Radial deficiency-tibial hypoplasia syndrome

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Overview

Radial deficiency-tibial hypoplasia syndrome is an extremely rare genetic condition that affects the development of certain bones in the arms and legs. The condition is sometimes also called radial ray deficiency with tibial hypoplasia. In this syndrome, the radius bone (one of the two bones in the forearm, on the thumb side) is underdeveloped or absent, and the tibia (the larger shinbone in the lower leg) is also underdeveloped (hypoplastic) or missing. These bone abnormalities are typically present at birth and can affect one or both sides of the body. People with this condition may have shortened or bowed forearms, absent or small thumbs, and problems with the hands and wrists. In the legs, the underdeveloped tibia can lead to leg length differences, bowing of the lower legs, and difficulty walking. Some individuals may also have abnormalities of the feet. The severity of the condition varies from person to person, even within the same family. There is currently no cure for this syndrome. Treatment focuses on managing symptoms and improving function. Orthopedic surgery may be needed to correct bone deformities, stabilize joints, or address leg length differences. Physical and occupational therapy can help improve mobility and daily functioning. Prosthetic devices or adaptive equipment may also be recommended. A team of specialists typically works together to create a personalized care plan for each patient.

Key symptoms:

Absent or underdeveloped radius bone in the forearmUnderdeveloped or absent tibia (shinbone)Shortened forearmsAbsent or small thumbsBowed lower legsLeg length differencesWrist abnormalities or instabilityHand and finger abnormalitiesFoot deformitiesDifficulty walking or abnormal gaitLimited range of motion in affected limbsJoint instability

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Radial deficiency-tibial hypoplasia syndrome.

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No actively recruiting trials found for Radial deficiency-tibial hypoplasia syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Radial deficiency-tibial hypoplasia syndrome community →

No specialists are currently listed for Radial deficiency-tibial hypoplasia 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 Radial deficiency-tibial hypoplasia syndrome.

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Community

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Latest news about Radial deficiency-tibial hypoplasia 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.How severe are my child's bone abnormalities, and which limbs are affected?,Will my child need surgery, and if so, what procedures are recommended and when?,What physical and occupational therapy should we start, and how often?,Are there adaptive devices or braces that could help my child right now?,Should we pursue genetic testing, and what would the results mean for our family?,What is the long-term outlook for my child's mobility and independence?,Are there other specialists we should be seeing as part of our care team?

Common questions about Radial deficiency-tibial hypoplasia syndrome

What is Radial deficiency-tibial hypoplasia syndrome?

Radial deficiency-tibial hypoplasia syndrome is an extremely rare genetic condition that affects the development of certain bones in the arms and legs. The condition is sometimes also called radial ray deficiency with tibial hypoplasia. In this syndrome, the radius bone (one of the two bones in the forearm, on the thumb side) is underdeveloped or absent, and the tibia (the larger shinbone in the lower leg) is also underdeveloped (hypoplastic) or missing. These bone abnormalities are typically present at birth and can affect one or both sides of the body. People with this condition may have sh

How is Radial deficiency-tibial hypoplasia syndrome inherited?

Radial deficiency-tibial hypoplasia syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Radial deficiency-tibial hypoplasia syndrome typically begin?

Typical onset of Radial deficiency-tibial hypoplasia syndrome is neonatal. Age of onset can vary across affected individuals.