Tessier number 6 facial cleft

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Overview

Tessier number 6 facial cleft is an extremely rare congenital craniofacial malformation classified within the Tessier cleft system, which categorizes rare facial clefts based on their anatomical position relative to the orbit. The Tessier number 6 cleft extends from the medial aspect of the lower eyelid (near the inner canthus) downward through the cheek toward the oral commissure region, running between the Tessier number 5 and number 7 clefts. It involves both soft tissue and potentially the underlying bony structures, specifically affecting the area between the maxillary bone (zygomatic process of the maxilla) and the zygoma. Clinically, this cleft may present as a groove or fissure in the cheek, coloboma of the lower eyelid, disruption of the lateral nasal and cheek soft tissues, and incomplete formation of the zygomatic-maxillary junction. Affected individuals may exhibit asymmetry of the midface, soft tissue deficiency along the cleft line, lower eyelid abnormalities including coloboma, and potential exposure of the orbit. The cleft can vary in severity from a subtle soft tissue notch to a complete cleft involving both skin and bone. Associated dental anomalies and orbital dystopia may also be present depending on the extent of the malformation. As with other rare Tessier clefts, the condition is apparent at birth. Treatment is primarily surgical and requires a multidisciplinary craniofacial team. Surgical reconstruction aims to restore facial symmetry, close the cleft defect, reconstruct the lower eyelid and cheek soft tissues, and address any underlying bony deficiencies. Multiple staged surgeries may be necessary throughout childhood. Supportive care may include ophthalmologic monitoring, dental and orthodontic management, and speech therapy if oral function is affected. There is no known medical or pharmacological treatment for the structural defect itself.

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 Tessier number 6 facial cleft.

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No actively recruiting trials found for Tessier number 6 facial cleft at this time.

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No specialists are currently listed for Tessier number 6 facial cleft.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Tessier number 6 facial cleft.

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Community

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Caregiver Resources

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Tessier number 6 facial cleft

What is Tessier number 6 facial cleft?

Tessier number 6 facial cleft is an extremely rare congenital craniofacial malformation classified within the Tessier cleft system, which categorizes rare facial clefts based on their anatomical position relative to the orbit. The Tessier number 6 cleft extends from the medial aspect of the lower eyelid (near the inner canthus) downward through the cheek toward the oral commissure region, running between the Tessier number 5 and number 7 clefts. It involves both soft tissue and potentially the underlying bony structures, specifically affecting the area between the maxillary bone (zygomatic pro

How is Tessier number 6 facial cleft inherited?

Tessier number 6 facial cleft follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Tessier number 6 facial cleft typically begin?

Typical onset of Tessier number 6 facial cleft is neonatal. Age of onset can vary across affected individuals.