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The TOF Cough – Tracheomalacia

Mild tracheomalacia

The normal trachea (windpipe or airway) is held open with horseshoe-shaped rings of cartilage which maintain structural rigidity and keep the airway open – a bit like the stiff rings that keep the pipe open on vacuum cleaners.

Babies with OA/TOF tend to have soft tracheal cartilages in the region of the TOF and this is called tracheomalacia.

Tracheomalacia causes the characteristic TOF cough that virtually all children have after OA/TOF repair. 

The forceful effort required to cough causes the soft/floppy segment of the trachea to close slightly, which causes an unusual sound from the airway.

  • Children with mild tracheomalacia tend to have more than their fair share of chest infections in early life, but otherwise do well.

  • The sound of the TOF cough may however continue into adulthood.

Watch “The TOF Cough” with Professor Alyn Morice.

Severe tracheomalacia

Severe tracheomalacia (softening of the cartilage rings in the trachea so that the airway is prone to collapsing) is uncommon  but potentially life-threatening. It becomes obvious during the first few months of life.

When a baby with severe tracheomalacia is distressed or crying, the floppy trachea can narrow so much that the baby stops breathing and goes blue(apnoea).

Sometimes this occurs during feeding but more usually when the baby gets upset.

Tracheomalacia tends to improve as babies grow and it is very uncommon for severe tracheomalacia to become a problem over the age of one year.

Severe tracheomalacia with the baby turning apnoeic (stopping breathing) and blue (due to lack of oxygen) is serious and very frightening for the parents.

The best treatment is an operation called an aortopexy.

  • Around 1 in 20 OA/TOF babies require aortopexy.
  • The operation is usually performed at a few months of age.
  • An aortopexy reduces the severity of tracheomalacia but it is important to remember that it cannot completely cure tracheomalacia. The operation is however usually sufficient to prevent blue spells.


Occasionally an aortopexy will not prevent blue spells.

When this happens the baby may need a tracheostomy (a breathing tube which is placed into the trachea through the baby’s neck).

This is effective, but has drawbacks.

As the baby grows, the trachea enlarges and the tracheomalacia improves. It is normally possible to remove the tracheostomy (decannulate) after one to two years.

Would you like more information?

Published by TOFS, The TOF Book is the must have guide for anyone affected by, or caring for someone with OA/TOF.

With contributions from medical experts, the TOF Book contains chapters on every aspect of OA/TOF and VACTERL condition, from infancy to adulthood all presented in an easy-to-understand format.