Pinnae fistula or cyst

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Overview

Pinnae fistula or cyst, also known as preauricular sinus, preauricular fistula, or preauricular pit, is a common congenital malformation resulting from incomplete or abnormal fusion of the auricular hillocks during embryonic development of the external ear. The condition presents as a small pit, sinus tract, or cyst located typically near the anterior margin of the ascending helix of the ear (pinna). It may be unilateral or bilateral and can occur as an isolated finding or, less commonly, as part of a broader syndrome such as branchio-oto-renal syndrome or other first and second branchial arch anomalies. Most individuals with preauricular sinuses are asymptomatic and the condition is discovered incidentally. However, the sinus tract can become a site of recurrent infection, leading to abscess formation, discharge of purulent or sebaceous material, pain, and swelling in the preauricular region. Repeated infections can cause scarring and chronic drainage. The condition primarily affects the skin and soft tissues surrounding the external ear. Treatment is generally not required for asymptomatic preauricular sinuses. When recurrent infections occur, acute episodes are managed with antibiotics and, if necessary, incision and drainage of abscesses. Definitive treatment involves complete surgical excision of the sinus tract and any associated cyst to prevent recurrence. Surgical excision is recommended after recurrent infections, as incomplete removal can lead to recurrence. Prognosis is excellent following complete excision. In cases where the preauricular sinus is associated with a syndromic condition, evaluation for associated anomalies, particularly renal and hearing abnormalities, may be warranted.

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 Pinnae fistula or cyst.

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No actively recruiting trials found for Pinnae fistula or cyst at this time.

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No specialists are currently listed for Pinnae fistula or cyst.

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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

No travel grants are currently matched to Pinnae fistula or cyst.

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Community

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Common questions about Pinnae fistula or cyst

What is Pinnae fistula or cyst?

Pinnae fistula or cyst, also known as preauricular sinus, preauricular fistula, or preauricular pit, is a common congenital malformation resulting from incomplete or abnormal fusion of the auricular hillocks during embryonic development of the external ear. The condition presents as a small pit, sinus tract, or cyst located typically near the anterior margin of the ascending helix of the ear (pinna). It may be unilateral or bilateral and can occur as an isolated finding or, less commonly, as part of a broader syndrome such as branchio-oto-renal syndrome or other first and second branchial arch

At what age does Pinnae fistula or cyst typically begin?

Typical onset of Pinnae fistula or cyst is neonatal. Age of onset can vary across affected individuals.