Frontorhiny

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ORPHA:391474OMIM:136760Q75.8
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1Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Frontorhiny is an extremely rare genetic condition that affects the development of the face, particularly the forehead and nose areas. The name comes from 'fronto' (forehead) and 'rhiny' (nose), reflecting the main areas of the body that are affected. People with frontorhiny typically have widely spaced eyes (hypertelorism), a broad nasal bridge, a flat or bifid (split) nose tip, and sometimes a groove or cleft in the middle of the nose. The forehead may appear broad, and there can be a widow's peak hairline. Some individuals may also have a cleft in the upper lip or other midline facial differences. The condition is present from birth and is caused by problems in how the facial structures develop during pregnancy. Frontorhiny is classified as a craniofacial malformation, meaning it primarily involves the bones and soft tissues of the skull and face. Intelligence is usually normal in affected individuals, which is an important distinction from some other craniofacial conditions. The severity of facial features can vary from person to person, even within the same family. Treatment is mainly surgical and focuses on correcting the facial differences to improve both function and appearance. A team of specialists, including craniofacial surgeons, ear-nose-throat doctors, and geneticists, typically work together to manage the condition. While there is no cure that addresses the underlying genetic cause, surgical interventions can significantly improve quality of life and facial appearance.

Also known as:

Key symptoms:

Widely spaced eyesBroad or flat nasal bridgeSplit or bifid nose tipGroove or cleft along the middle of the noseBroad foreheadWidow's peak hairlineCleft upper lip in some casesShort noseThickened or underdeveloped nostrilsMidline facial cleftingSkin tags or extra tissue on the noseNormal intelligence in most cases

Clinical phenotype terms (29)— hover any for plain English
Widow's peakHP:0000349Hypoplastic frontal sinusesHP:0002738Midline nasal grooveHP:0004112Cranium bifidum occultumHP:0004423Pericallosal lipomaHP:0006931
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 Frontorhiny.

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No actively recruiting trials found for Frontorhiny at this time.

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Specialists

1 foundView all specialists →
SD
Sarah R Cope, DClinPsy
Specialist
PI on 1 active trial

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 Frontorhiny.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Frontorhiny

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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 facial features does my child have that are related to frontorhiny, and how might they change as my child grows?,What surgeries are recommended, and at what ages should they be performed?,Should we pursue genetic testing to confirm the diagnosis, and what does this mean for future pregnancies?,Are there any breathing or feeding concerns we should watch for right now?,What kind of psychological or emotional support services do you recommend for my child?,How often should we schedule follow-up appointments with the craniofacial team?,Are there any other health problems associated with frontorhiny that we should screen for?

Common questions about Frontorhiny

What is Frontorhiny?

Frontorhiny is an extremely rare genetic condition that affects the development of the face, particularly the forehead and nose areas. The name comes from 'fronto' (forehead) and 'rhiny' (nose), reflecting the main areas of the body that are affected. People with frontorhiny typically have widely spaced eyes (hypertelorism), a broad nasal bridge, a flat or bifid (split) nose tip, and sometimes a groove or cleft in the middle of the nose. The forehead may appear broad, and there can be a widow's peak hairline. Some individuals may also have a cleft in the upper lip or other midline facial diffe

How is Frontorhiny inherited?

Frontorhiny follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Frontorhiny typically begin?

Typical onset of Frontorhiny is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Frontorhiny?

1 specialists and care centers treating Frontorhiny are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.