Introduction

Tracheal stenosis (TS) is a rare but serious condition where the child’s trachea (windpipe) is constricted and airflow is reduced. This leads to breathing difficulties and other symptoms, the severity of which depends on the length and degree the trachea has been narrowed, the presence of other respiratory disorders (e.g. pneumonia, asthma) as well as the involvement of the bronchi (large airway branches which further divide into smaller ones - bronchioles - in the lungs). Early diagnosis and treatment are necessary as it can turn into a life-threatening emergency due to inadequate respiratory sufficiency.^4,10

In this article, the causes, symptoms and management strategies of tracheal stenosis will be briefly discussed to provide information to parents regarding their child’s diagnosis.

Causes

According to aetiology, there are two types of tracheal stenosis. These include:

• Acquired tracheal stenosis (ATS): the most usual type. It is the narrowing of the trachea that results from injury or repeated irritation. Such cases include prolonged intubation, tracheostomy, neck trauma, tracheal mass, inflammation or swelling (e.g. due to inhalation injury)
• Congenital tracheal stenosis (CTS) is the narrowing of the trachea due to abnormalities that present at birth. These most commonly are composed of stenoses caused by complete tracheal rings of cartilage (normally these rings are C-shaped but are O-shaped due to the defect) and tracheal cartilaginous sleeves (the C-shape is absent, and the trachea is formed like a tube resembling a sleeve). These malformations have been associated with other genetic disorders, like Down syndrome, Pfeiffer syndrome and Crouzon syndrome^2,4,7,10

Signs and symptoms

Symptomatology can appear postnatally or post-traumatically. Presentation in children may manifest with quickly worsening symptoms. These include:

• Noisy breathing (stridor): This type of breathing occurs due to airflow obstruction in the constricted airway
• Shortness of breath (dyspnea): the feeling of ‘air hunger’ and trying harder to breathe
• Feeling of fullness in the chest (chest congestion): mucus is accumulated in the lungs and bronchi, resulting in a wet, productive cough
• Feeding issues: choking or breathlessness when eating
• Recurrent pneumonia: pneumonia that occurs more than 2 times per year or at least 3 times at any time, and chest X-ray improvement is seen between episodes
• Asthma: symptoms persist and do not resolve with treatment
• Apnea: breathing stops briefly and can occur during sleep 
• Cyanosis (blue spells): blue colour appears on the skin, nails and lips due to decreased oxygen intake
• Wheezing: high-pitched whistling heard when breathing due to the tracheal narrowing
• Upper respiratory infections: more frequent infections and existing symptoms worsen when these occur^8,9

Management approach

Treatment for the condition depends on various factors. In general, surgery is preferred and the surgical technique choice depends on the location and severity of the case as well as the presence of associated congenital anomalies. Specifically, management plans include the following:

• Observation: Some mild cases may resolve over time. Close monitoring and regular follow-up visits are essential.
• Balloon dilation: A balloon device is introduced into the airway, inflating the balloon in the respective tracheal area. It is then deflated, and the device is removed. Preferred in moderate to severe cases^5,9
• Tracheal resection and primary anastomosis (TRPA): The constricted part of the trachea is removed, and the healthy parts of the trachea are rejoined together. Studies have shown that in the case of ATS due to prolonged intubation, this is a safe treatment option and reduces the recurrent balloon dilations³
• Cricotracheal resection (CTR): the constricted portion of the trachea is removed, the vocal cords and trachea are sewn together, and this healthy part is reattached
• Slide tracheoplasty: This is preferred for the most severe cases. In this operation, the constricted trachea is surgically divided horizontally. The posterior of the lower tracheal part and the upper tracheal part are both cut. The ends of the parts are sutured and create a wider trachea due to their overlap. It is considered the gold standard for CTS^1,4,6,10

FAQs

How will I know if my child suffers from Tracheal Stenosis?

Your healthcare provider will request imaging and endoscopy studies to see the trachea. Diagnosis is made with a combination of clinical examination and tests. In addition, referral to pulmonologists (lung doctors) and gastroenterologists (digestive system doctors) for subsequent evaluation may be required to assess for other conditions that may affect the child’s health. Tests include:

• X-rays, CT, MRI: these imaging tests will be used to receive detailed images of the narrowed trachea and the extent of the damage
• Echocardiography: can be used to exclude potential heart abnormalities
• Microlaryngoscopy and bronchoscopy: During these procedures, your child will be sleeping while the doctor inserts a telescope from the mouth and takes a look throughout the respiratory system so as to assess the severity of the disorder^4,9,10

What is the outlook for Tracheal Stenosis?

Recovery periods and results vary according to the treatment of choice. Subsequent surgeries may also be required. In addition, follow-up visits are necessary for monitoring in case of recurrence.¹¹

When should I contact my healthcare provider?

If your child experiences any symptoms suggestive of tracheal stenosis, call a healthcare professional. If your child is a newborn or infant, visit an emergency department as soon as possible. Seek medical care as soon as possible, as the condition can become life-threatening.¹¹

Summary

Tracheal stenosis is a rare and serious condition in which the trachea narrows, resulting in decreased airflow, leading to breathing issues. Based on the cause, it is divided into two types: acquired (ATS), which results from trauma, and congenital (CTS), which occurs at birth. Symptoms include breathing and feeding issues, chest congestion, recurrent pneumonia, asthma, upper respiratory tract infections, as well as cyanosis. Treatment depends on case severity and includes observation and surgery. Diagnosis is made with a combination of clinical examination, imaging, echocardiography and endoscopic procedures. Medical care should be sought if the child develops symptoms, and monitoring is necessary for recovery post-operatively as well as for recurrence.