Tracheomalacia

Tracheomalacia occurs when the cartilage in the windpipe, or trachea, has not developed properly or was damaged, so instead of being rigid, the walls of the trachea are floppy or flaccid. The cartilage cannot keep the windpipe open, making breathing difficult — especially when breathing out (exhaling). Sometimes, pressure on the trachea from nearby blood vessels causes the trachea to collapse.

Congenital tracheomalacia means the baby was born with the condition. This is sometimes called type 1 tracheomalacia. Acquired or type 2 tracheomalacia is the result of an injury, most often repeated infections or a when a child has had a tracheostomy tube for a long time.

Tracheomalacia can be mild enough to be managed medically or it can be moderate or severe (life-threatening). Most children with tracheomalacia will improve by age 2 to the point that their symptoms that are not severe enough to require surgery.

If the floppiness extends to where the trachea branches into the lungs, called the mainstem bronchi, the condition is called tracheobronchomalacia.

While relatively rare, tracheomalacia is the most common congenital tracheal defect with 1 in 2,100 children having the condition. Babies born with tracheomalacia may have other congenital) abnormalities such as heart defects, developmental delay, esophageal abnormalities or gastroesophageal reflux. Acquired tracheomalacia is rarer than the congenital type.

Tracheomalacia symptoms tend to appear when a baby is between 4 and 8 weeks old as they start to breathe enough air to produce a wheezing sound. Sometimes, a child can develop tracheomalacia later in infancy or childhood.

Acquired tracheomalacia will appear when the injury or disruption of the airway is enough to cause symptoms.

Symptoms can range from mild to severe and 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 sounds when breathing (called wheeze or stridor), more frequently heard during exhalation and more pronounced with exercise/exertion
• rattling or noisy breaths
• frequent lung infections such as bronchitis or pneumonia
• apnea (halt in breathing) or cyanosis (bluish color to lips, skin) in infants
• choking during feeding
• chronic cough
• chronic chest congestion and infections
• difficulty breathing during activity

The doctor will listen to the child’s breathing sounds and take a medical history. At Children’s Hospital of Philadelphia, doctors from Pulmonology and Otolaryngology (Ear, Nose and Throat) work together to diagnose and treat children with tracheomalacia. Tracheomalacia has similar symptoms to other lung and airway disorders, so additional tests will be necessary to confirm the diagnosis and determine the severity.

Your child may have some of these tests:

• Bronchoscopy: a tube with a tiny camera goes in the mouth and down the airway, allowing the physician to see the trachea while the child breathes
• Laryngoscopy: similar to a bronchoscopy, but used to evaluate the voice box and upper airway and often done in conjunction with bronchoscopy
• Airway fluoroscopy: a kind of X-ray that shows movement of the cartilage in the trachea
• Esophagram: a kind of X-ray performed to help look for vascular rings
• Endoscopy: a thin, flexible tube with a light and camera on the end is inserted in the mouth in order to visualize the esophagus, stomach and beginning of the small intestine
• Computerized tomography (CT) scan: a combination of a lot of X-ray images to show an entire area of the body in detail
• Pulmonary function tests: that show how much air a child can breathe in and out
• Magnetic resonance imaging (MRI)

Treatment of tracheomalacia depends on the severity of the child’s condition.

Most babies respond well to humidified air, careful feedings and antibiotics for infections. While tracheomalacia doesn’t resolve entirely, often symptoms improve as the infant grows and are greatly reduced by 18 to 24 months as the tracheal cartilage strengthens.

Respiratory infections can be dangerous for newborns with tracheomalacia, as coughing causes the airway to collapse, making it even more difficult to breathe.

If the child also has gastroesophageal reflux disease (GERD), it is critical that this condition is also controlled with medication.

Children with tracheomalacia may be given medications to optimize airway clearance, such as ipratropium bromide (Atrovent). Saline solutions are also nebulized to thin the secretions as much as possible.

Children are helped by chest physiotherapy (percussion and vibration either by vest or manually) to help clear mucus in the airway to minimize the chance of infection. Some children will be taught how to perform pulmonary hygiene, which are a series of exercises and procedures that help to clear their airways of mucus and other secretions.

Some children may need noninvasive positive pressure ventilation, either bilevel positive airway pressure (BiPAP) or continuous positive airway pressure (CPAP). This involves wearing a mask over the nose and mouth so air can be gently pushed into the lungs.

In rare cases, a child may require tracheostomy, when a tube is put into an opening in the neck to allow for mechanical ventilation until the child has outgrown the problem.

In some severe cases, the child will need surgery to add stability to their airway.

If your doctor determines that surgery is necessary, the type of surgery will be customized depending on the severity and where the trachea is collapsing.

Factors determining the surgical approach include:

• cartilage deformation
• vascular anomalies
• mediastinal masses
• tracheoesophageal fistula
• abnormal airway branching
• chest wall and spine deformity

Surgical options for the treatment of tracheomalacia include:

• tracheostomy to afford a safe airway and allow the patient to hopefully outgrow the problem
• pexy procedures (anterior aortopexy, anterior and/or posterior tracheopexy, anterior and/or posterior mainstem bronchopexy, posterior descending aortopexy and thymectomy). These procedures attach the weak section of the trachea to other nearby organs or tissues to help hold it open.
• tracheal resection with end-to-end anastomosis
• placement of external splints and internal stents, either absorbable or permanent

At Children’s Hospital of Philadelphia, surgeries are performed by otolaryngologists (surgeons who specialize in ear, nose and throat problems) in our Head and Neck Disorders Program or our Pediatric Airway Program. Surgeons collaborate with pulmonologists and other specialists or general surgeons as needed.

Congenital tracheomalacia in babies tends to improve with noninvasive therapies by the age of 18 to 24 months in most children. As the cartilage gets stronger and the trachea grows, the noisy and difficult breathing slowly improves.

Children with tracheomalacia, who are being managed with medication and noninvasive therapies or who have had surgery, must be monitored closely by a pulmonologist or an ear, nose and throat doctor since the condition can lead to recurrent respiratory problems that can, eventually, injure the child’s lungs.

Babies born with tracheomalacia may have other congenital abnormalities, such as heart defects, developmental delay or gastroesophageal reflux, so appointments with additional specialists may be necessary.