophageal Fistula?

Routine ultrasound scans can early diagnose esophageal atresia and tracheoesophageal fistula. Other than that, most of the tests are carried out after the birth of the baby. After a complete physical examination, the doctor can advise the tests if the baby is having problems with swallowing or breathing. For diagnosis, a thin feeding tube can be passed down from the throat of the baby through the nose to check if it can reach the stomach, and x-rays can be taken to check the esophagus.

What Are the Treatment of Esophageal Atresia and Tracheoesophageal Fistula?

The most common approach for treating esophageal atresia and tracheoesophageal fistula is surgery, done soon after the birth of the baby. The surgery is done under general anesthesia (GA), and the baby will receive their nutrition intravenously (IV). And a suction tube is used to remove the fluid from the pouch in the esophagus.

The surgeon makes an incision on the right side of the chest between the ribs, and the abnormal opening (fistula) is closed between the esophagus and windpipe. And after that, the upper and lower parts of the esophagus will be attached.

And in some cases where the gap is large, the surgery has to be delayed for a few months so that the gap can be reduced with the growth of the esophagus.

Post-surgical Care

After the surgery, the baby is kept in the intensive care unit (ICU) and has to be placed in an incubator. Moreover, they need the following things to survive -

  • Antibiotics.

  • Ventilator to help them breathe.

  • A tube is inserted into the chest to drain trapped fluids or air.

  • Oxygen through a mask.

  • Analgesics (pain killers).

What Are the Risks of Surgery?

Some of the risks involved with this surgery include the following-

  • Leakage of food or air from the operated area.

  • Narrowing of the operated esophagus.

  • Recurrence of fistula or reopening between the esophagus and trachea.

Conclusion:

Esophageal atresia and tracheoesophageal fistula are rare and life-threatening conditions; that require early treatment to save the child. Regular checkups and ultrasounds are the ideal methods for the early diagnosis of the disease. And even after the surgery, proper care has to be taken of the child because of the risk factors associated with the conditions.

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Dr. Ghulam Fareed
Dr. Ghulam Fareed

Medical Gastroenterology

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