Trachea – Esophageal Atresia

Subject: Child Health Nursing

Overview

One of the severe forms of congenital malformation of gastrointestinal tract represents a failure of the esophagus to develop as a continuous passage and failure of the trachea and esophagus to separate into distinct structures. This defect may occur as separate entities or in combination with cardiovascular, renal, anorectal and radius deformities.An exact cause of the malformation is unknown, however, some association are most affected newborn have significant low birth weight and may have maternal polyhydramnios during pregnancy.Choking and coughing on feeds, may associate with cyanosis, respiratory distress, distended abdomen in case of distal fistula and scaphoid abdomen if no fistula.Surgical correction is done as soon as possible after birth. Surgical management depends on types on the anomaly, other associated anomalies, the general condition of the infant. Common surgical procedures are Thoracotomy with separation of the trachea with esophagus and ligation of fistula and end anastomosis of an esophagus.

One of the severe forms of congenital malformation of the gastrointestinal tract represents a failure of the esophagus to develop as a continuous passage and a failure of the trachea and esophagus to separate into distinct structures. This defect may occur as separate entities or in combination with cardiovascular, renal, anorectal, and radius deformities.

Types of Trachea Esophageal Atresia

  • Esophageal atresia without fistula: the upper part of the esophagus end blindly. There is no connection between the esophagus and the trachea. This is known as a pure type of esophageal atresia.
  • Proximal trachea- esophageal fistula with esophageal atresia
  • Distal tracheoesophageal fistula atresia: This is the most common type of disorder seen in more than 80% of cases. The upper part of the esophagus ends blindly and the lower part is connected to the trachea or primary bronchus by a fistula.
  • Proximal and distal fistula with esophageal atresia.
  • The trachea-esophageal fistula without atresia (H-type fistula): The less common type where the normal trachea and esophagus are connected by a fistula referred to as the 'H' type.

Causes of Trachea Esophageal Atresia

The exact cause of the malformation is unknown, however, some association is following;

  • Most affected newborns have significantly low birth weight.
  • May have maternal polyhydramnios during pregnancy.
  • May be present with other malformations e.g. abnormalities of vertebra, colorectal, cardiovascular, renal, and limb.

Pathophysiology

The esophagus and trachea both develop from the primitive foregut. During the 4th – 5th weeks of gestation, the foregut normally lengthens and separates longitudinally and fuses to form the parallel channel (esophagus and trachea) that is joined only at the larynx. The abnormality is caused by defective separation, incorrect fusion, or altered cellular growth.

Clinical manifestation

  • Continuous drooling and frothing of saliva from the mouth and nose that is excessive in amount.
  • Choking and coughing on feeds, may associate with cyanosis, respiratory distress, and distended abdomen in case of distal fistula and scaphoid abdomen if no fistula.
  • Regurgitation of food through nose and mouth.
  • Aspiration
  • The presence of a single umbilical artery.
  • In type, C stomach becomes distended with air especially during crying causing regurgitation of gastric contents through the fistula into the trachea.

Diagnostic evaluation

  • Presence of clinical manifestation
  • Presence of maternal polyhydramnios
  • X – ray detects blind pouch of esophagus, presence of gas in stomach
  • USG is also useful to identify the type of anomaly.

Management and treatment

Surgical management

Surgical correction is done as soon as possible after birth. Surgical management depends on types of anomalies, other associated anomalies, and the general condition of the infant. Common surgical procedures are:

  • Thoracotomy is performed in which, division and ligation of the trachea-esophageal fistula and an end-to-end anastomosis of the esophagus will be done a chest tube is placed for drainage.
  • Cervical esophagostomy: - makes stoma in the neck and allows the drainage of saliva through a stoma.
  • If multiple anomalies, gastrostomy, with ligation of TEF and constant drainage of an esophageal pouch. Anastomosis is postponed till the upper part is elongated.
  • Endotracheal intubations (supportive care)

Nursing management

Preoperative care

  • Keep the newborn in 30o-40o head up position.
  • Frequent suctioning of the upper pouch immediately after diagnosis.
  • Trachea-bronchial aspiration is done several times/day through an ET tube.
  • Administer oxygen through the ET tube to correct hypoxia.
  • Keep NPO
  • Keep the child in a radiant warmer or incubator to prevent hypothermia
  • Administer I\V fluid and electrolytes to correct hypoglycemia and hypovolemic
  • Continue to the monitoring of the infant's general condition, vital signs, and oxygen saturation

 Postoperative care

  • Ensure the ET tube in the proper place
  • Do not extend the neck
  • Suction the oropharynx using a soft catheter carefully to avoid injury to the incision area. Insert catheter up to the measured mark (usually 7 cm 0 only)
  • Check the inter-coastal thoracic drainage
  • Position change 2 hourly
  • Baseline observation: vital signs, oxygenation, and respiratory status
  • Chest physiotherapy
  • Daily x-Ray of chest
  • Keep the child in radiant warmer\incubator
  • Keep gastrostomy tube on gravity drainage
  • Feeding is started after the removal of chest drainage and confirming the integrity of the esophagus. Start oral feeding with sterile water followed by frequent breastfeeding.

 
Things to remember
  • One of the severe forms of congenital malformation of gastrointestinal tract represents a failure of the esophagus to develop as a continuous passage and failure of the trachea and esophagus to separate into distinct structures.
  • This defect may occur as separate entities or in combination with cardiovascular, renal, anorectal and radius deformities.
  • An exact cause of the malformation is unknown, however, some association is most affected newborn have significant low birth weight.
  • Choking and coughing on feeds, may associate with cyanosis, respiratory distress, distended abdomen in case of distal fistula and scaphoid abdomen if no fistula.
  • Thoracotomy with separation of the trachea with esophagus and ligation of fistula and end anastomosis of an esophagus.
Videos for Trachea – Esophageal Atresia
Tracheoesophageal Fistula Repair
Questions and Answers

One of the most severe types of gastrointestinal congenital malformation is the inability of the esophagus to form as a continuous channel and the trachea and esophagus to divide into separate structures. This deficiency may manifest alone or in conjunction with malformations of the heart, kidneys, anorectal region, and radius.

 

Types:

  • Esophagus atresia without fistula:
    • When there is no fistula in the esophagus, the upper portion of the esophagus ends blindly. The esophagus and the trachea are unrelated. This is referred to as an esophageal atresia of pure type.
  • Proximal trachea:
    • Fistula between the esophagus and the proximal trachea.
  • Distal trachea: 
    • More than 80% of patients with distal trachea-esophagus fistula and esophageal atresia have this condition. The lower section of the esophagus is attached to the trachea or primary bronchus by a fistula, and the upper part of the esophagus ends blindly.
  • Trachea esophagus fistula without atresia (H-type fistula):
    • Without atresia, a fistula connecting the normal trachea and esophagus is known as a "H-type fistula" and is a less common condition.

Management and treatment

Surgical management:

Surgery to address birth defects is performed as soon as feasible. Surgical care is based on the type of aberration, any accompanying anomalies, and the infant's overall health. Typical surgical techniques include:

  • Thoracotomy with trachea and esophagus separation, fistula ligation, and end anastomosis of an esophagus.
  • Repair of the esophagus utilizing a portion of the colon if the two segments are not long enough.

Nursing management

Preoperative care:

  • Keep the infant's head in a 30° to 40° upward position.
  • As soon as the diagnosis is made, the upper pouch is frequently suctioned.
  • Through an ET tube, trachea-bronchial aspiration is performed numerous times per day.
  • To treat hypoxia, provide oxygen through an ET tube.
  • Hold NPO
  • To prevent hypothermia, keep the kid in an incubator or radiant warmer.
  • I.V. hydration and electrolytes should be given to treat hypoglycemia and hypovolemia.
  • Continue to keep an eye on the baby's vital signs, oxygen saturation, and general health.

Postoperative care:

  • Place the ET tube properly.
  • Avoid extending your neck.
  • To prevent damaging the incision area, carefully suction the oropharynx using a soft catheter up to the measured mark, insert the catheter (usually 7 cm 0 only).
  • Examine the thoracic intercoastal drainage.
  • 2 hourly position changes.
  • Initial assessment: respiratory state, oxygenation, and vital signs.
  • Therapy for the chest.
  • Daily chest x-ray.
  • Keep the infant in the incubator/radiant warmer.
  • Gastrectomy tube should remain on gravity drainage.
  • After the chest drainage has been removed and the integrity of the esophagus has been verified, feeding can begin. Start oral feeding with sterilized water and then nurse frequently after that.
  • Continuous profuse drooling and foaming of saliva from the mouth and nose.
  • On feedings, choking and coughing may be accompanied by cyanosis, respiratory distress, a swollen belly in the event of a distal fistula, or a scaphoid abdomen in the absence of a fistula.
  • Food vomiting from the mouth and nose.
  • Aspiration.
  • A single umbilical artery is present.
  • In type C, the stomach swells up with air, especially when the patient is crying, which causes the stomach's contents to reflux into the trachea through the fistula.

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