What is esophageal atresia?Esophageal atresia (absence or abnormal narrowing of an opening or passage in the body), is a birth defect of the esophagus that occurs early in pregnancy as the baby is developing. Often times, this condition is not diagnosed during pregnancy. The esophagus and trachea form as one structure in the first few months of fetal life as a long, hollow, continuous tube joining the mouth to the stomach. In newborns with this birth defect the esophagus grows into two separate segments that do not connect.
In most cases, two separate tubes are formed, an upper tube that ends blindly in a pouch in the upper chest, and a lower tube that is connected to the trachea (airway) on the upper end and connected to the stomach below. These separated tubes are sealed off creating a pouch on either side; the gap between these pouches can be short or very long. Saliva can accumulate in the upper pouch as it cannot drain into the stomach.
Other birth defects may co-exist, particularly in the heart, but sometimes also in the anus, spinal column, or kidneys. This is known as VACTERL association because of the possible involvement of Vertebral column, Anorectal, Cardiac, Tracheal, Esophageal, Renal (kidney), and Limbs. Associated congenital anomalies are discovered in approximately one half of infants with esophageal atresia. Other midline defects are the most common of these anomalies. Most infants have more than one malformation. If suspected, your child will have associated anomalies ruled out by imaging such as an echocardiogram, spine ultrasound, and abdominal ultrasound.
Tracheosophageal FistulaThere are five combinations of defects of esophageal atresia and tracheoesophageal fistula. In the most common type (85% of the time), newborns with esophageal atresia often also have a tracheoesophageal fistula which is a direct connection between the esophagus and the trachea (windpipe). Most commonly with esophageal atresia, this connection forms off of the lower pouch; however, it can also form off of the upper pouch or both, and in some cases, a fistula will form without atresia. In another distinct type, there is atresia without a fistula, and often the distance between the two ends of the esophagus is long.
- Frothy white bubbles in your baby’s mouth
- Coughing or choking when feeding
- Blue color of the skin, especially during feedings
- Difficulty breathing
- Round, full abdomen
- Inability to pass a tube from the nose through to the stomach
How is it treated? What will happen in the hospital?Other birth defects may co-exist if your child has esophageal atresia, particularly in the heart, but sometimes also in the anus, spinal column, or kidneys. This is known as VACTERL association because of the involvement of Vertebral column, Anorectal, Cardiac, Tracheal, Esophageal, Renal, and Limbs. It is associated with polyhydramnios in the third trimester. The work up of esophageal atresia includes a complete physical exam with special attention to identify associated anomalies. An oral gastric tube is passed from the mouth into the esophagus. If it does not pass into the stomach, the diagnosis is highly suspected. A chest x-ray is then obtained with the oral tube in place. This helps confirm placement and allows the surgeon to estimate the gap between the 2 ends of the esophagus. If there is no air in the stomach, no TEF (tracehoesophageal) fistula is present. Most isolated esophageal atresia’s are “long gap,” and requires multiple staged surgeries to repair. An echocardiogram of the heart must be obtained prior to surgery. A renal ultrasound to examine the kidneys is preferably done prior to surgery as well. Other studies such as a spinal ultrasound for annormalities of the spinal cord can be done after surgery. The final confirmation of the diagnosis is made in the operating room. An intra-operative bronchoscopy (a device placed inside to view the airway, trachea and lungs) may be performed to confirm the presence of a fistula to the distal esophagus.
Your baby will be cared for in an Intensive Care Nursery by specially trained doctors and nurses. Before the esophagus is repaired, saliva can accumulate in the upper pouch, and may spill into the lungs. This can lead to choking. To prevent choking, a small tube, attached to suction, is passed through the mouth into the upper pouch to provide continuous drainage of saliva.
If a tracheoesophageal fistula is present, it must be surgically closed soon after birth. During fistula closure, if the distance or gap between the two ends of the esophagus is short, they will be sewn together and the esophageal atresia will be repaired. This is called a primary repair. If the upper and lower pouches are too far apart to bring together, only the fistula will be closed and the repair of the esophagus may be delayed. If the gap is very wide, an alternative method of surgical repair of esophageal atresia may be offered. The operation may be done through an incision in the chest, or with small incisions (minimally invasive surgery). The approach will be discussed with you by your child's pediatric surgeon.
At the time of esophageal repair, a tube will be placed in your child's chest to drain any fluid that might accumulate near the surgical site. Approximately a week after surgery a special x-ray, called a contrast esophogram, will be performed. This test is done to ensure there is no leak at the esophageal repair site. If the repair has healed, the chest tube will be removed and feedings will be started by mouth. In some cases the esophageal repair may take longer to heal, and a leak may be visible on the x-ray study. In this case, the chest tube will remain in place until the esophageal repair has completely healed as confirmed by x-ray studies.
When will my child be discharged?Your baby will remain in the hospital until he/ she is taking full feedings by mouth, or gastrostomy tube, and is gaining weight. This usually takes two to four weeks. Babies born with esophageal atresia and tracheoesophageal fistula may have tracheomalacia (soft airways). This occurs during pregnancy as a result of compression of the dilated upper pouch of the esophagus on the developing trachea. Tracheomalacia may contribute to breathing problems after birth and after hospital discharge, and can contribute to a “barking cough.” Tracheomalacia is a condition the baby usually outgrows as the airways become firmer. Sometimes tracheomalacia is severe and requires additional treatment. Other risks of surgery include recurrent fistula ( when the fistula re-forms), which occurs a small proportion of the time, and poor function of the vocal cords, which can develop in some cases after the operation and can affect swallowing. This usually improves with time.
What will be my child's recovery?Babies with esophageal atresia and tracheoesophageal fistula also have gastroesophageal reflux, a back flow of contents from the stomach into the esophagus. This is due to poor motility (function) of the lower portion of the esophagus. Reflux may contribute to irritation of the esophagus and cause pain. The irritating effects of the stomach contents on the esophagus may cause tightening (stricture) at the surgical site in the esophagus. To prevent this problem, your baby will be started on an antacid medication and these medications will be continued after hospital discharge, with dosages adjusted as your child gains weight. It is very important that your baby stays on these medications after discharge.
If your baby has a gastrostomy tube, the nurses will show you how to care for it and use it to administer feeding or decompress air from the stomach. A replacement gastrostomy tube of the same size will be sent home with you at discharge. Feeding supplies will be ordered for you before you leave the hospital. These supplies will be delivered to your home by a home care company. Nurses will be requested to visit you and your child after discharge to help ease the transition from the hospital to home.
What should I look out for after the operation? Hospitalization?Children with esophageal atresia and tracheoesophageal fistula may develop esophageal narrowing (stricture) at the site of repair. This may occur about 30% of the time. If this occurs, your child will have trouble swallowing liquids or solids. If the problem is severe you may notice your child coughing or choking on food, vomiting, or refusing to take food or liquids. If this occurs, call our office as soon as possible. We will arrange for a special x-ray called an esophagram, which will help identify any narrowing or stricture of the esophagus. Surgical dilation (stretching) of the esophageal stricture may be required and will be performed under anesthesia. There can also be other causes of these symptoms, such as reflux, tracheomalacia, or poor esophageal function, and our team will perform appropriate tests to determine the cause and treatment.
What complications may arise after the repair?As with any surgery, complications may arise after the procedure. These include:
- Esophageal Leak: This usually heals over several weeks if minor. If more significant, the esophagus may require re-repair or diversion of the esophagus into the neck.
- Gastroesophageal reflux: Treated with antacids but may require a surgical anti-reflux procedure in the future.
- Tracheomalacia: Barking type cough. Cyanosis immediately after feedings. Onset is usually 2 to 3 months post-op. If severe, may require a procedure to lift the trachea away from the esophagus.
- Poor esophageal motility: is common. It generally improves with time but may require medical treatment or dietary modification.
- Esophageal Strictures: develop in many patients and can be managed with outpatient dilations if uncomplicated. More severe strictures may require resection of the stricture.
- Recurrent TEF (fistulas): May require additional procedures to remove the recurrent fistula.
- Dysphagia: difficulty swallowing that generally improves over time and may require intensive swallow therapy.
- Vocal cord palsy: is weakness of one or both vocal folds or cords. It may occur as the nerves going to the vocal cords run close to the esophagus and may be affected during surgery. Symptoms include hoarseness, reduction in vocal volume, pain in the throat when speaking, shortness of breath, aspiration (food or liquids going down the trachea) with frequent resultant coughing. This generally improves over weeks to months.
HomecareYour baby will remain in the hospital until he or she is taking full feedings by mouth, or gastrostomy tube, and is gaining weight. This usually takes several weeks. Once discharged, babies may have problems with acid reflux for the first year. If your baby has a gastrostomy tube, the nurses will show you how to care for it and use it to administer feeding or decompress air from the stomach.
When do I call the surgeon's office?Call our office at 203-785-2701 for the following:
- Any concerns you have about your child's recovery
- A temperature of 101°F or higher
- A red incision
- Increasing pain and tenderness at the incision
- Any liquid coming out of the incision
- Difficulty swallowing liquids or solids, choking episodes
- Questions regarding the gastrostomy tube