Acrootoocular syndrome

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ORPHA:2980OMIM:264475Q87.0
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Overview

Acrootoocular syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the hands and feet (acro-), ears (oto-), and eyes (ocular). This syndrome is present from birth and involves a combination of birth defects. People with this condition may have malformations of the fingers and toes, such as short digits or fused fingers, along with abnormalities of the outer ear and hearing problems. Eye abnormalities can also occur, including small eyes or other structural differences that may affect vision. Because this syndrome is so rare, with only a handful of cases described in the medical literature, our understanding of the full range of symptoms and the best approaches to care is still limited. The condition falls under the broader category of multiple congenital anomaly syndromes, classified under ICD-10 code Q87.0. There is currently no cure for acrootoocular syndrome. Treatment focuses on managing individual symptoms and may involve surgery to correct structural abnormalities of the hands, feet, ears, or eyes. Hearing aids or other assistive devices may be recommended if hearing loss is present. A team of specialists typically works together to address the various aspects of this condition. Early intervention and supportive therapies can help improve quality of life for affected individuals.

Also known as:

Key symptoms:

Short fingers or toesFused fingers or toesAbnormally shaped earsHearing lossSmall eyes or other eye abnormalitiesVision problemsFacial differencesFoot deformitiesHand malformationsPossible developmental delays

Clinical phenotype terms (42)— hover any for plain English
Abnormality of facial musculatureHP:0000301Abnormal earlobe morphologyHP:0000363PseudopapilledemaHP:0000538AnodontiaHP:0000674Grayish enamelHP:0000683Small thenar eminenceHP:0001245
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 Acrootoocular syndrome.

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

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No specialists are currently listed for Acrootoocular 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 Acrootoocular syndrome.

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Community

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Latest news about Acrootoocular 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

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 abnormalities does my child have, and how severe are they?,Is genetic testing recommended for our family, and what might it tell us?,What surgeries or treatments are recommended, and when should they happen?,How often should my child's hearing and vision be checked?,What early intervention services should we start right away?,What is the chance of having another child with this condition?,Are there any specialists or centers with experience in this syndrome?

Common questions about Acrootoocular syndrome

What is Acrootoocular syndrome?

Acrootoocular syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the hands and feet (acro-), ears (oto-), and eyes (ocular). This syndrome is present from birth and involves a combination of birth defects. People with this condition may have malformations of the fingers and toes, such as short digits or fused fingers, along with abnormalities of the outer ear and hearing problems. Eye abnormalities can also occur, including small eyes or other structural differences that may affect vision. Because this syndrome is so rare, with only a handful

How is Acrootoocular syndrome inherited?

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

At what age does Acrootoocular syndrome typically begin?

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