Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.
Eyes front and ears open. Class is now in session.
The rare disease that we will be learning about today is:
Subglottic Stenosis
What is Subglottic Stenosis?
- Subglottic stenosis is a condition of narrowing of the subglottic airway
- The subglottic airway is positioned between the vocal cords and the lower portion of the cricoid cartilage
- In an infant, a subglottic airway less than 4mm in diameter is defined as subglottic stenosis
- MRI and CT scans are useful in diagnosis
- X-rays can also be important for identifying the region and size of the affected area
- Optical coherence tomography (OCT) is used to monitor the progression of the condition
- Subglottic stenosis is graded at 4 different levels, with grade 1 being least severe and grade 4 being the most
- The condition has decreased in frequency due to superior management of people on ventilators
How Do You Get It?
- Subglottic stenosis can be congenital or acquired
- Many cases are considered iatrogenic, meaning that they are caused by medical examinations or procedures
- After the introduction of long-term intubation in the 1960s, acquired cases are more common than congenital ones
- The most common causes of subglottic stenosis are medical procedures and external trauma
- In adults, external trauma is the most frequent cause; examples include vehicle accidents and clothesline injuries
- 90% of acquired cases in children are from intubation
- Medical procedures that can cause subglottic stenosis include:
- Tracheostomy
- Intubation
- Excessive endotracheal tube cuff pressure
- A week of intubation is enough to cause the condition in newborns; 17 hours can cause it in adults
- Certain infections can also cause subglottic stenosis, such as:
- Tuberculosis
- Laryngeal papillomatosis
- Bacterial traechitis
- Diphtheria
- Histoplasmosis
- Systemic diseases linked to the condition include:
What Are the Symptoms?
- Signs and symptoms include:
- Airway obstruction
- Stridor during exercise
- Difficulty breathing
- In infants:
- Unusual cry
- Inability to vocalize
- Air hunger
How Is It Treated?
- The main goal of treatment is opening the airway and relief of any breathing problems
- Endoscopic balloon dilation is a successful treatment procedure in mild cases
- However, severe subglottic stenosis can often recur afterwards
- Repeat procedures are usually avoided due to the risk of further damage to the affected area
- Open surgery is the typical approach when dilation isn’t successful
- Options include endoscopy, tracheotomy, and open neck surgery
- Overall prognosis is good as long as surgery is conducted skillfully to avoid complications
Where Can I Learn More???
- Check out our cornerstone on this disease here.
