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Child Health Guideline Identifier

Oesophageal atresia (OA) with/without tracheo-oesophageal fistula (TOF)

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All congenital anomalies are rare and OA/TOF, despite being one of the relatively more common anomalies, still only has a population prevalence of 1 in 3000 live births2.

Antenatal

Although prenatal diagnosis can be challenging, typical findings are small or absent gastric bubbles usually in association with polyhydramnios (67% of those diagnosed prenatally)1. However, polyhydramnios in itself is a frequent finding on antenatal scanning (10%) and has a spectrum of possible causes.

Care at birth

The diagnosis should be suspected in a baby that is mucousy, has cyanotic episodes (due to aspiration of saliva which has accumulated in a blind upper pouch), had polyhydramnios or has other anomalies, e.g. imperforate anus, radial dysplasia.

When the diagnosis is suspected we recommend gently attempting to pass a 10Fr orogastric tube. Arrest of this tube at approximately 10cm from the incisors confirms the diagnosis3.

Figure 1

Figure 1

Plain film showing malposition of the orogastric tube within the upper oesophageal pouch which confirms the diagnosis of oesophageal atresia. The tube can curl up in the upper pouch giving a false impression of oesophageal continuity. The presence of gas-filled normal appearing bowel distally confirms the presence of a distal tracheo-esophageal fistula (from the lower oesophageal pouch).

Transfer of the child with oesophageal atresia

The over-riding principle of transport of the newborn is stabilization before transport, followed by supervision of transport by specialist nursing and medical personnel with appropriate equipment.

Special considerations include3:

  • Posture: the infant with oesophageal atresia should be nursed in the right lateral position and head up. The rationale for this is to encourage gastric emptying through the pylorus, improve effective ventilation and allow spontaneous clearing of the airway.
  • Care of the upper pouch: The upper oesophageal pouch needs to be cleared of saliva every 10-15 minutes. Saliva may accumulate 'silently' in large volumes and cause regurgitation and aspiration. This may also compress the trachea particularly in the context of associated tracheomalacia. Suctioning needs to be with a soft tube and performed gently to minimise mucosal damage.
  • Gentle handling: All care should be taken to minimize crying as this tends to fill the stomach with air via the fistula impairing ventilation.
  • Minimal pressure ventilation: If ventilation is required the minimum pressure required should be used to discourage gastric distension.

Figure 2: Illustration of two pathophysiological consequences of the distal tracheo-oesophageal fistula.

Figure 2
1. High ventilation pressures encourage air to pass through the distal fistula causing gastric distension and diaphragmatic splinting. Passage of air through the distal fistula also diminishes the effective tidal volume. 2. Inadvertent feeding or overflow of secretions can cause aspiration. Furthermore, retrograde flow of gastric juices through the distal fistula can lead to soiling of the lungs.

Diagnosing associated anomalies

In 50% of cases OA/TOF can be associated with other congenital anomalies or syndromes: such as VACTERL association, CHARGE association and chromosomal anomalies e.g. Trisomy 182. The incidence of anomalies are provided below4:

Anomaly Frequency
Congenital heart disease 25%
Urinary tract 22%
Orthopaedic (vertebral, radial) 15%
Gastrointestinal (e.g. duodenal atresia, anorectal malformation) 22%
Chromosomal (usually trisomy 18 or 21) 7%
Total with 1 or more associated anomalies 58%

The relevance of these associated anomalies and the timing of investigation need to carefully considered:

Anomalies Relevance
Trisomy 18, bilateral renal agenesis Incompatible with survival, definitive surgery not indicated
Duct-dependent congenital heart disease Prostaglandin E1 commenced pre-operatively. If neonate remains unstable the heart may take priority over oesophageal repair 
Right-sided aortic arch May affect operative approach to reconstruction
Duodenal atresia and anorectal malformations Demands treatment early following closure of tracheo-oesophageal fistula, needs to be co-ordinated with treatment of oesophageal atresia 

In this regard there are two important nursing observations:

  • That the neonate has passed urine is important to rule out bilateral renal agenesis.
  • That the neonate has passed meconium and the anus is normally placed and patent.

Post-operative management

The primary objective of surgery is to ligate the communication between the oesophagus and airway (to prevent lung soiling and air leak from ventilation).

The secondary objective is to reconstitute oesophageal continuity.

Primary oesophageal repair is usually possible - the exception is cases where the two ends of the oesophagus are too far apart to anastomose safely (long-gap oesophageal atresia). In these cases a gastrostomy is placed.

Figure 3: Bedside instructions in a baby following oesophageal atresia repair.

Figure 3

Following reconstruction of the oesophagus a trans-anastomotic (TAT) tube is placed. This must not be dislodged in the post-operative period as replacement of it can cause damage to the repaired oesophagus.

Timing of extubation needs to be carefully considered as attempts at reintubation can cause disruption of the ligated tracheo-oesophageal fistula.

Feed post-operatively and the timing of a contrast study will be recommended by the paediatric surgical team.

Completion of work-up for any associated anomalies needs to be completed once the neonate has recovered from surgery (ECHO, renal ultrasound, spinal ultrasound).

The nursing goals in long-gap oesophageal atresia are: prevention of aspiration and nursing of the upper pouch as pre-operatively, provision of optimal nutrition including care of the gastrostomy, facilitate normal growth and development and encourage the parents to be involved in the daily care of the infant.

Prognosis, complications and long-term care

The two major determinants of outcome are birth weight and the presence of co-existing cardiac disease:

Spitz classification - based on birth weight and presence of cardiac disease
1. BW>1.5kg, without major cardiac disease - 97% survival
2. BW<1.5kg OR major cardiac disease - 59% survival
3. BW <1.5kg AND major cardiac disease - 22% survival

Early post-operative complications include anastomotic stricture (40-60%), anastomotic leak, recurrent tracheo-oesophageal fistula and vocal cord paresis6.

With regard to long-term outcomes the interplay between gastro-oesophageal reflux and/or tracheomalacia can cause significant morbidity, including acute life-threatening events. These problems often require medical and/or surgical management.

References

  1. Bradshaw CJ, Thakkar H et al. Accuracy of prenatal detection of tracheoesophageal fistula and oesophageal atresia. Journal of Pediatric Surgery. 2016 Aug 31;51(8):1268-72.
  2. Spitz L. Oesophageal atresia. Orphanet J Rare Dis 2007;2:24.
  3. Myers NA, Beasley SW, Auldist AW. In: Oesophageal Atresia 1991:211-228. Springer US.
  4. Beasley SW. Chapter 65: Esophageal Atresia and Tracheoesophageal Fistula. In: Oldham KT, Colombani PM, Foglia RP, Skinner MA. Principles and Practice of Pediatric Surgery.
  5. Spitz L. Esophageal atresia: lessons I have learned in a 40-year experience. Journal of Pediatric Surgery. 2006 Oct 31;41(10):1635-40.
  6. Teague WJ, Karpelowsky J. Surgical management of oesophageal atresia. Paediatric Respiratory Reviews. 2016 Jun 30;19:10-5.

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Document Control

  • Date last published: 28 November 2017
  • Document type: Clinical Guideline
  • Services responsible: Paediatric Surgery
  • Author(s): Kiarash Taghavi
  • Owner: Steve Evans
  • Editor: Greg Williams
  • Review frequency: 2 years

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