Tracheomalacia

Definition
Tracheomalacia is a weakness and floppiness of the walls of the trachea (main airway). There are three types of tracheomalacia. One type is congenital (present at birth), while the others are acquired, meaning that the trachea is normal at birth but becomes floppy later in life in response to something else.

Causes, incidence, and risk factors

Tracheomalacia in an infant occurs when the cartilage in the trachea fails to develop properly. This causes the wall of the trachea to be floppy, rather than relatively rigid as it is supposed to be. The breathing difficulties associated with congenital tracheomalacia (also called Type 1) begin soon after birth.

Other types of tracheomalacia occur because of degeneration of previously normal cartilage making up the wall of the trachea. This can happen from something outside of the trachea causing pressure on the airway (for example, an abnormality of the blood vessels surrounding the trachea or a tumor in the neck or throat) and, therefore, causing changes to the cartilage. This is called type 2 tracheomalacia. Breakdown of the cartilage in the trachea can also happen from prolonged intubation or chronic, recurrent infections involving the trachea - Type 3 tracheomalacia.

All three types of tracheomalacia are very uncommon.

Symptoms

     
  • High-pitched breathing (called stridor) and rattling noisy breaths.  
  • Breathing problems worsen with coughing, crying, or (in the case of infants) feeding. Upper respiratory infections also worsen symptoms.  
  • Breathing noises tend to improve during sleep.

Signs and tests

A doctor’s physical examination confirms the symptoms. A chest x-ray may show narrowing of the trachea when exhaling. Even if the x-ray shows nothing abnormal, it is needed to rule out other problems.

A definitive diagnosis is obtained by laryngoscopy, which is performed by a otolaryngologist (Ear, Nose, Throat doctor). This allows the doctor to visually examine the airway anatomy and helps determine the extent of tracheomalacia.

Other tests which may be perfomed include airway fluroscopy, barium swallow, bronchoscopy, lung function tests, and Magnetic Resonance Imaging (MRI).

Treatment

Those with tracheomalacia must be monitored very closely when they have respiratory infections. Most infants will respond well to humidified air, physical therapy, careful feedings, and antibiotics for infections.

Continuous positive airway pressure (CPAP) may be necessary in patients with respiratory distress. Rarely, surgery is needed. For example, in the case of acquired tracheomalacia, stent placement to hold the airway open may be necessary.

Expectations (prognosis)

Congenital tracheomalacia generally resolves on its own by age 18-24 months. As the tracheal cartilage strengthens and the trachea grows, the noisy respirations and breathing difficulties stop.

Complications

Congenital tracheomalacia can be associated with other congenital abnormalities like defects of the heart, developmental delay, and gastroesophageal reflux.

Aspiration Pneumonia can happen from inhaling food contents.

Calling your health care provider

Call your health care provider if you or your child breathe in an abnormal manner. If breathing difficulties are present or develop, this can become an urgent or emergency condition.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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