Radioulnar synostosis-developmental delay-hypotonia syndrome

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ORPHA:3270OMIM:266255Q87.8
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Overview

Radioulnar synostosis-developmental delay-hypotonia syndrome is a very rare genetic condition that affects multiple parts of the body. The hallmark feature is radioulnar synostosis, which means the two bones in the forearm (the radius and ulna) are fused together. This fusion limits the ability to rotate the forearm, making it difficult to turn the palm up or down. In addition to the bone abnormality, children with this syndrome typically have developmental delay, meaning they are slower to reach milestones like sitting, walking, and talking compared to other children their age. Hypotonia, or low muscle tone, is another key feature, which can make babies appear "floppy" and contribute to feeding difficulties and delayed motor skills. Other features that may be seen include intellectual disability of varying degrees, distinctive facial features, and sometimes additional skeletal abnormalities. Some affected individuals may also have behavioral challenges. The severity of symptoms can vary from person to person, even within the same family. There is currently no cure for this condition, and treatment focuses on managing individual symptoms through therapies such as physical therapy, occupational therapy, speech therapy, and sometimes surgical intervention for the forearm fusion if it significantly limits function. Early intervention services are important to help children reach their full developmental potential.

Also known as:

Key symptoms:

Fusion of the forearm bones (cannot rotate the forearm normally)Low muscle tone (floppiness)Delayed development of motor skills like sitting and walkingDelayed speech and language developmentIntellectual disabilityFeeding difficulties in infancyDistinctive facial featuresPoor coordinationDifficulty with fine motor tasks like writing or buttoning clothesLimited ability to turn the palm up or downBehavioral challengesShort stature in some cases

Clinical phenotype terms (20)— hover any for plain English
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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Radioulnar synostosis-developmental delay-hypotonia syndrome.

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No actively recruiting trials found for Radioulnar synostosis-developmental delay-hypotonia syndrome at this time.

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No specialists are currently listed for Radioulnar synostosis-developmental delay-hypotonia 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 Radioulnar synostosis-developmental delay-hypotonia syndrome.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the specific genetic cause of my child's condition, and should we pursue further genetic testing?,What therapies are most important to start right away?,Is surgery an option for the forearm fusion, and what are the risks and benefits?,What developmental milestones should I watch for, and when should I be concerned?,Are there any other medical issues we should screen for?,What educational supports and services should we request for my child?,What is the chance that future children could also be affected?

Common questions about Radioulnar synostosis-developmental delay-hypotonia syndrome

What is Radioulnar synostosis-developmental delay-hypotonia syndrome?

Radioulnar synostosis-developmental delay-hypotonia syndrome is a very rare genetic condition that affects multiple parts of the body. The hallmark feature is radioulnar synostosis, which means the two bones in the forearm (the radius and ulna) are fused together. This fusion limits the ability to rotate the forearm, making it difficult to turn the palm up or down. In addition to the bone abnormality, children with this syndrome typically have developmental delay, meaning they are slower to reach milestones like sitting, walking, and talking compared to other children their age. Hypotonia, or

At what age does Radioulnar synostosis-developmental delay-hypotonia syndrome typically begin?

Typical onset of Radioulnar synostosis-developmental delay-hypotonia syndrome is neonatal. Age of onset can vary across affected individuals.