What is Esophageal Atresia?
What is Esophageal Atresia?Esophageal atresia is a congenital (born with) condition in which a baby’s esophagus does not connect the mouth to the stomach. No food can reach the stomach, and digestive fluids can get into the windpipe and lungs.
Esophageal atresia is life-threatening. It requires surgery soon after birth. About one in 4,100 babies is born with esophageal atresia in the United States.
Types of Esophageal Atresia
TypesIn a baby born with esophageal atresia, the esophagus (swallowing tube) has developed in two parts that don’t connect. In most cases, either the upper or lower part attaches to the trachea (windpipe, or breathing tube). This abnormal connection is called a fistula. The two abnormalities together are commonly called esophageal atresia/tracheoesophageal fistula (EA/TEF).
The types of esophageal atresia are:
- Type A. Both the upper and lower parts of the esophagus have closed ends. They do not connect to each other or to the trachea. This type of esophageal atresia is rare, occurring in five to seven percent of cases.
- Type B. The upper part of the esophagus connects to the trachea (windpipe). The lower part has a closed end. Type B is very rare.
- Type C. The upper part of the esophagus has a closed end. The lower part is attached to the trachea, connecting the stomach and the trachea. This is the most common abnormality; about 85 percent of cases of esophageal atresia are Type C.
- Type D. The upper and lower parts of the esophagus each connect separately to the trachea. This is the rarest and most severe type of esophageal atresia.
- Type E. The upper and lower parts of the esophagus connect, but there is an extra branch of the esophagus that connects to the trachea. This type of esophageal atresia is rare, occurring in five to seven percent of cases.
Signs & Symptoms of Esophageal Atresia
SymptomsThe pediatric specialists who care for newborns will notice symptoms of esophageal atresia within a few hours of a baby’s birth and will refer the infant for supportive care.
Newborns with esophageal atresia may have symptoms including:
- A bluish tint to the skin or lips (cyanosis) during attempted feeding
- Coughing and choking with attempted feeding
- Drooling
- Poor feeding
- Trouble breathing
What Causes Esophageal Atresia?
CausesThe causes of esophageal atresia are not known. In some instances, genetic abnormalities may cause or contribute to problems with the way the esophagus develops before birth.
Two risk factors are known to increase the chances of a baby being born with this condition:
- Father over the age of 40 at the time of the baby’s conception
- Use of infertility treatments, including intrauterine insemination (IUI) and in vitro fertilization (IVF), to conceive
Risk Factors
Risk FactorsEsophageal atresia is rare. However, the chances of a baby being born with this condition are increased if:
- The age of the father is over 40 at the time of conception
- Infertility treatments, including intrauterine insemination (IUI) and in vitro fertilization (IVF) are used to conceive
Babies with esophageal atresia may have other physical abnormalities at birth, including:
- Heart problems such as ventricular septal defects or Tetralogy of Fallot
- Malformations in the gastrointestinal (digestive) system, like imperforate anus (incomplete or closed anal opening) or intestinal atresia (incomplete bowel formation)
- Problems in the urogenital tract that can affect the production or release of urine
- Neurological and musculoskeletal malformations, like abnormalities of the spine, ribs, or arms
Complications
ComplicationsNewborns with esophageal atresia must have surgery, often very soon after birth, to connect the segments of the esophagus and separate it from the trachea. Surgery is successful in the vast majority of children. However, as children continue to grow and develop, they often need continued care.
Complications can include:
- Gastroesophageal reflux disease (GERD), meaning stomach acid goes up the esophagus, which can lead to inflammation
- Narrowing of the esophagus (stricture), caused by scarring from surgery; this makes it harder for food to pass through
- Muscles in the esophagus have trouble moving food into the stomach
- Digested food in the stomach moves back up into the esophagus
- Tracheomalacia, a condition in which the walls of the windpipe are weak and floppy, leading to trouble breathing
- Recurrent cough or pneumonia
Trust the Children's Hospital of New York for Esophageal Atresia Care
The Children's Hospital of New York is among the nation’s leading centers for the diagnosis and treatment of childhood diseases.
For children, expert and timely esophageal atresia diagnosis and treatment can be life-changing. Schedule an appointment for esophageal atresia care. Our multidisciplinary teams of specialists provide children with esophageal atresia with a comprehensive and individualized plan of care that addresses each child’s unique needs throughout their childhood, giving them the best chance to thrive.