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Laryngomalacia – Causes, Symptoms, Diagnosis, Treatment

Laryngomalacia presents with inspiratory stridor that typically worsens with feeding, crying, supine positioning, and agitation. The symptoms begin at birth or within the first few weeks of life, peak at 6 to 8 months, and typically resolve by 12 to 24 months [rx]. Laryngomalacia is usually diagnosed within the first 4 months of life [rx]. Although inspiratory stridor is the classic symptom of laryngomalacia, there are a number of associated symptoms. The most common associated symptoms are related to feeding which includes regurgitation, emesis, cough, choking, and slow feedings.

Laryngomalacia is the most common cause of chronic stridor in infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. It can also be seen in older patients, especially those with neuromuscular conditions resulting in weakness of the muscles of the throat. However, the infantile form is much more common. Laryngomalacia is one of the most common laryngeal congenital diseases in infancy and public education about the signs and symptoms of the disease is lacking.

Types of Laryngomalacia

Laryngomalacia is generally classified by its laryngoscopic appearance. The most commonly used classification, proposed by [rx], is as follows:

Type 1—prolapse of the mucosa overlying the arytenoid cartilages,
Type 2—foreshortened aryepiglottic folds, and
Type 3—posterior displacement of the epiglottis.
Various combinations of these types may be seen. Furthermore, LM can be accompanied by synchronous airway lesions (SAL), predominantly: another airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)], subglottic stenosis, and vocal cord paralysis. It has been shown that SAL is more frequent in infants with severe LM [rx, rx–rx].

Causes of Laryngomalacia

Although laryngomalacia is not associated with a specific gene, there is evidence that some cases may be inherited.^[rx]^[rx] Relaxation or a lack of muscle tone in the upper airway may be a factor. It is often worse when the infant is on his or her back, because the floppy tissues can fall over the airway opening more easily in this position.^[rx]

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The exact cause of laryngomalacia is not known. Relaxation or a lack of muscle tone in the upper airway may be a factor. The malformation is usually present at birth or appears within the first month of life. Gastroesophageal reflux (GE reflux) may contribute to the severity of the symptoms. The noisy breathing is often worse when the infant is on his back or when crying.

If your child is born with laryngomalacia, symptoms may be present at birth, and can become more obvious within the first few weeks of life. It is not uncommon for the noisy breathing to get worse before it improves, usually around 4 to 8 months of age. Most children outgrow laryngomalacia by 18 to 20 months of age. Symptoms include:

Noisy breathing (stridor) — An audible wheeze when your baby inhales (breaths in). It is often worse when the baby is agitated, feeding, crying, or sleeping on his back.
High pitched sound
Difficulty feeding
Poor weight gain
Choking while feeding
Apnea (breathing stoppage)
Pulling in neck and chest with each breath
Cyanosis (turning blue)
Gastroesophageal reflux (spitting, vomiting, and regurgitation)
Aspiration (inhalation of food into the lungs)
Difficulty feeding or nursing
Slow weight gain, or even weight loss
Choking when swallowing
Aspiration (when food or liquids enter the lungs)
Pausing while breathing, also known as apnea
Turning blue, or cyanosis (caused by low oxygen levels in the blood)

Laryngomalacia saw during an exam your doctor will ask you some questions about your baby’s health problems and may recommend a test called a nasopharyngolaryngoscopy (NPL) to further evaluate your baby’s condition.

During this test, done in your doctor’s office, a tiny camera that looks like a strand of spaghetti with a light on the end is passed through your baby’s nostrils and into the lower part of the throat where the larynx is. This allows your doctor to see your baby’s voice box.

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If laryngomalacia is diagnosed, the doctor may want to do other diagnostic tests to evaluate the extent of your child’s problems and to see whether the lower airway is affected. These tests may include:

X-ray of the neck
Airway fluoroscopy
Microlaryngoscopy and bronchoscopy (MLB)
Esophagogastroduodenoscopy (EGD) and pH probe
Functional endoscopic evaluation of the swallow (FEES)

Treatment of Laryngomalacia

Time is the only treatment necessary in more than 90% of infant cases.^[rx] In other cases, surgery may be necessary^[rx] Most commonly, this involves cutting the aryepiglottic folds to let the supraglottic airway spring open. Trimming of the arytenoid cartilages or the mucosa/ tissue over the arytenoid cartilages can also be performed as part of the supraglottoplasty. Supraglottoplasty can be performed bilaterally (on both the left and right sides at the same time), or be staged where only one side is operated on at a time.^[rx]

Treatment of gastroesophageal reflux disease can also help in the treatment of laryngomalacia since gastric contents can cause the back part of the larynx to swell and collapse even further into the airway. In some cases, a temporary tracheostomy may be necessary.

Laryngomalacia surgery

A surgery called supraglottoplasty is the treatment of choice if your child’s condition is severe. Symptoms that signal the need for laryngomalacia surgery include:

Life-threatening apneas (stoppages of breathing)
Significant blue spells
Failure to gain weight with feeding
Significant chest and neck retractions
Need for extra oxygen to breathe
Heart or lung issues related to your child’s inability to get enough oxygen

This surgery may not completely eliminate the noisy breathing but it should help to:

Reduce the severity of the symptoms
Lessen the apneas (breathing stoppages)
Reduce the extra oxygen requirements
Improve swallowing
Help your child gain weight

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The safety of your child’s swallow should be re-evaluated after the surgical procedure.

Supraglottoplasty has been shown to decrease the duration of symptoms of laryngomalacia significantly.[rx] Patients generally tolerate the procedure well and are observed in the hospital postoperatively. Steroid administration during surgery and in the postoperative period to decrease airway inflammation is typically the recommended pharmaceutical therapy.[rx]

Tips to reduce reflux

Babies with laryngomalacia may have trouble keeping food down. This means food often comes back up into the mouth (reflux). Follow any instructions the healthcare provider gives you to reduce your child’s reflux. The following precautions for feeding your child can help:

Hold your child in an upright position during feeding and at least 30 minutes after feeding. This helps keep food from coming back up.
Burp your child gently and often during feeding.
Avoid juices or foods that can upset your child’s stomach, like orange juice and oranges.
Talk to your child’s healthcare provider if food comes up a lot during feeding. You may be told to give your child less milk to avoid reflux.
Never lay your baby flat on his or her back with a propped bottle.

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