Currently common treatment methods
After the diagnosis has been made, usually by a general practitioner, ENT doctor or pulmonologist, you should visit a specialized clinic with as much experience as possible. There you will usually first be given a consultation appointment, during which the stenosis will be examined and the various treatment options discussed.
As a rule, the first treatment recommended is dilation, i.e. stretching the narrow area. This is a short endoscopic procedure that quickly leads to good breathing again.
You should always go to treatment appointments in good time and not delay them for too long. There is little point in waiting until the strain becomes too great. If the stenosis is very narrow and the strain has become high (especially due to a very late correct diagnosis), you can often contact the treating clinician directly to get an early appointment for the dilation.
Our members are currently being treated primarily with high-pressure balloon dilation and have had good long-term experiences with it. Cricotracheal resection (CTR) is occasionally recommended, but should not be the first choice. Its benefits and risks should be carefully weighed up without any time pressure. Get a second opinion beforehand if possible.
Some are gaining initial experience with outpatient steroid injections or are successfully using inhaled steroids to prolong the intervals between dilations.
Endoscopic Treatments:
High-Pressure Balloon Dilation
The scarred stenosis ring is first incised in a star shape and then expanded with a balloon catheter. In addition, a long-acting cortisone preparation (usually triamcinolone) is injected into the tissue. The injected cortisone extends the interval until the next treatment. Budesonide can also be inhaled daily to extend the interval.
It is a gentle procedure that requires a short general anesthetic (approx. 30 minutes). You will usually stay in the clinic overnight for monitoring.
The risks of this procedure are minimal, but the narrowing usually returns. For most of the patients in our group, however, repeated dilation at increasing intervals makes sense in the long term and, if carried out gently and without a laser, can be repeated several times into old age. If dilation is required every 1-2 years, the dilation is considered successful. The intervals between dilations also often become longer.
After dilation, most of our members report a significant improvement in their shortness of breath, and some have been thinking "why not sooner?" You should therefore arrange follow-up appointments as soon as possible and not let the strain become too great. We should not risk an emergency situation, and that is what the doctors advise us too!
Various balloon catheters are used for dilation:
Some prefer the smooth surface of a conventional balloon catheter. Here, supersaturation is achieved by adding oxygen before and between each dilation procedure, which makes it possible to dilate the stenosis once or several times.
The trachealator catheter, on the other hand, allows ventilation during dilation, but must then be rotated to evenly dilate the stenosis.
Source: Courtesy of https://www.spiggle-theis.com/de/produktuebersicht/trachealator
laser dilation
Laser and balloon or laser and manual manipulation of the scar.
The tendency to open idiopathic tracheal stenosis using laser incisions is declining, as laser treatment carries a high risk of causing permanent damage to the cricoid cartilage and the structure surrounding the stenosis. In this case, too, the stenosis usually returns. The risk of injury increases with each treatment, which is why laser treatment is not suitable for repeat therapy.
The new guideline "What we learned - 2024" presents a study that shows that the intervals between dilations can be extended if the incisions before balloon dilation are made with a laser rather than a scalpel. However, there remains concern that the laser may cause permanent damage to deeper structures. Unfortunately, some members of our group experienced injuries and scarring during lasering.
Other endoscopic procedures
are described in Catherine Anderson's guide, but are hardly used in this country and are at least not known to our members.
The use of stents should generally be avoided in idiopathic stenosis because they do not stop the growth of the stenosis and the stents grow into the stenosis.
Open Surgical Treatments:
Tracheal resection, usually cricotracheal resection
A tracheal resection is a complex operation in which a scarred part of the trachea is removed and the healthy ends are reconnected. It is also called cricotracheal resection (CTR), laryngeal tracheal resection (LTR) or tracheal resection and reanastomosis. They differ slightly from each other but also have similarities. For example, if the scarred part is particularly long, a stent or piece of rib is inserted into the neck to replace the lost cartilage and prevent the trachea from collapsing.
The literature indicates a success rate of 80-95%. On average, a resection lasts about 10 years. As with all averages, some procedures last shorter and others longer.
The greatest risk is restenosis, but damage to the vocal cord nerves can also occur. About 5% of patients operated on by a very experienced surgeon will experience re-stenosis within 3 years. Most patients experience a permanent change in their voice (often deeper) because of a change in the height of the larynx (which is necessary for a good vocal range, especially in singers). Some patients are unable to raise their voice or speak loudly.
In many cases, removing the constriction can stop growth or at least pause it for many years. Due to the higher risk, specialists now only recommend cricotracheal resection if dilations are unsuccessful or if there are other reasons not to continue dilations.
In our group, some members have had very different experiences with CTR. Most of them had no breathing problems for several years. However, the operation often had a permanent impact on their voice.
Detailed descriptions can be found at www.klinikum-stuttgart.de and in Catherine Anderson’s guide.
Further reatments:
Outpatient Steroid Injection
In the USA and partly in Europe, more and more patients are being treated with steroid injections directly into the neck. This can significantly slow the growth of the stenosis after dilation and thus prolong the symptom-free period. The dilation intervals are therefore longer.
No general anesthesia is required for the outpatient injection; it is performed using a local anesthetic in the trachea. First, a local anesthetic is injected, which spreads through the trachea when coughing. The steroid is then injected directly into the scar tissue under visual control using an endoscope. The procedure only takes a few minutes and you can leave the practice immediately afterwards.
Our group had initial positive experiences with a series of approximately five injections at intervals of approximately three months.
Other supportive measures that can slow the progression of stenosis and alleviate accompanying symptoms are
Daily inhalation with budesonide
regular inhalation with saline solution
drink plenty of fluids to keep the mucus fluid
various aids for better mucus dissolution
healthy diet and plenty of exercise in the fresh air
Prevention or good healing of respiratory infections
Further tips are also available in the internal forum and through discussions in the self-help group as well as in Catherine Anderson’s guide .
What we learned in self-help:
Idiopathic stenosis is chronic and none of the current treatment options can guarantee that the stenosis will disappear for life.
This may seem frightening at first, but fortunately the stenosis is benign and, thanks to ever-improving treatment methods and supportive measures, enables a largely symptom-free life.
We can actively campaign for the treatment that is best for us. Since our disease is so rare and the diagnosis is still very new (the first names only appeared in the 1970s), we cannot assume that every doctor and every clinic is knowledgeable and has experience in this field.
For example, the fact that the latest, very gentle balloon dilatations can produce very good results and that this method can also be used permanently is not widely known. Some surgeons may only be familiar with major operations and are quicker to recommend cricotracheal resection than others.
It is therefore very important to obtain detailed information and, if necessary, at least a second opinion.