Choanal atresia, unilateral

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:137917Q30.0
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Choanal atresia, unilateral, is a congenital condition in which one of the two choanae — the posterior nasal passages that connect the nasal cavity to the nasopharynx — is blocked by abnormal bony or membranous tissue. Unlike bilateral choanal atresia, which presents as a neonatal emergency due to obligate nasal breathing in newborns, unilateral choanal atresia often has a more subtle presentation and may not be diagnosed until later in infancy or childhood. The condition primarily affects the upper respiratory system, specifically the nasal airway on the affected side. Patients with unilateral choanal atresia typically present with chronic unilateral nasal obstruction, persistent mucoid nasal discharge on the affected side, and difficulty breathing through one nostril. Some infants may experience feeding difficulties, though these are generally less severe than in the bilateral form. The condition may be isolated or can occur as part of a broader syndrome, most notably CHARGE syndrome (coloboma, heart defects, atresia choanae, retardation of growth, genital abnormalities, and ear anomalies). Diagnosis is typically confirmed by the inability to pass a catheter through the affected nasal passage and by CT imaging of the nasal cavity and nasopharynx, which delineates the type (bony, membranous, or mixed) and extent of the atresia. Treatment for unilateral choanal atresia is surgical, with the goal of creating a patent nasal airway. The most commonly used approach is transnasal endoscopic repair, in which the atretic plate is perforated and the opening is widened, sometimes with temporary stenting to prevent restenosis. Because unilateral cases are not immediately life-threatening, surgery can often be planned electively, allowing the child to grow before intervention. Restenosis remains a recognized complication, and some patients may require revision surgery. Long-term outcomes are generally favorable with appropriate surgical management.

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Choanal atresia, unilateral.

View clinical trials →

No actively recruiting trials found for Choanal atresia, unilateral at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Choanal atresia, unilateral community →

No specialists are currently listed for Choanal atresia, unilateral.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Choanal atresia, unilateral.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Choanal atresia, unilateralForum →

No community posts yet. Be the first to share your experience with Choanal atresia, unilateral.

Start the conversation →

Latest news about Choanal atresia, unilateral

No recent news articles for Choanal atresia, unilateral.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Choanal atresia, unilateral

What is Choanal atresia, unilateral?

Choanal atresia, unilateral, is a congenital condition in which one of the two choanae — the posterior nasal passages that connect the nasal cavity to the nasopharynx — is blocked by abnormal bony or membranous tissue. Unlike bilateral choanal atresia, which presents as a neonatal emergency due to obligate nasal breathing in newborns, unilateral choanal atresia often has a more subtle presentation and may not be diagnosed until later in infancy or childhood. The condition primarily affects the upper respiratory system, specifically the nasal airway on the affected side. Patients with unilater

At what age does Choanal atresia, unilateral typically begin?

Typical onset of Choanal atresia, unilateral is neonatal. Age of onset can vary across affected individuals.