Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome

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ORPHA:357158OMIM:602562Q87.0
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Overview

Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome is an extremely rare genetic condition that affects the development of the face and skull. The name describes its main features: mandibulofacial dysostosis refers to abnormal development of the jaw and facial bones, macroblepharon means unusually large eyelid openings, and macrostomia means an abnormally wide mouth. This syndrome belongs to a group of conditions sometimes called mandibulofacial dysostoses, which share problems with how the bones and soft tissues of the face form before birth. People with this condition typically have distinctive facial features that are present from birth. These may include underdeveloped cheekbones, a small or recessed lower jaw, a wide mouth, large eyelid openings that may expose more of the eye than usual, and sometimes ear abnormalities. Some individuals may also experience hearing difficulties and dental problems due to the abnormal jaw development. Because this syndrome is so rare, there is no specific cure. Treatment focuses on managing individual symptoms and may involve surgery to correct facial abnormalities, dental care, hearing support, and eye protection. A team of specialists typically works together to address the various aspects of this condition. Early intervention and coordinated care can significantly improve quality of life and functional outcomes for affected individuals.

Also known as:

Key symptoms:

Underdeveloped cheekbonesSmall or recessed lower jawAbnormally wide mouthUnusually large eyelid openingsDownward-slanting eyesEar abnormalities or malformed earsHearing loss or hearing difficultiesDental problems or misaligned teethDifficulty closing the eyes completelyDry eyes due to increased eye exposureFeeding difficulties in infancySpeech difficulties

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome.

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No actively recruiting trials found for Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome community →

No specialists are currently listed for Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome.

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 Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

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

Questions for your doctor

Bring these to your next appointment

  • Q1.What specific features of this syndrome does my child have, and how severe are they?,Will my child need surgery, and if so, what procedures and at what ages?,Should we have genetic testing done, and what can it tell us about the cause and recurrence risk?,How should we monitor and protect my child's eyes and hearing over time?,Are there any breathing or airway concerns we should watch for?,What therapies (speech, feeding, etc.) should we start, and when?,Can you refer us to a multidisciplinary craniofacial team for coordinated care?

Common questions about Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome

What is Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome?

Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome is an extremely rare genetic condition that affects the development of the face and skull. The name describes its main features: mandibulofacial dysostosis refers to abnormal development of the jaw and facial bones, macroblepharon means unusually large eyelid openings, and macrostomia means an abnormally wide mouth. This syndrome belongs to a group of conditions sometimes called mandibulofacial dysostoses, which share problems with how the bones and soft tissues of the face form before birth. People with this condition typically h

How is Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome inherited?

Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome typically begin?

Typical onset of Mandibulofacial dysostosis-macroblepharon-macrostomia syndrome is neonatal. Age of onset can vary across affected individuals.