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.
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Choanal atresia, unilateral.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Choanal atresia, unilateral.
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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.