Choanal Atresia

What is choanal atresia?

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

Blockages or narrowing (choanal stenosis) in your child’s nasal passages may make it harder for them to breathe. But how serious the issue is (and how soon they’ll need treatment) depends on the extent of the blockage and whether it blocks one or both nasal passages.

Types of choanal atresia

The two types of choanal atresia are:

• Unilateral choanal atresia. Most infants with chronic atresia have a nasal blockage in just one side of their nose. As they can learn to breathe through one nasal passage, symptoms may not show up until later in childhood or even adulthood.
• Bilateral choanal atresia. Newborns with blockages in both nasal passages need life-saving emergency care to help them breathe. This is because infants breathe almost entirely through their noses. (Typically, babies only breathe through their mouths if they’re crying.) Your provider can insert a small tube into your baby’s windpipe immediately so they can get air. Once your baby is stable, your provider can perform surgery to permanently correct the problem.

How common is choanal atresia?

Choanal atresia occurs in about 1 out of every 7,000 live births. More than 60% of diagnoses are unilateral choanal atresia. The condition is about twice as likely to affect babies assigned female at birth (AFAB).

What are the symptoms of choanal atresia?

Healthcare providers often recognize symptoms of bilateral choanal atresia at birth. Babies with choanal atresia affecting both sides of their nose have trouble breathing unless they’re crying. Their skin and lips may look blue (cyanosis), and they may be unable to breathe. If this happens, your provider will resuscitate your baby immediately.

Babies that have a blockage in one side of their nose (unilateral choanal atresia) usually have milder symptoms that may not show up until you take them home. They may not show up until childhood. Symptoms include:

• A newborn whose chest sinks in unless they’re crying.
• A newborn that’s fussy during feedings (mouth breathing is especially difficult for an infant trying to nurse).
• Runny or stuffy noses that only affect one side (one-sided nasal drainage that doesn’t stop is the most common sign of choanal atresia).
• Long-lasting sinus infections (chronic sinusitis).

What causes choanal atresia?

The exact cause of choanal atresia is unknown. Most medical 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.

Conditions associated with choanal atresia

About half of the babies with choanal atresia are born with inherited syndromes and genetic disorders associated with other developmental abnormalities. Medical experts don’t know why. Associated conditions include:

• CHARGE syndrome. (About 60% of babies born with bilateral choanal atresia have CHARGE syndrome.)
• Treacher Collins syndrome.
• Crouzon syndrome.
• Pfeiffer syndrome.
• Antley-Bixler syndrome.
• Marshall-Smith syndrome.
• Schinzel-Giedion syndrome.

What are the complications of this condition?

Over time, untreated choanal atresia can cause long-term effects related to fluid build-up in the blocked nasal passage. For example, your child may get frequent ear infections. Eventually, damage to their ear can lead to hearing loss. This is more common if your child also has CHARGE syndrome.

They may struggle to get enough air while eating and aspirate (food goes down their windpipe instead of their esophagus).

Your healthcare provider can provide treatment sooner rather than later if they’re concerned about these risks.

How is choanal atresia diagnosed?

Healthcare providers usually diagnose newborns with bilateral choanal atresia while they’re still in the hospital. A provider may hold up a laryngeal mirror (the tiny mirror your dentist uses) to your baby’s nose to see if they’re breathing enough air to fog it up.

If they suspect your baby has choanal atresia, they may perform tests, including:

• CT (computed tomography) scan. This imaging test creates detailed 3D images that can show structural abnormalities or blockages in your baby’s nasal passages.
• Nasal endoscopy. Your provider will insert a thin tube with a small camera (endoscope) into your baby’s nose to get a better look at their nasal structures.

Your baby’s provider will ensure they’re getting the breathing support they need throughout these tests so there aren’t any risks.

How is choanal atresia treated?

Surgery is the only long-term treatment for choanal atresia. If your baby is born with blockages in both nasal passages, they’ll need surgery as soon as possible. In some cases, infants with blockages on one side learn to breathe through their mouths, delaying the need for immediate surgery. In that case, you can work to manage symptoms at home.

But eventually, babies and children need choanal atresia repair to improve their health and quality of life. Endoscopic choanal atresia repair is the most common approach.

Surgery

During choanal atresia repair, the surgeon creates a hole through the bone or tissue that’s blocking your baby’s airway. This can be achieved through the nose (transnasal) or roof of the mouth (transpalatal). In some cases, they may place a stent (a small tube) inside your baby’s nasal passage to keep the airway open during healing. Your baby’s surgeon will remove the stent a few months after placement.

Surgeons perform choanal atresia repair in an operating room while your baby is under general anesthesia.

Management

If your baby has unilateral choanal atresia, you and your child’s provider may decide to wait on surgery until they’re older. This is fine, as long as your baby doesn’t have severe breathing problems or difficulty eating. In the meantime, ask your provider how to properly manage choanal atresia symptoms. For example, you may need to flush their nasal passages with saline to keep the tissues clean and healthy.

What can I expect if my baby has choanal atresia?

Your healthcare provider will discuss all treatment options with you in detail if your baby is born with choanal atresia. With unilateral choanal atresia, they’ll let you know when it’s safe to wait on surgery and when delaying poses risks.

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

When should I call my healthcare provider?

Schedule a visit with a healthcare provider any time you notice changes in the way your baby or child is breathing or eating. Sometimes, choanal atresia symptoms don’t surface until later — especially in mild unilateral cases. It’s important to get them checked.

What questions should I ask my healthcare provider?

• Does my baby have blockages in one nasal passage or both?
• Is my child’s condition associated with another syndrome like CHARGE?
• When will they need surgery?
• What surgical approach do you recommend?
• How will we know the surgery was successful?