OBSOLETE: Syndromic frontonasal dysplasia

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ORPHA:391479
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8Treatment centers

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Overview

Syndromic frontonasal dysplasia refers to a group of rare birth defects that affect the development of the face, particularly the middle part of the face including the nose, forehead, and the area between the eyes. The term 'OBSOLETE' in the official name means that this category has been retired and reclassified into more specific subtypes in medical databases. Patients previously grouped under this label are now categorized into more precise diagnoses such as frontonasal dysplasia with alopecia and genital anomalies (ALX homeobox gene-related), acromelic frontonasal dysplasia, or other specific syndromic forms. In these conditions, the face does not form properly during early pregnancy. Common features include a wide space between the eyes (hypertelorism), a broad or split nose, a cleft in the middle of the upper lip, and sometimes a gap in the front part of the skull. Because these are 'syndromic' forms, other body systems may also be affected, including the brain, limbs, eyes, or genitals, depending on the specific subtype. Treatment is mainly supportive and surgical. Reconstructive surgery can help correct facial differences, and other specialists may be needed depending on which body systems are involved. Genetic testing is important to determine the exact subtype, which helps guide treatment and predict what other health issues may arise. Early diagnosis allows families to plan appropriate care and connect with the right medical team.

Also known as:

Key symptoms:

Wide-set eyesBroad or split noseCleft lip or palate in the midline of the faceGap or defect in the forehead bonesFlat or underdeveloped middle faceHair loss or sparse hair (in some subtypes)Abnormalities of the fingers or toesGenital differences (in some subtypes)Intellectual disability (in some cases)Brain abnormalities such as missing or underdeveloped brain structuresShort stature or limb differencesEye abnormalities or vision problemsSkin tags or extra tissue on the face

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 OBSOLETE: Syndromic frontonasal dysplasia.

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No actively recruiting trials found for OBSOLETE: Syndromic frontonasal dysplasia at this time.

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No specialists are currently listed for OBSOLETE: Syndromic frontonasal dysplasia.

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 OBSOLETE: Syndromic frontonasal dysplasia.

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Community

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Latest news about OBSOLETE: Syndromic frontonasal dysplasia

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

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 subtype of frontonasal dysplasia does my child have, and what gene is involved?,What surgeries will be needed, and what is the typical timeline?,Are there any brain abnormalities, and how might they affect my child's development?,What therapies (speech, developmental, etc.) should we start, and when?,Are there other organs or body systems we need to monitor?,What is the chance of this condition occurring again in future pregnancies?,Can you connect us with a craniofacial team and support groups for families?

Common questions about OBSOLETE: Syndromic frontonasal dysplasia

What is OBSOLETE: Syndromic frontonasal dysplasia?

Syndromic frontonasal dysplasia refers to a group of rare birth defects that affect the development of the face, particularly the middle part of the face including the nose, forehead, and the area between the eyes. The term 'OBSOLETE' in the official name means that this category has been retired and reclassified into more specific subtypes in medical databases. Patients previously grouped under this label are now categorized into more precise diagnoses such as frontonasal dysplasia with alopecia and genital anomalies (ALX homeobox gene-related), acromelic frontonasal dysplasia, or other speci

At what age does OBSOLETE: Syndromic frontonasal dysplasia typically begin?

Typical onset of OBSOLETE: Syndromic frontonasal dysplasia is neonatal. Age of onset can vary across affected individuals.