Index
It is a congenital narrowing of the back of the nasal cavity that causes shortness of breath.
It occurs when the nasal passages do not develop properly during the prenatal stage.
This condition is rare, occurs in about 1 in 7,000 live births, and is seen more often in women than men.
Types of choanal atresia
If a child is diagnosed with choanal atresia, it can take two different forms. It can be bilateral or unilateral.
Bilateral choanal atresia
It blocks both nasal passages and is life threatening, with symptoms appearing immediately after birth.
Babies breathe only through their noses when they are very young, so blocked nasal passages will cause extreme difficulty in breathing.
Unilateral choanal atresia
Where only one nasal passage is blocked, it is more common, less severe, and sometimes appears later in childhood, because the child has been able to control himself while breathing through only one side of the nasal passage.
Both types of choanal atresia are categorized according to the type of block:
- The blockage is made up of bones and soft tissues. About 70% of babies with the disease have this type.
- The blockage is made up of only bones. About 30% of babies with choanal atresia have this type.
Causes
Choanal atresia is a developmental abnormality, the abnormality is present at birth and can be associated with certain inherited disorders that cause fetal defects and abnormalities in combination with environmental factors.
There have also been reports that women who took certain thyroid medications, such as carbimazole and methimazole (tapazole), during pregnancy delivered babies with choanal atresia at a higher rate.
However, the association is not clear, nor could it be determined whether the mothers’ thyroid disease might have led to choanal atresia, or whether the medications themselves were a factor.
Studies have shown that choanal atresia is linked to a common herbicide (atrazine) used to treat crops.
The study found that women exposed to the chemical had 80 times the risk of giving birth to children with coanasss atresia than others who were not exposed.
There is no known specific cause of choanal atresia. Choanal atresia occurs when the tissue that separates the nose and mouth area during fetal development and remains after birth.
Risk factor’s
It is important to note that having a risk factor does not mean that the person will get the condition.
A risk factor increases the chances of contracting a condition compared to an individual without the risk factors.
Also, not having a risk factor does not mean that a person does not have the condition. It is always important to discuss some risk factors for the development of choanal atresia with your doctor.
Choanal atresia is the most common nasal abnormality in newborns; 1 in 7,000 newborns may be affected.
Young girls are twice as likely to develop this condition as young boys, but there is no racial or ethnic preference.
Exposure to chemicals and drugs that act as endocrine disruptors during pregnancy.
Smoking or exposure to secondhand smoke during pregnancy (pregnancy period).
Excessive coffee consumption by pregnant mothers, exposing the developing fetus.
Elevated levels of vitamin B-12 and zinc in a pregnant woman increase the risk.
Multiple pregnancies, such as twins, carry an increased risk of choanal atresia.
This common type of nasal defect among newborn boys is often associated with other developmental abnormalities, such as:
CHARGE syndrome
This inherited condition causes severe hearing loss, loss of vision, breathing, and swallowing problems.
More than half of children with CHARGE have choanal atresia, and about half of them have both sides of the nose.
Treacher Collins syndrome
This condition affects the development of bones in a baby’s face.
Crouzon’s syndrome
This genetic condition causes the bones of a baby’s skull to fuse together too soon. This prevents the skull from growing as it normally would.
Tessier’s syndrome
This condition causes large openings (slits) that divide the baby’s face.
Signs and symptoms of choanal atresia
Some common signs and symptoms in a child with choanal atresia include:
- The back of the nasal passage is blocked by the growth of abnormal or soft tissues formed during the fetal stage of development, causing respiratory distress
- Nasal drainage with continuous discharge from one of the nostrils.
Retraction of the skin between or around the bones of the chest when inhaling. - Difficulty inserting a 5/6 French suction catheter into the nasopharynx easily through the nostrils.
- Presence of cyanosis or a blue color to the skin due to low oxygen levels in the blood. Cyanosis worsens during breastfeeding, causing feeding difficulties, but there is cyclical respiratory relief that improves when the baby is crying
Diagnosis
The following methods can be used to accurately diagnose the condition.
Physical assessment
A thorough physical evaluation, performed by a health specialist is the first step.
Insertion of a nasal catheter into the nose (failure to do so could be an indication of Choanal atresia)
Symptom assessment
Patients with bilateral choanal atresia often have episodes of respiratory distress immediately after birth.
Because of this, babies with bilateral choanal atresia often require intubation immediately after delivery.
Once the airway is secured, the patient can be evaluated for other developmental abnormalities.
Patients with unilateral choanal atresia often show symptoms later in infancy and childhood with signs of unilateral nasal obstruction.
Since one side is open at birth, it is rare for these children to have shortness of breath when they are first born.
Tests
Many clinical conditions can have similar signs and symptoms. The doctor may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
- Computed tomography of the affected region. Computed tomography shows whether choanal atresia is unilateral or bilateral.
- Endoscopy of the nose.
- X-rays of the paranasal sinuses.
Treatment of choanal atresia
Treatment depends on the severity of the abnormality (unilateral or bilateral), how much it affects the child’s breathing and eating, and what other medical conditions the patient has.
Treatment for mild cases
Treatment for mild cases is based on providing temporary relief by inserting an oral airway, which helps with breathing.
Sometimes a child with unilateral choanal atresia does not have significant respiratory distress or feeding problems.
These patients can be treated initially with close observation and occasionally supplemental oxygen.
Nasal saline can help keep your nasal passages healthy and discharge-free.
Parents of children diagnosed with unilateral choanal atresia can wait until the child grows up before deciding to repair the condition.
Treatment for severe cases
If the child is diagnosed with bilateral choanal atresia, the condition should be treated as soon as possible.
Surgery should be performed as soon as the patient is stable and has been evaluated for other abnormalities.
The main goal of surgical repair is to open the nasal airway sufficiently, allowing the child to breathe on his or her own.
Surgical intervention to permanently correct the defect: This is done by removing the obstruction to create a nasal airway, which is done through the nose or mouth.
There are several approaches available to achieve this goal, all of which are performed in the operating room under general anesthesia.
Transnasal endoscopic approach
Currently, surgeons prefer to treat children with choanal atresia using the transnasal endoscopic approach.
It can be done safely in babies a few days old.
The surgeon places a small visor with small instruments attached through the baby’s nose
To do this, the surgeon makes a cut in the roof of the baby’s mouth and removes the tissue or bone that is blocking the baby’s breathing.
Less commonly, surgery is done through an open procedure.
Babies with other problems, such as CHARGE syndrome, may need more surgeries to treat these conditions.
In some cases, a piece of endotracheal tube is placed inside the nasal passage as a stent to hold the opening while the area heals.
It is drilled through the bone (if the obstruction is created by a bone) and a stent is inserted.
The stent will be removed a few weeks after surgery.
Transpalatal approach
On the rare occasion when the skull base develops abnormally (other craniofacial abnormalities exist), the surgeon may need to use a transpalatal approach to reach the area of choanal atresia through the roof of the mouth.
Aero-digestive evaluation
If the baby has choanal atresia, he should be evaluated for feeding and airway problems.
Airway management often requires a combination of medical, surgical, and supportive care.
Feeding and swallowing problems are very common in children with these conditions and often need to be addressed by speech pathologists and specialists.
Children born with syndromes often need other subspecialty evaluations (cardiology, ophthalmology, etc.) and benefit greatly from coordinated care.
Complications of choanal atresia
Choanal atresia could lead to potential complications.
Babies with bilateral choanal atresia often turn blue while sleeping or eating because they cannot get enough oxygen.
In rare cases, if not treated properly, it can cause death by aspiration while breastfeeding.
Respiratory arrest may occur, breathing may slow or even stop
Prevention of choanal atresia
Currently, there are no known methods or guidance for the prevention of choanal atresia.
However, pregnant women should consider the following general health measures that can minimize the risk of the condition:
- Avoid smoking or receiving secondhand smoke.
- Avoid certain medications or chemicals that can put the baby at risk for the condition.
- Ensure the intake of a well balanced diet (minerals and vitamins) during pregnancy.
- Minimize or limit the consumption of coffee or caffeinated beverages.
Prognosis of choanal atresia
The prognosis for choanal atresia is considered very good, with effective early management, timely surgery, and proper care of the baby.
Babies with a mild form of unilateral choanal atresia may not need treatment. However, they will need to be watched closely for any signs of a breathing problem.
Using saline nasal spray can also help keep the nostril open.
Once the blockage has been removed, the outlook for infants with choanal atresia is good. They can grow to have normal, healthy lives.
