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Tracheomalacia - congenital

Definition

Congenital tracheomalacia is weakness and floppiness of the walls of the windpipe (trachea). Congenital means it is present at birth. Acquired tracheomalacia is a related topic.

Alternative Names

Type 1 tracheomalacia

Causes

Tracheomalacia in a newborn occurs when the cartilage in the windpipe has not developed properly. Instead of being rigid, the walls of the trachea are floppy. Because the windpipe is the main airway, breathing problems begin soon after birth.

Congenital tracheomalacia is very uncommon.

Symptoms

Symptoms can range from mild to severe. Symptoms may include:

  • Breathing noises that may change with position and improve during sleep
  • Breathing problems that get worse with coughing, crying, feeding, or upper respiratory infections (such as cold)
  • High-pitched breathing
  • Rattling or noisy breaths

Exams and Tests

A physical exam confirms the symptoms. A chest x-ray will be done to rule out other problems. The x-ray may show narrowing of the trachea when breathing in.

A procedure called laryngoscopy provides the most reliable diagnosis. In this procedure, an otolaryngologist (ear, nose, and throat doctor, or ENT) will look at the structure of the airway and determine how severe the problem is.

Other tests may include:

  • Airway fluoroscopy -- a kind of x-ray that shows the images on a screen
  • Barium swallow
  • Bronchoscopy -- camera down the throat to see the airways and lungs
  • CT scan
  • Lung function tests
  • Magnetic resonance imaging (MRI)

Treatment

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

Often, the symptoms of tracheomalacia improve as the infant grows.

Rarely, surgery is needed.

Outlook (Prognosis)

Congenital tracheomalacia most often goes away on its own by the age of 18 to 24 months. As the cartilage gets stronger and the trachea grows, the noisy and difficult breathing slowly improves. People with tracheomalacia must be monitored closely when they have respiratory infections.

Possible Complications

Babies born with tracheomalacia may have other congenital abnormalities, such as heart defects, developmental delay, or gastroesophageal reflux.

Aspiration pneumonia can occur from inhaling food into the lungs or windpipe.

When to Contact a Medical Professional

Call your health care provider if your child has breathing difficulties or noisy breathing. Tracheomalacia can become an urgent or emergency condition.

References

Finder JD. Bronchomalacia and tracheomalacia. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 416.

Green GE, Ohye RG. Diagnosis and management of tracheal anomalies and tracheal stenosis. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 210.

Wert SE. Normal and abnormal structural development of the lung. In: Polin RA, Abman SH, Rowitch DH, Benitz WE, Fox WW, eds. Fetal and Neonatal Physiology. 5th ed. Philadelphia, PA: Elsevier; 2017:chap 61.

Last reviewed April 14, 2021 by Charles I. Schwartz, MD, FAAP, Clinical Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, General Pediatrician at PennCare for Kids, Phoenixville, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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