TEF and Associated Anomalies: A Comprehensive Clinical Insight

1. Introduction of OA and TEF:

• · VACTER acronyms used to described associated abnormalities- vertebral defects, imperforated anus, cardiac defects, and renal congenital anomalies.

  ·  Approximately 50% of the cases of OA and TEF are a component of VACTER.

  ·  At 4^th or 5^th weeks of gestation, separation of respiratory tract from the esophagus

2. What is the meaning of tracheoesophageal fistula and esophagus atresia:

• · Tracheoesophageal fistula (TEF) is a congenital or acquired abnormal communication between the trachea and the esophagus. 

  · Esophagus is the failure of the esophagus to continuous canal from pharynx to stomach during embryonic development.

3. Incidence of tracheoesophageal fistula and esophagus atresia:

• ·  1 in 4000 affected neonates

  ·   Equal sex incidence

  ·   A history of maternal polyhydramnios is present in approximately 50% of infants with defects

  ·   More common in premature and LBW babies.

4. Etiology of tracheoesophageal fistula and esophagus atresia:

• ·       Exact cause is unknown

  ·       Heritable and genetic factor

  ·       Teratogenic factor

  ·       Abnormality in intrauterine environment

  ·       Teratogenic infection

5. Assessment:

· History of polyhydramnios during pregnancy.

· Inability to handle oral secretions.

· Cyanosis during feeding.

· Resistance encountered during the passage of a feeding tube.

· Persistent choking while feeding.

6. Types of OA and TEF: There are five recognized types of TEF:

1.Oesophageal atresia without fistula:

• · Second most common types of OA and TEF (5-8%).
• · Proximal and distal segment of the oesophagus are blind; associated with a small stomach and a gasless abdomen on X-ray.
• · There is no fistula communication between either upper and lower part of oesophagus and trachea.

2.Oesophageal atresia with a proximal tracheoesophageal fistula (1%).

• · Most dangerous type of OA and TEF.
• · Upper segment of oesophagus is connected to the trachea and lower segment is blind/Separate.
• Gasless abdomen on X-ray.
• · Rarely found.

3.Oesophageal atresia with distal tracheoesophageal fistula:

• · Most common types of OA and TEF (86%).
• · The proximal segment of oesophagus has a blind pouch in the upper mediastinum and the distal segment      of oesophagus is connected to the tracheobronchial tree by a fistula.
• Abdominal distension is observed due to the presence of gas in the abdomen.

4.Reverse ‘K’ shape: (1%)

• · The proximal and distal segments of the esophagus are both connected to the trachea, forming a                 configuration that resembles a reverse “K” shape.

5.Isolated TEF:(4-5% cases)

• · The oesophagus is continuous but connects to the trachea via a fistula, known as the “H-type” tracheoesophageal fistula.
• · No atresia of the oesophagus
• · Abdominal distension is observed due to the presence of gas in the abdomen.

7. Sign and symptoms:

• · Excessive salivation and drooling.
• · Excessive oral secretions at birth due to the infant’s inability to swallow saliva.
• · The classic three C’s of TEF:
  • Coughing
  • Choking
  • Cyanosis during the first feeding
• · Non bilus regurgitation vomiting.
• · Abdominal distension
• · Poor feeding
• · Increased respiratory distress during feeding

8. Sign and symptoms:

• · Diagnosis is primarily established based on clinical manifestations and is often suspected in cases of  maternal polyhydramnios.
• · Antenatal and postnatal ultrasonography (USG) may provide supportive findings.
• · Plain chest and abdominal X-rays are helpful in detecting air patterns and confirming the diagnosis.
• · Inability to pass a plain catheter through the nose or mouth into the stomach indicates the presence of a blind pouch, suggestive of oesophageal atresia.

9. Treatment:

• · Treatment includes maintenance of a patent airway, prevention of pneumonia and surgical repair.

• Immediately After Birth

  • ·  Keep the infant NPO (nothing by mouth) as soon as the condition is suspected.
  • ·  Insert a nasogastric tube into the upper oesophageal pouch and connect to low suction to remove secretions.
  • ·  Elevate the head to reduce aspiration risk.

  Definitive Treatment

  ·  Surgical repair of the fistula is usually performed within hours or days after birth to prevent aspiration of gastric contents into the respiratory tract.

1. 1. First Stage Surgery:
   • · Surgical correction involves end-to-end oesophageal anastomosis along with excision of the fistula.
   • · This procedure is typically performed when the gap between the oesophageal segments is less than 2.5 cm and the baby is in good clinical condition.
   2. Staged Surgical Approach (Two-Stage Repair)
   • · In cases where the baby’s condition is not stable or the gap between the oesophageal segments is more than 2.5 cm, a staged surgical approach is followed.
   • · In the first stage, a gastrostomy is performed to allow for feeding and gastric decompression.
   • · After about one year, when the child’s condition improves and growth has occurred, definitive esophageal anastomosis is performed to connect the esophageal segments.

9. Treatment:

• Preoperative care:

  • · Proper suctioning, adequate hydration, and administration of antibiotics are essential to prevent aspiration pneumonia.
  • · Maintain IV access for fluids and antibiotics.
  • · Monitor for respiratory distress and provide oxygen support if needed.

  Postoperative Care:

  • · Assess vital signs regularly to monitor for signs of shock.
  • · Evaluate for respiratory distress, signs of infection, and monitor hydration status.
  • · Educate parents about:
    • Gastrostomy tube care
    • Proper suctioning techniques
    • Safe and effective feeding practices

• Gastrostomy Tube Management

  • · If a gastrostomy tube is inserted, it may be left open to allow air entering the stomach through the fistula to escape, thereby minimizing the risk of gastric content regurgitation.
  • · During and after feeding, the gastrostomy tube should be elevated to facilitate the passage of gastric secretions into the duodenum.
  • · Before removing the gastrostomy tube, confirm the integrity of the esophageal anastomosis by performing a barium X-ray study.

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References (Vancouver Style)

1. Paul VK, Bagga A. Ghai Essential Pediatrics. 9th ed. New Delhi: CBS Publishers & Distributors; 2019.
2. Hockenberry MJ, Wilson D. Wong’s Essentials of Pediatric Nursing. 8th ed. St. Louis: Mosby Elsevier; 2009.
3. Kliegman RM, Stanton B, St. Geme JW, Schor NF. Nelson Textbook of Pediatrics. 20th ed. Philadelphia: Elsevier; 2016.

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