Supernumerary nostril

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ORPHA:141096Q30.8
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Overview

Supernumerary nostril (also known as accessory nostril or third nostril) is an extremely rare congenital malformation of the nose characterized by the presence of one or more additional nostril-like openings, typically located near the normal nostrils. This condition is classified among other congenital malformations of the nose (ICD-10: Q30.8). The accessory nostril may be a simple blind-ending pit or may communicate with the nasal cavity, and it can vary in size and degree of development. It may contain cartilage, sebaceous glands, and hair follicles similar to a normal nostril. The extra nostril is usually unilateral and is most commonly found medial, lateral, or superior to the normal nostril. Supernumerary nostril is present from birth and is typically identified in the neonatal period or early infancy. In most reported cases, the condition is an isolated anomaly, though it may occasionally be associated with other craniofacial malformations such as cleft lip or palate, nasal dermoid cysts, or other midline defects. The condition primarily affects the external nose and nasal passages. Functionally, the accessory nostril may or may not be patent (open to the nasal cavity). Patients may be asymptomatic or may experience cosmetic concerns, nasal discharge from the accessory opening, or rarely, recurrent infections. Treatment is primarily surgical and is undertaken for cosmetic reasons or to address functional complications such as obstruction or recurrent infection. Surgical excision with rhinoplasty techniques can achieve good cosmetic and functional outcomes. Given the rarity of this condition, management is individualized and typically involves a multidisciplinary team including otolaryngologists and plastic surgeons. Fewer than 100 cases have been reported in the medical literature worldwide.

Also known as:

Clinical phenotype terms— hover any for plain English:

Supernumerary narisHP:0009934Tessier cleftHP:0002006Abnormality of ethmoid sinusHP:3000040
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Supernumerary nostril.

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No specialists are currently listed for Supernumerary nostril.

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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

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Common questions about Supernumerary nostril

What is Supernumerary nostril?

Supernumerary nostril (also known as accessory nostril or third nostril) is an extremely rare congenital malformation of the nose characterized by the presence of one or more additional nostril-like openings, typically located near the normal nostrils. This condition is classified among other congenital malformations of the nose (ICD-10: Q30.8). The accessory nostril may be a simple blind-ending pit or may communicate with the nasal cavity, and it can vary in size and degree of development. It may contain cartilage, sebaceous glands, and hair follicles similar to a normal nostril. The extra no

How is Supernumerary nostril inherited?

Supernumerary nostril follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Supernumerary nostril typically begin?

Typical onset of Supernumerary nostril is neonatal. Age of onset can vary across affected individuals.