Tracheomalacia

Tracheomalacia

Tracheomalacia is a comparatively rare disease that is associated with instability or softness of the windpipe (trachea) and can be attributed to congenital (innate) and acquired causes. The prognosis and course of tracheomalacia depend on the underlying cause.

What is tracheomalacia?

Tracheomalacia is an instability of the trachea that is caused by insufficient strength of the cartilage braces that stabilize the trachea and can affect individual sections or the entire trachea. For slang hf, please visit electronicsencyclopedia.com.

As a result of the softness of the tracheal cartilage structures, breathing can be impaired because the resistance to breathing is increased. This can also lead to a collapse of the trachea due to the greatly reduced inspiratory negative pressure when inhaling, especially when there is an increased need for oxygen.

Tracheomalacia manifests itself in the form of inspiratory and expiratory stridor (inhalation and exhalation noise), limited exercise capacity, functional stenosis (narrowing), coughing, tachy- and dyspnea (increased or difficult respiratory function) and cyanosis.

In many cases, affected infants also hyperextend their neck to widen the lumen of the trachea (so-called opisthotonus posture). In the further course, the impairment can spread to the bronchi (tracheobronchomalacia).

Causes

Depending on the underlying cause, three types of tracheomalacia are differentiated. In the congenital or primary form, there is usually a congenital connective tissue disease such as campomelia syndrome, esophageal atresia (malformation of the esophagus) or tracheoesophageal fistulas, which lead to impaired growth of the tracheal cartilage rings.

In addition, tracheomalacia can be caused by external compression (Type 2) that narrows the trachea. The narrowing ( stenosis ) is typically due to mediastinal tumors (including a hemangioma ), congenital vascular anomalies (including a double aortic arch, the so-called pulmonary loop), bronchogenic cysts, a megaesophagus, or goiter.

The third form is caused by chronic infections (including recurrent polychondritis) or excessively long intubation with high ventilation pressures, with an increased risk here, particularly in premature babies.

Symptoms, Ailments & Signs

Tracheomalacia leads to breathing difficulties and abnormal breathing sounds. Doctors differentiate between congenital and acquired tracheomalacia. The innate expression is regularly associated with a positive outcome. It is no longer present after the first or second year of life, even without therapy.

Symptoms occur primarily in patients in the prone position. There is an improvement in an upright or inclined position. Clear signs can be detected when inhaled. Then the resistance of those affected is comparatively greater. Doctors listen to the breathing as part of their diagnosis and usually describe the sound as hens cackling.

At the same time, the nostrils move with inhalation and exhalation, which often remains hidden from the patient. Pendulum movements can occur on the abdomen. Sick children and adults are often unable to identify the symptoms themselves. That is why consulting a doctor is essential.

If those affected name problems in advance, they relate to breathing. Problems in stressful situations are often referred to. Shortness of breath quickly sets in during sport or in the context of pronounced gaits. Those affected then react by coughing and gasping for air. Fear and panic often set in.

Diagnosis & History

Initial suspicion of tracheomalacia often results from the clinical symptoms. A decrease in air flow during expiration (breathing out) as part of a lung function check also indicates the possible presence of tracheomalacia.

In addition, a functional stenosis due to tracheomalacia can be differentiated from a fixed tracheal stenosis. The diagnosis is confirmed by a fiberoptic endoscopy, which enables an assessment of the dynamic changes in the tracheal lumen in the various respiratory phases. Tracheomalacia due to external compression can be detected by imaging diagnostic procedures such as MRI (magnetic resonance imaging), CT (computed tomography) or angiography. The course and prognosis of tracheomalacia depend heavily on the underlying cause.

While congenital forms usually have a very good prognosis and are largely self-remitting, the infection-related form has a significantly less favorable prognosis. The prognosis of other acquired forms of tracheomalacia depends on whether the triggering factors (tumors or malformations) can be eliminated.

Complications

The breathing difficulties typical of tracheomalacia can cause various complications as the disease progresses. First, there may be shortness of breath. This results in an insufficient supply of oxygen to the brain, which can have life-threatening consequences, especially for infants and small children.

In the case of severe tracheomalacia, which is also aggravated by infections, there is an acute danger of suffocation. Complications can also arise from the underlying disease. For example, if tracheomalacia is caused by a tumor, there is always a risk that it will metastasize or grow and make breathing difficult.

If the course is severe, the prognosis is rather poor. Many children die from the disease or suffer from stunted growth and mental problems due to lack of oxygen. Drug treatment represents an enormous burden for infants and young children. An overdose can quickly cause serious complications, and side effects can also occur.

A surgical procedure involves the usual risks, i.e. the risk of infections, bleeding and nerve or vascular injuries. Splinting the trachea is particularly problematic. Inflammation or sensitive injuries to the trachea occur again and again. Wound healing disorders and postoperative bleeding can also occur after an operation.

When should you go to the doctor?

General breathing difficulties are always a cause for concern. If they occur in physically demanding situations or during sporting activities, they are often part of a natural reaction. As soon as the symptoms recede within a short time after taking a rest phase, there is in most cases no further need for action. However, if the breathing irregularities persist, if they occur repeatedly or if they increase in intensity, a doctor must be consulted.

Since this disease can be congenital or acquired, a doctor is needed as soon as newborns show the first abnormalities. If there are changes in breathing activity during the development and growth process or in adulthood, a doctor must also be consulted. Coughing, looking pale and a decrease in physical performance are signs of a health problem. In the case of fatigue, rapid exhaustion and exhaustion, a search for the causes should be initiated.

If the person concerned shows signs of emotional or psychological peculiarities, medical help is also required. In states of uncertainty, fear and panic, a racing heart and sweating, a doctor should be consulted. If there is resistance in the organism when inhaling oxygen, this is a characteristic feature of the disease and should be discussed with a doctor as soon as possible.

Treatment & Therapy

The therapeutic measures in tracheomalacia depend on the extent, the underlying cause and the age of the specific affected person. The congenital form is usually self-remitting and growth-related stabilization of the tracheal wall takes place in the first two to one and a half years of life.

Physiotherapeutic measures can support an improvement in respiratory function. If secondary infections also occur as a result of increased secretion retention, antibiotic therapy in combination with inhalations to moisten the airways may be indicated. In some cases, especially in the case of diffuse tracheobronchomalacia or insufficient oxygen supply, long-term CPAP therapy (CPAP = Continuous Positive Airway Pressure) via a tracheostomy tube or ventilation mask is recommended to permanently generate positive pressure until the cartilaginous structures have stabilized due to growth.

Surgical intervention is indicated for severe or life-threatening forms and children who cannot be weaned from CPAP therapy. In order to avoid tracheal compression in the case of short stretches of tracheomalacia, the aorta is fixed to the sternum (breastbone) as part of an aortopexy, so that the mediastinum (medium skin space) is given more space. If long-distance tracheomalacia is present, however, the trachea is splinted using an expandable intraluminal metal stent.

In addition, the triggering factors in acquired tracheomalacia should be eliminated. Compressing structures such as tumors, vascular anomalies or goiter should be surgically removed. However, if the tracheomalacia can be traced back to an infection-related instability, antibiotic therapy is indicated.

Prevention

The congenital form of tracheomalacia can be partially prevented by treating the malformations that cause it as far as possible. Acquired tracheomalacia can be prevented by appropriate caution during long-term intubations and consistent treatment of the underlying diseases such as tumors or goiter.

Aftercare

Tracheomalacia is a slackening of the trachea that can have various causes. The aftercare, which must focus on the underlying disease, is correspondingly diverse. In infants who may have congenital tracheomalacia, repositioning the child can often temporarily relieve the symptoms. If spontaneous healing occurs after some time, this does not require any special follow-up care, but only regular visits to a pediatrician, who will ensure complete healing.

If tracheomalacia occurs as a result of external influences such as goiter, the causative disease must be treated and, as a rule, an operation must be carried out. Here the follow-up care is designed for the underlying disease and can therefore not be presented as a single valid form. The consequences of the operation and its after-effects must be observed, which accompanies healing.

The tracheomalacia and its healing must also be included in the aftercare by an appropriate specialist. If tracheomalacia was caused by an infectious trigger, acute treatment with antibiotics is required. In the follow-up care, the specialist ensures that the tracheomalacia is free from bacteria and heals. It must also be noted that antibiotic therapy also affects the intestinal flora. This should be rebuilt during aftercare.

You can do that yourself

Congenital tracheomalacia can be improved by placing the infant on its stomach for the first few months of life. This often leads to an improvement in the symptoms over a period of months or years. If the disease is based on external compression, the cause must be treated. This can be achieved, for example, with radioiodine therapy or with a strumectomy if the symptoms are caused by goiter. In addition, the typical general measures apply, i.e. rest and protection.

An infection-related tracheomalacia must be treated with medication, for example by the patient taking antibiotics. The appropriate accompanying treatment is rest. Severe tracheomalacia can cause life-threatening breathing difficulties. Good monitoring of the person concerned is all the more important. If the mentioned respiratory problems occur, the emergency doctor must be called. The patient must be in the stable lateral position be brought to ensure adequate ventilation of the lungs. Depending on the intensity and localization of the symptoms, tracheostomy tubes can then be inserted, which ensure the supply of oxygen. The tube must be checked for inflammation and bleeding, which occurs primarily in people whose immune systems have already been significantly weakened by the disease.

Patients suffering from tracheomalacia must be constantly monitored in everyday life, as complications can occur again and again. To ensure that help arrives quickly in the event of a medical emergency, close contact should be maintained with the emergency telephone service.

Tracheomalacia