Choanal Atresia

Choanal atresia (CA) is a rare condition where a baby is born with tissue blocking their nasal airway. It’s also called congenital choanal atresia (pronounced “ko-UH-nul uh-TREE-zhuh”). The blockage may consist of bone or a mix of bone and soft tissue. It can affect one or both sides of your baby’s nose.

Blockages in your child’s nasal passages may make it harder for them to breathe. But how serious the issue is depends on whether the blockage affects one or both nasal passages. Either way, surgery can fix it.

Some babies with CA need surgery immediately. Others can wait until they’re older. Healthcare providers will advise you based on what’s best for your baby.

Types of choanal atresia

There are two types:

• Unilateral choanal atresia: Most children with CA have a blockage in just one side of their nose. They often learn to breathe through one nasal passage, so symptoms may not show up right away.
• Bilateral choanal atresia: Newborns with blockages in both nasal passages need emergency care to help them breathe.

Symptoms of choanal atresia

The bilateral type usually shows up at birth. Newborns with a blockage in both sides of their nose have trouble breathing unless they’re crying. Their skin and lips may turn blue. In these instances, healthcare providers rush to provide lifesaving care.

Babies with a blockage in one side of their nose often have mild symptoms. You may not notice them until your baby is older. Symptoms include:

• A stuffy or runny nose that only affects one side (most common)
• A newborn whose chest sinks in unless they’re crying
• A newborn that’s fussy during feedings
• Long-lasting sinus infections

Choanal atresia causes

The exact cause is unknown. Most experts believe it happens when the piece of tissue that separates the mouth and nose during fetal development stays intact after a baby is born.

It’s about twice as likely to affect female babies. Experts don’t know why.

What syndromes are associated with choanal atresia?

About half of babies with CA are born with genetic conditions that impact how their bodies develop. Conditions include:

• CHARGE syndrome (most common)
• Treacher Collins syndrome
• Crouzon syndrome
• DiGeorge syndrome
• Pfeiffer syndrome
• Antley-Bixler syndrome
• Marshall-Smith syndrome
• Schinzel-Giedion syndrome

Complications of this condition

Your child may struggle to get enough air while eating. When this happens, food can go down their windpipe instead of their esophagus (food tube). This can lead to pneumonia and be serious without treatment.

Your healthcare provider will suggest timely treatment to protect your child from risks.

How doctors diagnose this condition

Healthcare providers usually diagnose newborns with bilateral choanal atresia at birth. They may hold up a small mirror to your baby’s nose to see if they’re breathing enough air to fog it up.

Tests that check for a blockage in your baby’s nasal passages include:

• Nasal endoscopy: Your baby’s provider inserts a thin tube with a small camera (endoscope) into their nose so they can view the tissue inside.
• CT scan: This test creates detailed 3D images that can show blockages in your baby’s nasal passages. It can help their provider plan surgery.

Your baby’s care team will ensure they’re getting the breathing support they need during these tests to keep them safe.

How is choanal atresia treated?

Surgery can fix this condition. Babies born with bilateral CA need surgery as soon as possible. You may be able to delay surgery if the blockage is only on one side and isn’t impacting your child’s quality of life. Waiting can be worth it if it gives your child time to grow into their facial features more. This can lead to better surgery results.

Surgeons most often use a technique called transnasal endoscopic repair. They’ll use a camera with operating instruments that can go up your child’s nose. (That way, surgeons don’t have to make cuts to your child’s face.) Using the tools, they create a hole through the excess nasal tissue. They may place absorbable stents inside your child’s nasal passage to keep it open while it heals.

Your child will be asleep through the entire procedure, so they won’t feel any pain. Most children with unilateral atresia go home the same day. Infants who need bilateral repair will likely spend a few nights in the hospital so their care team can make sure they’re recovering well.

When should I see a healthcare provider?

Schedule a visit with your child’s pediatrician if you suspect your child is having trouble feeding or getting enough air. Sometimes, choanal atresia symptoms don’t surface until later — especially in mild CA that only affects one side. But it can negatively impact your child’s quality of life until it’s treated.

It’s important to get your child checked out so they get the care they need.

What can I expect if my baby has this condition?

Treatment for choanal atresia is usually successful. Most babies make a full recovery after surgery.

But recurrence is possible. This can cause the area to become too narrow, which may cause symptoms to return. Recurrence is more common in babies with bilateral CA who also have a related condition, like CHARGE syndrome. If this happens, their healthcare provider may need to do revision surgery.

Your child’s provider can explain how likely it is that they may need another procedure based on their condition and surgery results. But most children breathe better and have an improved quality of life after surgery.