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:
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Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Supernumerary nostril.
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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
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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.