OBSOLETE: Humeral agenesis/hypoplasia, bilateral

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ORPHA:295063
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Overview

Bilateral humeral agenesis or hypoplasia is an extremely rare congenital condition in which both upper arm bones (the humerus bones) are either completely absent (agenesis) or significantly underdeveloped (hypoplasia) at birth. This condition affects both arms, meaning the child is born with shortened or missing upper arms on both sides. The hands and forearms may be present but are positioned closer to the shoulders than normal, or in some cases, additional limb abnormalities may also be present. This entry in Orphanet is marked as 'OBSOLETE,' which means it has been retired or reclassified. It may now be categorized under a broader group of limb reduction defects or a more specific syndrome that includes bilateral humeral involvement. Limb reduction defects can occur as isolated findings or as part of a larger genetic syndrome. Because this is an obsolete classification, patients and families who have been given this diagnosis should work closely with a clinical geneticist to determine whether a more specific or updated diagnosis applies. Treatment is generally supportive and may include prosthetics, occupational therapy, and surgical interventions to improve function. The goal of care is to maximize independence and quality of life. Early intervention services for children can be very helpful in developing adaptive skills.

Also known as:

Key symptoms:

Absent or very short upper arm bones on both sidesShortened armsLimited arm movement and reachHands positioned close to the shouldersDifficulty with tasks requiring arm length such as reaching or liftingPossible additional limb differencesReduced grip strength or arm strengthAsymmetry in body proportions

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Humeral agenesis/hypoplasia, bilateral.

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No actively recruiting trials found for OBSOLETE: Humeral agenesis/hypoplasia, bilateral at this time.

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No specialists are currently listed for OBSOLETE: Humeral agenesis/hypoplasia, bilateral.

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 OBSOLETE: Humeral agenesis/hypoplasia, bilateral.

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Community

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Latest news about OBSOLETE: Humeral agenesis/hypoplasia, bilateral

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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.Is there an underlying genetic syndrome causing this condition, and should we pursue genetic testing?,What type of prosthetic options are available for my child at this age?,How often should we schedule occupational and physical therapy?,Are there any associated health problems we should screen for?,Can you refer us to a specialized limb difference clinic?,What early intervention services should we enroll in?,Is there a risk of this condition occurring in future pregnancies?

Common questions about OBSOLETE: Humeral agenesis/hypoplasia, bilateral

What is OBSOLETE: Humeral agenesis/hypoplasia, bilateral?

Bilateral humeral agenesis or hypoplasia is an extremely rare congenital condition in which both upper arm bones (the humerus bones) are either completely absent (agenesis) or significantly underdeveloped (hypoplasia) at birth. This condition affects both arms, meaning the child is born with shortened or missing upper arms on both sides. The hands and forearms may be present but are positioned closer to the shoulders than normal, or in some cases, additional limb abnormalities may also be present. This entry in Orphanet is marked as 'OBSOLETE,' which means it has been retired or reclassified.

At what age does OBSOLETE: Humeral agenesis/hypoplasia, bilateral typically begin?

Typical onset of OBSOLETE: Humeral agenesis/hypoplasia, bilateral is neonatal. Age of onset can vary across affected individuals.