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Key messages

  • Oesophageal atresia (OA) involves an interruption to the lumen of the oesophagus, with the oesophagus ending in a blind pouch. OA is usually associated with a tracheo-oesophageal fistula (TOF), which is an abnormal connection between the oesophagus and the trachea.
  • Early diagnosis is important to minimise pulmonary complications.
  • Regular, thorough suctioning of the upper oesophageal pouch is required prior to definitive surgical repair.
  • Referral to PIPER and a tertiary neonatal surgical unit should be made as soon as possible.

Oesophageal atresia (OA) and tracheo-oesophageal fistula often occur together. Oesophageal atresia is when the oesophagus ends blindly and is not in continuity with the stomach. This means food cannot reach the stomach. The baby is at risk of aspirating feed and saliva. Tracheo-oesophageal fistula is an abnormal connection between the oesophagus and the trachea. Gastric fluid can reflux into the lungs via the TOF.

Oesophageal atresia and tracheo-oesophageal fistula are congenital defects. The cause is unknown; however OA & TOF often occur in conjunction with other anomalies.

Cause of oesophageal atresia and tracheo-oesophageal fistula

The cause of oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) is unknown. Disruption during development of the embryonic trachea and oesophagus in the fourth week of fetal life results in incomplete oesophageal development and the persistence of a connection (fistula) between the oesophagus and trachea.

Incidence of oesophageal atresia

Points about the incidence of OA:

  • The incidence of oesophageal atresia (OA) is approximately one in 3,000  births.
  • These infants are more likely to be premature because of the high association with polyhydramnios (excess amniotic fluid).
  • OA with a distal TOF is the most common variant of the disorder (approximately 85% of cases). This involves the oesophagus ending in a blind pouch, with a fistula connecting the distal oesophageal segment to the trachea, at or close to the carina.
  • Other uncommon variants include:
    • OA without a fistula
    • OA with a proximal TOF
    • OA with a double TOF
    • Isolated (H type) TOF.
Figure 1: Oesophageal atresia
Figure 1: Types of OA / TOF

 

Clinical signs of OA

Clinical signs of OA include:

  • antenatal polyhydramnios
  • often premature
  • excessive oral secretions, mucousy baby
  • respiratory distress and/ or cyanotic episodes
  • choking and regurgitation accompany feeding
  • abdominal distension (due to air transmitted through the distal fistula).

Associations

VACTERL associations:

  • Vertebral defects
  • Anal (imperforate anus)
  • Cardiac (VSD most common)
  • Tracheal
  • Esophagus’ (OA)
  • Renal anomalies
  • Limb deformities.
     

CHARGE associations:

  • Coloboma
  • congenital Heart disease
  • choanal Atresia
  • growth and mental Retardation
  • Genital hypoplasia
  • Ear anomalies.
     

Chromosomal anomalies:

Investigation and diagnosis

Issues related to the investigation and diagnosis for OA and TOF include:

  • Diagnosis may be suggested on antenatal ultrasound with polyhydramnios or the failure to visualise the fetal stomach.
  • If the diagnosis is suggested antenatally or there are clinical signs at birth, an attempt should be made to pass a firm suction catheter or feeding tube (size 10F is adequate) to the stomach before the first feed. The inability to pass the tube into the stomach confirms the diagnosis of OA. Most often the tube comes to a halt at about 9-13 cm from the gums (although this will depend on the size and gestation of the baby). A smaller, softer tube may curl up in the upper oesophageal pouch giving a false negative result.
  • A chest and abdominal x-ray taken with the catheter tip in the pouch will confirm the position of the upper oesophageal pouch and air within the bowel confirms the presence of a distal TOF. Contrast in the upper pouch is usually not required. The absence of air below the diaphragm excludes a distal fistula.
  • A H-type TOF often presents within the first few days of life with coughing or cyanosis with feeding. These infants may also present with respiratory symptoms due to aspiration of a feed. If an H-type TOF is suspected this is usually revealed by a contrast swallow at a Level 6 Neonatal unit.
  • An echocardiography will be required to exclude cardiac anomalies and to establish the position of the aortic arch.
  • A renal ultrasound should be performed if the neonate is anuric to exclude bilateral renal agenesis or a congenital anomaly.

Management

Management of oesophageal atresia and TOF involves:

  • If oesophageal atresia is suspected antenatally baby should be delivered close to a Level 6 surgical neonatal unit.
  • The neonate must be kept nil by mouth, commenced on intravenous fluids and nursed supine in a head up position (approximately 30-60 degrees).
  • The upper pouch must be kept clear of secretions by frequent oral suctioning to 1 cm above the distal end of the oesophageal pouch (every 15 minutes or more frequently as required). Time between suctioning should not exceed 30 minutes due to the risk of aspiration of saliva.
  • Another method of oesophageal pouch suction is to position a Replogle tube 0.5 cm above the end of the oesophageal pouch and connect the Repogle tube to continuous low pressure suction (10 cm H2O) to aspirate saliva and prevent aspiration. To maintain the patency of the Repogle tube and facilitate the removal of thick, tenacious secretions, instill 0.5-1 ml of normal saline (0.9% sodium chloride) every 15-30 minutes and ensure return of the normal saline.
  • Antibiotics are recommended if there is evidence of aspiration pneumonia or for presumed sepsis.
  • The patient should be transported to a Level 6 surgical neonatal unit as soon as possible. In most cases early primary surgical repair is appropriate.
  • Babies with an oesophageal atresia without TOF (pure OA) or those with OA and a proximal TOF usually have a large gap between the ends of their oesophagus. Surgery to repair the OA may involve a delayed oesophageal repair or an oesophageal replacement procedure.
  • A multidisciplinary approach, including surgeon, respiratory physician, physiotherapist, dietitian and speech therapist is needed.
  • The presence of coexisting severe anomalies or extreme prematurity may impact on the likelihood of survival.

More information

Clinical

Parent information

References

  • Blake, K.D., & Prasad, C. (2006). CHARGE Syndrome. Orphanet Journal of Rare Diseases. 1:34.
  • Gwee, A., Rimer, R., & Marks, M. (2015). Paediatric Handbook. (9th Ed.). Oxford, UK: Wiley-Blackwell.
  • Hawley, A.D. (2001). Long-gap oesophageal atresia - a nursing perspective. Journal of Child Health Care. 5(1), 19-25.
  • Hudson, J.M., O'Brien, M., Beasley, S.W., Teague, W.J., & King, S. (2015). Jones' Clinical Paediatric Surgery. (7th Ed.). Oxford, UK: Wiley-Blackwell.
  • Spitz, L.  2007. Oesophageal atresia. Orphanet Journal of Rare Diseases. 2:24.

Get in touch

Maternity and Newborn Clinical Network
Safer Care Victoria

Version history

First published: September 2013

Last web update: October 2018

Review by: March 2020

Uncontrolled when downloaded

Page last updated: 23 Nov 2018

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