Brachydactyly-short stature-retinitis pigmentosa syndrome

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ORPHA:166035OMIM:250410Q87.8
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Overview

Brachydactyly-short stature-retinitis pigmentosa syndrome is an extremely rare genetic condition that affects multiple body systems at the same time. The name describes its three main features: brachydactyly (unusually short fingers and toes), short stature (being significantly shorter than average), and retinitis pigmentosa (a progressive eye disease that damages the retina and leads to vision loss over time). People with this syndrome are typically born with noticeably short digits and may have delayed growth throughout childhood. The eye problems usually begin with difficulty seeing in dim light (night blindness) and gradually progress to loss of side vision, sometimes leading to significant visual impairment. Because this condition is so rare, with only a handful of cases described in the medical literature, the full range of symptoms and the best approaches to treatment are not yet well understood. There is currently no cure for this syndrome. Treatment focuses on managing each symptom individually — for example, using low-vision aids for eye problems, monitoring growth, and providing orthopedic support if needed. Regular follow-up with multiple specialists is important to address the different aspects of this condition as they develop over time.

Key symptoms:

Short fingers and toesShort stature or growth delayNight blindnessProgressive loss of side (peripheral) visionGradual vision lossDifficulty seeing in low lightShortened hand bones visible on X-rayDelayed growth during childhoodPossible narrowing of visual field (tunnel vision)

Clinical phenotype terms (46)— hover any for plain English
Retinal degenerationHP:0000546
Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Brachydactyly-short stature-retinitis pigmentosa syndrome.

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No actively recruiting trials found for Brachydactyly-short stature-retinitis pigmentosa syndrome at this time.

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No specialists are currently listed for Brachydactyly-short stature-retinitis pigmentosa 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 Brachydactyly-short stature-retinitis pigmentosa syndrome.

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Community

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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 is the expected rate of vision loss for my child, and how will it be monitored?,Are there any treatments or supplements that could slow down the retinitis pigmentosa?,Should my child be evaluated for growth hormone deficiency?,What genetic testing is available, and should other family members be tested?,What school accommodations should we request for vision and physical differences?,Are there any clinical trials my child might be eligible for?,When should we start low-vision rehabilitation services?

Common questions about Brachydactyly-short stature-retinitis pigmentosa syndrome

What is Brachydactyly-short stature-retinitis pigmentosa syndrome?

Brachydactyly-short stature-retinitis pigmentosa syndrome is an extremely rare genetic condition that affects multiple body systems at the same time. The name describes its three main features: brachydactyly (unusually short fingers and toes), short stature (being significantly shorter than average), and retinitis pigmentosa (a progressive eye disease that damages the retina and leads to vision loss over time). People with this syndrome are typically born with noticeably short digits and may have delayed growth throughout childhood. The eye problems usually begin with difficulty seeing in dim

How is Brachydactyly-short stature-retinitis pigmentosa syndrome inherited?

Brachydactyly-short stature-retinitis pigmentosa syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Brachydactyly-short stature-retinitis pigmentosa syndrome typically begin?

Typical onset of Brachydactyly-short stature-retinitis pigmentosa syndrome is childhood. Age of onset can vary across affected individuals.