Choanal atresia

Choanal atresia is a

Choanal atresia is diagnosed based on the inability to place a nasal catheter, and radiology results (particularly CT scans). Treatment involves maintaining an open airway, and may involve surgery to reopen the airway, potentially with a stent. Choanal atresia is a fairly rare condition, affecting between 1 in 7,000 to 1 in 5,000 live births. It is more common in females and is more often unilateral.

Presentation

Choanal atresia can be unilateral or bilateral.

• A unilateral choanal atresia may not be detected until much later in life because the baby manages to get along with only one nostril available for breathing.^[1] Symptoms are minor, including persistent rhinorrhea (mainly normal mucus) and chronic sinusitis.^[1]
• Bilateral choanal atresia is a life-threatening condition because the baby will be unable to breathe directly after birth as babies are obligate nasal breathers (they mainly use their noses to breathe).^[1] In some cases, this may present as cyanosis while the baby is feeding, because the oral air passages are blocked by the tongue, further restricting the airway.^[2] Cyanosis may improve when the baby cries, as the oral airway is used.^[1] These babies may require airway resuscitation soon after birth.

Associated conditions

Choanal atresia is associated with a higher risk of other airway problems, including:

• tracheomalacia.^[1]
• laryngomalacia.^[1]
• subglottic stenosis.^[1]

Sometimes, babies born with choanal atresia also have other abnormalities:

• coloboma.^[1]
• heart defects and cardiovascular disease.^[1]
• intellectual disability.^[1]
• growth impairment.^[1]
• genital hypoplasia.^[1]
• CHARGE syndrome.^[1]
• others.

Also any condition that causes significant depression of the nasal bridge or midface retraction can be associated with choanal atresia. Examples include the

Cause

Choanal atresia is caused by problems with the development of the

Very few risk factors for choanal atresia have been identified. In general, choanal atresia is associated with a higher risk of other birth defects.^[1] Bilateral choanal atresia is more associated than unilateral choanal atresia.^[1]

While causes are unknown, both genetic and environmental triggers are suspected.

Choanal atresia causes closure of the posterior choanae in the nasal cavity.^[1] Around 30% of these affect just the bone, while around 70% affect both bone and membranes.^[1] Bones affected can include the body of the sphenoid bone, the vomer, the medial pterygoid process of the sphenoid bone, and the horizontal plate of the palatine bone.^[1]

Diagnosis

Nasal catheter

Choanal atresia can be suspected if it is impossible to insert a nasal catheter.^[2] The length of catheter that can be inserted indicates where choanal atresia has occurred: shorter distances indicate a problem with the vomer, while longer distances indicate a problem with the posterior choanae.^[1] Mucus can be cleared (using suction) to visualise the abnormality.^[1]

Radiology

Diagnosis is confirmed using CT scan.^[1][2] This is also useful for differential diagnosis.^[1]

Treatment

Airway management

As bilateral choanal atresia is an emergency, the airway is secured. A small tube may be placed to the

Choanal atresia is fairly rare.[6] It may have a frequency between 1 in 7,000 births and 1 in 5,000 births.^[1]

History

Choanal atresia was first described by Roederer in 1755.^[9]

Society and culture

In the movie City of Angels, Dr. Maggie Rice (played by Meg Ryan) correctly diagnoses the cause of a newborn baby's failure to thrive as due to choanal atresia.^{[citation needed]}

References