Please note that all guidance is currently under review and some may be out of date. We recommend that you also refer to more contemporaneous evidence in the interim.
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.
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).
- Vertebral defects
- Anal (imperforate anus)
- Cardiac (VSD most common)
- ‘Esophagus’ (OA)
- Renal anomalies
- Limb deformities.
- congenital Heart disease
- choanal Atresia
- growth and mental Retardation
- Genital hypoplasia
- Ear anomalies.
- Trisomy 13, 18, 21 (Down syndrome).
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 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.
- Oesophageal Atresia Research Association (OARA) Australia
- Tracheo-Oesophageal Fistula Support (TOFS) - support for families of children unable to swallow
- 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.
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First published: September 2013
Last web update: October 2018
Review by: March 2020
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Page last updated: 17 Feb 2021