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Laryngomalacia, Tracheomalacia and Bronchomalacia

Pediatric doctor examines infant with larynomalacia

Laryngomalacia

What is laryngomalacia?

Laryngomalacia is a disorder that effects the larynx (voice box) of infants. Laryngomalacia most often presents as noisy breathing or stridor as early as 2 weeks of age. Infants with laryngomalacia will have stridor and may also have difficulty in breathing, feeding and gaining weight, failure to thrive and respiratory distress. This can lead to poor quality of life and anxiety in parents.

The larynx of a child with laryngomalacia will demonstrate one or all of the following:

  • Excess tissue overlying the arytenoid cartilages (cartilage above the voice box) with evidence of arytenoid cartilages falling into the airway (arytenoid prolapse).
  • Short aryepiglottic folds (AE folds).
  • Omega-shaped epiglottis and prolapse of the epiglottis into the airway.

This prolapse of airway structures during inspiration is what leads to airway distress in children with laryngomalacia. The noisy breathing in these children is caused by increased resistance of air flowing though the larynx due the tissue blocking the airway.

Causes

The exact cause of laryngomalacia is not known. The underlying dysfunction is related to redundant mucosa, poor support of laryngeal cartilages and poor neurological tone. The most widely accepted theory describes laryngomalacia as a delay in neurological function and tone of the laryngeal structures above the vocal cords.

Treatment

First line treatment for laryngomalacia includes positioning and treatment for gastric reflux. Typically, parents will be instructed to position their child on their stomach when they are having difficulty breathing. It is important never to leave a baby alone when they are on their stomach. Eating is typically easier for babies with laryngomalacia if they are sitting up. Babies with laryngomalacia will often be prescribed medication to decrease gastric reflux. If these conservative measures do not help or if the baby has severe laryngomalacia, surgery may be necessary.

Surgery

Most of the time, laryngomalacia will resolve at 12 to 18 months of age without surgical intervention. Surgical intervention is warranted if a baby has severe laryngomalacia with recurrent apneic events, difficulty with feeding, failure to thrive and recurrent cyanosis (bluish discoloration of the skin) and respiratory distress.

A supraglottoplasty is a surgical technique in which the structures of the larynx are altered in order to prevent collapse of the airway and improve breathing. While the baby is asleep, the surgeon will use a laryngoscope to look inside the mouth and evaluate the larynx. The surgeon will then alter the airway to decrease obstruction caused by laryngomalacia. Short aryepiglottic folds are cut in order to lengthen them and provide a more open airway. Excess arytenoid tissue can be trimmed down so it no longer prolapses into the airway. A third technique that is performed is called an epiglottopexy. This technique attaches the epiglottis to the base of tongue to prevent the epiglottis from falling into the airway.


Tracheomalacia

What is tracheomalacia?

Tracheomalacia is a condition where the tracheal cartilage (windpipe) rings soften and partially collapse when a child is breathing. This collapse most often occurs during expiration. Tracheomalacia may be primary or secondary. Primary tracheomalacia is caused by anterior prolapse of the posterior tracheal wall, while secondary tracheomalacia (rare) is caused by external compression. This external compression is most often caused from a vascular anomaly where an artery compresses and weakens the tracheal cartilage leading to collapse. Examples of vascular malformations that can cause this problem include aberrant right subclavian artery, pulmonary artery sling and vascular rings and slings. In most cases, this is managed without surgical intervention.


Bronchomalacia

What is bronchomalacia?

Bronchomalacia is very similar to tracheomalacia, however in bronchomalacia, the cartilage softening occurs in the bronchi of the lungs. Bronchi are branches from the trachea and have cartilage that can become soft and collapse. When this collapse occurs in the bronchi, it is called bronchomalacia. Bronchomalacia can also be primary or secondary.


How common is laryngomalacia, tracheomalacia and bronchialmalacia?

Laryngomalacia is very common. It is the most common cause of stridor in children and affects 35-75% of infants presenting with stridor. Tracheomalacia and bronchomalacia are less common. Estimated incidence of tracheobronchomalacia is around one in 2,100.


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