“We live because we breathe, drink and eat. Something very crucial to lead our life.” Esophagus also known as the food pipe is the organ that connects our mouth (pharynx) with the stomach. Once food is swallowed from pharynx (mouth), the food passes into esophagus. Esophageal contractions helps to carry masticated food to stomach. Patient is often unable to pass food from pharynx to stomach when patient is suffering with esophageal defects. Narrowing, stricture or complete blockade of esophagus interferes or obstructs the passage of food towards stomach. Esophageal Atresia is seen in newborn and adult when esophageal lumen is completed blocked. The congenital Esophageal Atresia is present during birth and medical condition is considered as a birth defect. The completely blocked or closed esophagus in newborn infants causes obstruction to swallow milk or soft food. Esophageal atresia or narrowing is caused by constriction or closure of the esophageal lumen at some point along its length resulting in a breakdown of the connection between the mouth and the stomach.
Well! The baby with birth defects such as Esophageal Atresia may also suffer from shortness of breath or dyspnea. Following the description of esophageal atresia and associated disease-causing fistula, which is a connection between esophagus and trachea explains more of this condition. The article explains anatomical defects caused by Esophageal Atresia and tracheoesophageal fistula. The section of the articles also describes the latest treatment called “The Foker Technique” for treating Esophageal Atresia.
What Is Esophageal Atresia?
So, as mentioned above, Esophageal Atresia (EA) is actually a birth defect where the baby is unable to pass swallowed food to the stomach. In such a condition the food pipe or the esophagus does not connect the mouth with the stomach. This is, in fact, a very rare congenital birth defect and only an approximate of 1 in 4000 babies suffer from such a condition. In some cases, esophageal atresia is also associated with Tracheoesophageal Fistula (TEF). A newborn baby may be born with EA and TEF and both are congenital defects.Tracheoesophageal Fistula (TEF) is a tubular channel or passage between the esophagus and trachea. Tracheoesophageal fistula is a medical condition caused by the esophagus or the food pipe wrongly connected to the windpipe or the trachea. The continuous passage of small amounts of food or gastric secretions from the esophagus into the trachea causes tracheal and bronchial inflammation and pneumonia. The condition is also known as aspiration pneumonia, which results in difficulties of breathing. There are also some rare cases where the baby may suffer from additional birth defects along with the Esophageal Atresia (EA) or the Tracheoesophageal Fistula (TEF). The multiple organ defects are called the Vacterl. Vacterl is a medical condition associated with birth defects in the heart, kidney, anus, and limbs. Let us take the topic into more of its depth in the following paragraphs.
Classification and Types of Esophageal Atresia (EA) or Tracheoesophageal Fistula (TEF)
Birth defects such as EA/TEF are not detected during pregnancy and before the baby’s birth. Internal birth defects like EA and TEF cannot be diagnosed with radiological or ultrasound study of uterus and fetus. The infant during Intra uterus life receives oxygen and nutrients from the blood. The oxygen and nutrients from the mother are exchanged with fetal blood in the placenta. The oxygen and nutrients are transferred from mother to fetus blood at blood capillaries of the placenta. During growth in the uterus, the fetus does not eat or breath thus nothing passes through the esophagus to stomach nor air passes through the trachea. During the time of early fetal development, the two tubular structures such as the esophagus and the trachea originate side by side from common stem cells. Eventually, the esophagus and breathing passage are separated and do not maintain any connection below the pharynx. However rarely in a few cases, the connection between the esophagus and trachea remains as a fistula or tiny tubular connection. The association of esophageal atresia and tracheoesophageal fistula is presented as five major malfunction configurations. These five types or configurations are as follows.
- Type A: This type of configuration is seen in about 7.7% of the cases. Here in this case both the segments of the esophagus end in a blind pouch and neither of the segments is attached with the trachea. Esophageal atresia is not associated with tracheoesophageal fistula.
- Type B: Occurring in about 0.8% of the cases. Type B configuration occurs when the Esophageal atresia occurs with a conjugation of Tracheoesophageal fistula, where the upper segment of the esophagus forms a fistula with the trachea and the upper end of lower segment ends up in a blind pouch.1
- Type C: This is the most common form of configuration in EA where the upper segment of the esophagus is ended up in a blind pouch while the lower segment forms a fistula to the trachea. Around 86.5% of the cases hold Type C configuration in congenital defects of esophageal atresia and Tracheoesophageal fistula.
- Type D: The rarest form of Esophageal atresia or the Tracheoesophageal fistula where both the segments of the esophagus are attached to the Trachea by means of a fistula. It occurs in only 0.7% of the cases.
- Type E: The condition is presented with tracheoesophageal fistula otherwise normal esophagus connecting pharynx (mouth with stomach 4.2% of the cases presented with this type of anomaly. This is also called a Type H form.
Symptoms of Esophageal Atresia/Tracheoesophageal Fistula:
Major symptoms of this birth defect are summarized as follows-
Non-Specific Symptoms of EA/TEF-
- Restricted growth
- Lethargic child
- Dry skin
a. Symptoms of Atresia Resulting In Upper Esophageal Blind Pouch-
The following symptoms are observed when the upper half of the esophagus ends up in the blind pouch.
- Frothy White Mucous Oral Secretions- The infant though is able to swallow at first normally, however, there appears a kind of tiny, frothy and white color mucus bubbles in the mouth and sometimes also in the nose.
- Regurgitation of Content From Pouch- This happens due to the reflux and regurgitation of the ingested content, which is unable to pass beyond the esophageal pouch. The ingested milk or liquid is often mixed with mucus secretions.
b. Symptoms of Atresia Associated With Tracheal Fistula Connected With Upper Esophageal Blind Pouch-
- Frothy Secretions- Fistula is a tubular connection between the trachea and the esophagus. The air from the trachea passes through the fistula into the upper end of the esophagus and pouch. The frothy mucosal secretion is observed in the mouth and nose. The secretion becomes frothy because the trapped milk and liquid in the pouch is mixed with air transmitted from the trachea.
- Rattling Breathing- There may be a kind of rattling breathing sound heard in the baby’s chest, especially while drinking. Rattling sound occurs because of air transmission through fistula while fistula is filled with esophageal secretions. Rattling sound is also caused by inflammation and edema of bronchioli.
- Coughing and Choking- The noisy breathing is also accompanied by coughing and choking. The liquid passes from the esophagus to the trachea through the fistula. The presence of liquid in the trachea causes cough and choking.
- Short of Breath and Dyspnoea– Difficulty breathing starts appearing when the liquid passes from the esophagus to the trachea through the tracheoesophageal fistula. Short of breath is caused when the baby is struggling to breath. The inflammation of bronchioli and alveoli (lungs) caused by esophageal secretions in lungs results in swelling of smaller air passages (bronchioli). The bronchiolar mucosal edema or swelling causes difficulties in air transmission to alveoli or lung, which results in dyspnea or difficult breathing. Child tries to take deep breath each time child inhales air.
- Cyanosis- Cyanosis is caused by low blood oxygen. The secretion from esophagus enter into trachea through fistula and secretions spreads over the inner surface of lung or alveoli. The esophageal and gastric secretions causing bronchial and alveolar spasm, swelling and inflammation interferes with oxygen exchanged.
c. Symptoms of Atresia Associated With Upper Esophageal Blind Pouch and Fistula of Lower Esophageal Pouch –
- Frothy White Mucous Oral Secretions- The frothy secretions are observed in mouth and nasal passages.
- Regurgitation of Content From Pouch- Frequent regurgitation of milk or liquid is observed.
- Frequent Coughing- Symptom is caused by gastric secretion that passes through the fistula into trachea.
- Rattling Breathing- The gastric secretion causes bronchiolar inflammation and swelling resisting passage of air to alveoli.
- Short of Breath and Dyspnea- The low oxygen saturation in blood causes strenuous breathing.
Diagnosis of Esophageal Atresia (EA):
Examination of Esophageal Passage-
- The initial stage of diagnosing esophageal atresia begins by insertion of a long catheter through the nose into the esophagus.
- The distance from mouth to stomach is estimated by height and surface anatomy. The surgeon is unable to advance catheter beyond pouch, which is much shorter distance than to stomach.
- Usually the catheter would stop at 4 or 5 inches from the nostril in case there is EA present.
- Barium Study- Barium enhanced X-ray may reveal the dilated esophageal pouch. The pouch in few cases is substantially larger in size because of collection of amniotic fluid in the pouch during intrauterine fetal growth.
- Abdominal X-ray- This may show air in stomach when lower esophageal pouch is connected to trachea through fistula.
- Chest X-Ray- This may show signs of pneumonia and bronchitis.
- EKG may show increased heart rate because of low oxygen in blood.
- Blood oxygen and peripheral oxygen saturation is low. In few cases hemoglobin concentration is relatively high.
- Ultrasound of chest may show fistula as well as esophageal pouch.
- Tiny camera known as endoscope is passed through mouth into esophagus and trachea.2 The pediatric gastroenterologist can see the pharynx, esophagus and pouch. If upper esophagus is connected to fistula then fistula is also seen through the endoscope. The fistula attached to lower pouch cannot be seen through endoscope
Treatment of Esophageal Atresia and Tracheoesophageal Fistula
Esophageal atresia when associated with Tracheoesophageal fistula is usually a life threatening condition seen with 1 in 4000 babies. The combined birth defect of EA and TEF comprises difficulties in swallowing, digestions, breathing and thus may be highly dangerous, requiring an immediate medical care and attention. The most important treatment for EA/TEF usually involves surgery. The surgery usually can be done within 24 hours of the baby’s birth. However in case the baby has some other defects or infection along with esophageal atresia and may have some complication, then the surgery may be delayed for 3 months of age.
The disease is managed with medical treatment until baby is healthy enough to undergo surgery.
Medical Management of Esophageal Atresia-
- Frequent or Continuous Removal of Retained Secretions From Pouch- The mucus and saliva are continuously removed via a catheter. A tiny catheter is passed from the nose and tip is left in the pouch of proximal end of esophagus.
- Intravenous Feeding- Baby is fed through intravenous catheters. Nutrition and hydration are provided until the surgery is performed.
- Antibiotic Treatment- Infection is treated with antibiotics.
- Gastric Feeding- Baby is often fed through a tube, which is passed from outside directly into the stomach.
Surgical Treatments For Esophageal Atresia and Tracheoesophageal Fistula:
The surgery is done under general anesthesia.
Surgery For Esophageal Atresia With No Fistula-
- A tube is placed through the mouth into the esophageal pouch. All the secretions are aspirated prior to skin incision. In case the gap between the two ends of the esophagus is short then the surgeon may simply join the two ends, the procedure is known as anastomosis.
- In case there is a long gap between the two sections of the esophagus and it is difficult to reattach the two segments then the esophageal ends are first allowed to grow and then surgery is done.
Surgery For Esophageal Atresia Associated With Tracheoesophageal Fistula-
- The fistula is surgically exposed and two ends of fistula, which are attached to the trachea and esophagus are identified. Both ends are ligated with sutures. The closed sutures are applied to prevent any leak following surgery.
- Baby is often fed through a gastric tube after surgery. The tube is directly inserted into the stomach from the abdominal wall. The tube allows feeding of nutrition and fluid directly into the stomach.
Long-Gap Esophageal Atresia and the Foker’s Technique Treatment:
Foker surgical treatment is used to treat long gap Esophageal Atresia. The technique was developed by Dr. Foker and known as Foker’s Technique.3 Foker’s technique is the most advanced and effective form of treatment for long gap esophageal atresia. Published data suggest a success rate is near 100%. Foker’s surgical techniques are as follows-
- Long gap esophageal atresia is one of the rarest forms of EA birth defect where there is a very long or large gap between the two sections of the esophagus. It may be difficult to approximate the two segments via the usual surgical procedures used in the surgical treatment of the EA.
- Foker’s technique is an advanced treatment procedure developed by Dr. John Foker, MD, a Pediatric surgeon at the University of Minnesota.
- Dr. Foker modified the treatment to promote the rapid development of the esophageal pouch and ends. The procedure helps to make the upper and lower segment of the esophagus grow faster and come closer.
- The prime aim here is to get the two sections of the esophagus to come closer and then the two proximal ends are attached via anastomosis.
- During the growth period, the esophageal pouch is suctioned continuously.
- The procedure involves insertion and anchoring of the special stitches over both ends of the esophagus.
- The sutures or stitches are called traction sutures. The stitches are pulled with gentle pressure so as to promote both ends of the esophagus to come close to each other.
- The stitches come out of the abdominal skin and placed outside the body. The traction over the suture material allows pulling of the esophageal end towards each other.
Surgical Outcome- Surgical mortality is high if EA and TEF is associated with heart abnormality like ventricular septal defects.4
Complications or Risk Factors Involved With Foker’s Technique:
There are some of the complications involved in the technique. Let us take a look at them.
Bleeding- Surgery may cause bleeding and excessive loss of blood resulting in anemia. The treatment may require blood transfusion as well as re-exploration surgery to stop bleeding.
Infection- Surgery may be followed by an infection. The esophageal pouch and bronchitis may be a source of infection.
Leakage- The open end of the trachea and esophagus after removal of the fistula may leak in the mediastinum (chest cavity). The leak may cause infection and severe pain.
Additional Fistula Formation- Surgery may result in esophageal tissue trauma and the formation of a more esophageal fistula. The surgical incision and surgical field around the fistula are extremely small because of the size of the infant. The surgical field is often compromised by the use of surgical instruments and a pair of operating hands. Inadequate view of the surgical field may result in soft tissue damage. The damaged soft tissue once gets inflamed may result in fistula formations.
- Esophageal atresia with proximal tracheoesophageal fistula: a missed diagnosis. Parolini F1, Morandi A, Macchini F, Canazza L, Torricelli M, Zanini A, Leva E. J Pediatr Surg. 2013 Jun;48(6):E13-7. doi: 10.1016/j.jpedsurg.2013.04.018.
- Laryngotracheobronchoscopy prior to esophageal atresia and tracheoesophageal fistula repair–its use and importance. Sharma N1, Srinivas M2. J Pediatr Surg. 2014 Feb;49(2):367-9.
- Foker’s technique in oesophageal atresia with double fistula: a case report. Gaglione G1, Tramontano A, Capobianco A, Mazzei S. Eur J Pediatr Surg. 2003 Feb;13(1):50-3.
- A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. Wang B1, Tashiro J1, Allan BJ1, Sola JE1, Parikh PP1, Hogan AR1, Neville HL1, Perez EA2. J Surg Res. 2014 Aug;190(2):604-12.