Choanal atresia is a congenital malformation at the junction of the nasal and pharyngeal cavities. Rapid therapeutic measures are often important.
What is choanal atresia?
Choanal atresia is a complete closure of the posterior nasal opening that exists from birth. In this case, the posterior nasal opening (consisting of the paired choanal arches) represents the junction between the nasal and pharyngeal cavities. Because of the paired structure of the nasal opening, choanal atresia can be unilateral or bilateral. In the vast majority of cases, choanal atresia occurs unilaterally. In most cases of choanal atresia, the present closure of the nasal orifice is bony, while in rarer cases it may take the form of a membrane. By medical definition, choanal atresia is a malformation and occurs comparatively rarely. Since infants can only breathe in through the mouth to a very limited extent until about 6 weeks of age, bilateral choanal atresia often causes severe respiratory distress in them, especially during drinking. A possible symptom of unilateral choanal atresia is the discharge of purulent mucus from the affected nostril.
Causes
Usually, choanal atresia is due to a disorder during embryonic development. The posterior nasal opening forms on average between the 3rd and 7th embryonic weeks; if this formation is disturbed, choanal atresia is a possible consequence. The type of developmental disorder present determines whether subsequent choanal atresia is bony or membranous in structure.
Symptoms, complaints, and signs
Typical signs of choanal atresia include complete closure of the posterior nasal opening. This results in symptoms such as shortness of breath, secretions from the nose and mouth, and dominant mouth breathing. Due to inadequate nasal breathing, affected infants suffer from shortness of breath when drinking, which may result in decreased food intake. Respiratory failure may occur, which is a life-threatening complication, especially in severe choanal atresia. External signs of the malformation are conspicuous mouth breathing and pallor. The skin is often waxy, and the eye sockets are sunken. In unilateral choanal atresia, the turbinates are bluish in color. Signs of unilateral disease often do not appear for days or weeks. If both sides are affected, the malformation is usually detected immediately after birth. If the condition is not treated promptly, acute feeding problems may result, causing deficiencies and other problems. In the long term, untreated choanal atresia can cause developmental problems. Due to the lack of oxygen supply to the brain, various complications can occur, depending on the severity of the condition. To avoid this, treatment must be initiated immediately if a malformation is suspected.
Diagnosis and course
If choanal atresia is suspected in a patient because of mentioned symptoms such as shortness of breath or unilateral mucus discharge from the nose, this can be tested diagnostically in several ways. First, it is possible to insert a soft catheter into the nose to determine whether there is patency from the nasal to the pharyngeal cavity. The same can also be checked by an air injection, which takes place with the help of a balloon. If further examination steps are necessary to check the suspicion of choanal atresia, the attending physician can, for example, use nasal speculums or nasal endoscopes; these are medical instruments with which various structures of the interior of the nose can be examined. The course of unilateral choanal atresia in infants usually brings characteristic symptoms only after a few weeks. In contrast, bilateral choanal atresia often manifests itself very early; for example, by mouth breathing, which is generally rarely seen in infants. In most cases, the prognosis of choanal atresia is favorable with appropriate medical treatment.
Complications
Various complications can occur with choanal atresia. These depend mainly on the severity of the symptom and the malformations. In most cases, however, there is complete closure of the posterior nasal opening.As a result, the patient increases much less air than a healthy person, which leads to shortness of breath in many sufferers. In addition to shortness of breath, panic attacks and sweating often occur in stressful situations. The shortage of air can cause fatigue and headaches in many cases. The patient must compensate for this shortness of breath, breathing through the mouth to get enough oxygen. The quality of life is thereby reduced by the choanal atresia. If choanal atresia occurs directly in infants and babies, surgical intervention must be performed immediately to prevent death. Also, food cannot be given directly through the mouth and is transported through a tube. As a rule, the operations proceed without further complications and lead to success. The affected person can breathe freely again afterwards. If choanal atresia develops again in the course of life, another operation is usually necessary. With early treatment, there is no decreased life expectancy.
When should you see a doctor?
If choanal atresia is detected at birth, surgical treatment is usually given directly. In other cases, the nurse or physician must be notified immediately of the unusual breathing. Surgery is necessary in any case of congenital malformation and should be performed immediately. Parents who notice abnormal mouth breathing, shortness of breath and other signs of choanal atresia in their child are best to talk to their pediatrician quickly. In the event of complete closure of the posterior nasal opening – this is noticeable by pronounced shortness of breath – there should be no waiting to see a doctor. Sudden panic attacks and sweating are also clear warning signs that require medical clarification. If choanal atresia develops again in the course of life, the family doctor must be consulted. In case of a concrete suspicion, the ear, nose and throat specialist or a specialist for the respective symptom can be consulted directly. Emergency medical help is required if there is very sudden severe shortness of breath with panic attacks.
Treatment and therapy
In most cases, bilateral choanal atresia in an infant requires immediate emergency medical measures. These measures initially include keeping the newborn’s airway clear; this is done, for example, by placing what is known as a Rach tube. In some cases of bilateral choanal atresia, intubation (i.e., artificial respiration) of the infant may also be necessary. In addition, in order not to additionally influence mouth breathing, food is often administered via a tube. Surgeons are usually consulted as soon as possible for further treatment steps; if only mild membranous occlusions of the posterior nasal opening are present in choanal atresia, these occlusions can occasionally be broken through with the aid of a nasal catheter. Bony occlusions in the context of choanal atresia, on the other hand, are usually repaired surgically; in the first days of an infant’s life, such surgical measures may initially be provisional (temporary). Final surgery is then performed a few weeks or months later, depending on medical opinion (surgery may be performed from the nose or pharynx). After successful surgical treatment of bilateral choanal atresia, splinting, for example, helps to keep the airway clear. In unilateral choanal atresia, surgical intervention may often be sufficient while the child is still of school age, depending on the individual case.
Outlook and prognosis
Immediate treatment is necessary for choanal atresia. If treatment for this condition does not occur after birth, the affected individual usually dies from the symptoms. If the treatment is delayed, damage to the internal organs or the brain may also occur due to the undersupply of oxygen. In most cases, this damage can no longer be corrected and is therefore irreversible. The extent of this damage depends greatly on the duration of the undersupply. After the initial intervention, an additional procedure is performed a few months later to keep the airways permanently clear. Thereafter, the patient suffers no further discomfort or limitation, and there is no reduction in life expectancy. Life expectancy is negatively affected only if choanal atresia is not treated in time.If choanal atresia occurs only on one side, immediate treatment is usually not necessary. In this case, surgical intervention is usually sufficient even at school age. The symptoms are completely resolved by the operation and the risk of recurrence of choanal atresia is minimized.
Prevention
Because choanal atresia is already congenital, the malformation usually cannot be prevented. However, symptom exacerbation and possibly life-threatening respiratory distress can usually be prevented in infants by immediate medical intervention. A risk of recurrence of choanal atresia after successful treatment is counteracted by appropriate splinting in the surgical area.
Aftercare
In most cases of choanal atresia, the options for follow-up care are extremely limited. As a rule, the affected person is always primarily dependent on medical treatment by a physician to prevent further complications or further worsening of symptoms. The sooner a doctor is contacted in the case of choanal atresia and treatment is initiated, the better the further course of the disease usually is. An independent cure is not possible. In most cases, choanal atresia is treated with surgery, which completely relieves the symptoms. There are no particular complications. However, the patient should rest and take care of his body after the operation. It is advisable to take care of the nose in particular and to protect it especially well. To avoid infections or inflammations, the affected person should also take antibiotics after the procedure. It should be noted that these should not be taken together with alcohol. The life expectancy of the affected person is usually not reduced by choanal atresia. Furthermore, no further measures of follow-up care are necessary.
What you can do yourself
Choanal atresia is a medical emergency. First responders must call emergency medical services and provide first aid to the affected person. A mild obstruction can often be pierced by oneself, such as with a nasal catheter or other device. Patients then require inpatient treatment. After a procedure, the diet must be changed. Foods that could irritate the airways must be avoided. These include spicy, acidic, cool, hot and hard-to-chew foods. Sufferers should follow the doctor’s instructions. The medical professional will give strict guidelines regarding the intake of medications. Strict care of the nasal passages is also essential. This can prevent the posterior nasal openings from closing again. If athresia occurs repeatedly, surgical intervention may be necessary. The patient can also help to identify the cause by keeping a diary of complaints or consistently searching for triggers for the complaints. If these measures have no effect, a visit to the doctor is recommended. In most cases, suppurations or other complaints can be remedied with mild medications. Endurance sports, yoga or Pilates are recommended to improve breathing ability.
