Respiratory Problems in Children with Oesophageal Atresia

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Respiratory Problems in Children with Oesophageal
Atresia

• Dr Adam Jaffe
• Consultant and Honorary Senior Lecturer
• Great Ormond Street Hospital for Children and
  Institute of Child Health

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Summary

• Embryology
• Acute respiratory presentation
• Etc..

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Embryology

• Median phayngeal groove develops at 22 days and
  develops into respiratory and digestive tract
• Mesenchymal proliferation separates the tubes
• Excess tissue growth incorporates oesophagus into
  trachea and can cause atresia

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Acute Presentation

• Neonatal period respiratory distress
• Upper pouch secretions overflow into trachea
• Gastric juice reflux via fistula to lungs
• Gastric distension causes splinting of diaphragm
• Tachypnoea
• Dyspnoea
• Pneumonia

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Not necessary to use contract medium. If going
to use water soluble
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Associated Respiratory Anomalies

• Tracheal bronchus

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TOF (posteriorly) Short segment of complete
tracheal rings above carina (anteriorly)
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Associated Respiratory Anomalies

• General foregut malformations
• Pulmonary and lobar agenesis
• Horseshoe lung
• Pulmonary hypoplasia

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Respiratory Complications
Chetcuti et al Arch Dis Child 199368167
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Trachea

• Retains U shaped configuration rather than C
  shape
• Ciliated cells replaced by squamous ? increased
  risk of infection
• Tracheomalacia clinically significant 10-
• 20 Spitz L. Curr Opin Paediatr
  19935347
• Rarely associated with apnoeas

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Normal Trachea
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Tracheomalacia

• Cough may persist into adulthood
• Worsens with URTIs and GOR
• May contribute to
• sputum retention
• reduced cilia
• Atelactasis
• Recurrent pneumonia

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Tracheomalacia-Treatment

• Improves with age
• Other options are
• Aortopexy
• External splints
• Palmaz expandable stent
• Tracheostomy and CPAP
• Reserved for severe end of spectrum
• Careful of bronchodilators ? worse

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PEP mask
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Recurrent TOF

• 9
• 2-18 months post repair
• Usually in pouch of original fistula
• Ligation rather than complete division of
  original fistula increases incidence of
  recurrence
• Presentation
• Cough
• Choking
• Recurrent pneumonia

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Aspiration over the top

• Oesophageal peristalsis abnormal 75-100
• Immotile segment commonly observed
• May avoid meat
• Dysphagia common (up to 92 adults)
• Aspiration is a real problem
• Long segment atresia with delayed closure and
  cervical stoma at very high risk of aspiration

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Gastro-oesophageal reflux

• Up to 58 children
• Apnoea
• Wheeze due to reflux reflex
• Bronchial hyperreactivity
• Aspiration

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Wheezing

• 43 older children with OA/TOF had wheeze
• 2/3rd of these had asthma
• But.25 responded to bronchodilator suggesting
  asthma overdiagnosed
• Agrawal et al Arch Dis Child 199981404

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Bronchial Reactivity

• Abnormally high prevalence
• Likely due to aspiration rather than atopy
• Abnormal bronchoconstriction or response to
  bronchodilator 33-65
• Careful of bronchodilators ? tracheomalacia
  worse due to smooth muscle relaxation

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Lung Function

• Put in tables from Kovesi

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Chest wall deformity

• 16 isolated asymmetry
• 4 anterior chest deformity and scoliosis
• 19 had associated vertebral abnormalities
• Cudmore. In Lister, Irving eds. Neonatal
  Surgery, 3rd ed, London 1990231

VACTERL syndrome
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Summary of Respiratory Complications

• 46 respiratory complications
• 19 recurrent pneumonia
• 10 aspiration
• 13 choking/gagging
• 1 adults have bronchiectasis
• Causes
• GOR in 74
• Tracheomalacia 13
• Recurrent TOF 13
• Oesophageal stricture 10

Delius et al Surgery 1992112527
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Conclusions

• Respiratory problems common
• Mostly improve with time
• Look for GOR
• Assess swallowing
• Remember to consider recurrence