Commissural lip fistula

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ORPHA:141061Q38.0
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Overview

Commissural lip fistula, also known as commissural lip pit or congenital fistula of the lip commissure, is a rare congenital anomaly affecting the oral region. It is classified under congenital malformations of the lips (ICD-10: Q38.0). The condition is characterized by the presence of a small pit, sinus tract, or fistula located at or near the commissure (corner) of the mouth. These fistulae are believed to result from a developmental defect during embryogenesis, specifically involving incomplete fusion of the maxillary and mandibular processes that form the oral commissure. The condition primarily affects the orofacial structures and is typically present at birth. Clinically, commissural lip fistulae usually appear as small, often bilateral, openings at the corners of the mouth. They may be asymptomatic and discovered incidentally, or they may occasionally produce mucoid secretions from minor salivary glands that drain into the tract. In most cases, the condition is benign and does not cause significant functional impairment. However, some patients may experience cosmetic concerns, recurrent local irritation, or mild infection of the fistulous tract. Commissural lip fistulae should be distinguished from other congenital lip pits, particularly those associated with Van der Woude syndrome, which involve the lower lip and are associated with cleft lip and/or palate. Treatment, when indicated, is primarily surgical. Simple excision of the fistulous tract is the standard approach and is generally curative. Surgery may be recommended for cosmetic reasons, recurrent symptoms, or to prevent potential complications such as infection. The prognosis following surgical excision is excellent, with low recurrence rates. In asymptomatic cases, observation without intervention may be appropriate.

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 Commissural lip fistula.

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No actively recruiting trials found for Commissural lip fistula at this time.

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No specialists are currently listed for Commissural lip fistula.

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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 Commissural lip fistula.

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Community

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Latest news about Commissural lip fistula

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Common questions about Commissural lip fistula

What is Commissural lip fistula?

Commissural lip fistula, also known as commissural lip pit or congenital fistula of the lip commissure, is a rare congenital anomaly affecting the oral region. It is classified under congenital malformations of the lips (ICD-10: Q38.0). The condition is characterized by the presence of a small pit, sinus tract, or fistula located at or near the commissure (corner) of the mouth. These fistulae are believed to result from a developmental defect during embryogenesis, specifically involving incomplete fusion of the maxillary and mandibular processes that form the oral commissure. The condition pri

How is Commissural lip fistula inherited?

Commissural lip fistula follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Commissural lip fistula typically begin?

Typical onset of Commissural lip fistula is neonatal. Age of onset can vary across affected individuals.