Cerebrooculonasal syndrome

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ORPHA:66625OMIM:605627Q87.0
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8Treatment centers

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Overview

Cerebrooculonasal syndrome is a very rare condition present from birth that affects the brain, eyes, and nose together. It is sometimes called CEONS for short. Babies born with this syndrome typically have a combination of brain abnormalities, unusual eye development, and structural differences in the nose and face. The brain changes can include problems with how the brain formed before birth, which may lead to intellectual disability and developmental delays. Eye problems are a central feature and can range from very small or absent eyes (microphthalmia or anophthalmia) to other structural eye defects that affect vision. Nasal differences, such as a broad or malformed nose, are also commonly seen. Because this syndrome affects multiple body systems at once, children often need care from several different specialists. There is no cure for cerebrooculonasal syndrome, and treatment focuses on managing each symptom as it appears. This may include early intervention therapies like speech, occupational, and physical therapy, as well as support for vision impairment and educational accommodations. The severity of the condition can vary from child to child, and the overall outlook depends largely on how severely the brain and other organs are affected.

Key symptoms:

Very small or absent eyes at birthBrain malformations present before birthIntellectual disability or developmental delaysBroad, flat, or malformed noseLow or absent visionUnusual facial featuresDelayed speech and language developmentDelayed motor milestones such as sitting or walkingAbnormal skull shapePossible seizures

Clinical phenotype terms (27)— hover any for plain English
AnophthalmiaHP:0000528Abnormal nostril morphologyHP:0005288Underdeveloped supraorbital ridgesHP:0009891Large faceHP:0100729
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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cerebrooculonasal syndrome.

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No actively recruiting trials found for Cerebrooculonasal syndrome at this time.

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No specialists are currently listed for Cerebrooculonasal 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 Cerebrooculonasal syndrome.

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Community

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Latest news about Cerebrooculonasal syndrome

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Caregiver Resources

NORD Caregiver Resources

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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 brain abnormalities does my child have, and how will they affect development?,Should we do genetic testing, and what type would be most helpful?,What therapies should we start right away, and how often should they happen?,Are there any other health problems we should watch for as my child grows?,What does my child's vision situation mean for their daily life and schooling?,Is there a risk of this condition occurring again in future pregnancies?,Are there any specialists or centers with experience in this specific syndrome?

Common questions about Cerebrooculonasal syndrome

What is Cerebrooculonasal syndrome?

Cerebrooculonasal syndrome is a very rare condition present from birth that affects the brain, eyes, and nose together. It is sometimes called CEONS for short. Babies born with this syndrome typically have a combination of brain abnormalities, unusual eye development, and structural differences in the nose and face. The brain changes can include problems with how the brain formed before birth, which may lead to intellectual disability and developmental delays. Eye problems are a central feature and can range from very small or absent eyes (microphthalmia or anophthalmia) to other structural ey

How is Cerebrooculonasal syndrome inherited?

Cerebrooculonasal syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cerebrooculonasal syndrome typically begin?

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