OBSOLETE: Femoral agenesis/hypoplasia, bilateral

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ORPHA:295067
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Overview

Bilateral femoral agenesis or hypoplasia is a rare congenital condition in which both thigh bones (femurs) are either completely absent (agenesis) or significantly underdeveloped (hypoplasia). This condition is present at birth and affects the structure and function of both legs. The term 'OBSOLETE' in the Orphanet classification means this specific entry has been retired or merged into a broader or updated category, but the condition itself still exists and is recognized under related classifications such as proximal femoral focal deficiency (PFFD) or femoral hypoplasia. Babies born with this condition typically have noticeably shortened or absent thighs, which affects their ability to stand, walk, and move independently. The hips and knees may also be affected, and other skeletal abnormalities can sometimes be present. The severity varies widely — some children have mildly short femurs while others have near-complete absence of the bone on both sides. Treatment is primarily supportive and surgical. Options may include limb-lengthening procedures, prosthetic devices, reconstructive surgery, and physical therapy. The goal of treatment is to maximize mobility and independence. There is no cure for the underlying skeletal deficiency, but many individuals lead active and fulfilling lives with appropriate orthopedic care and adaptive equipment. Early intervention with a multidisciplinary team is important for the best outcomes.

Also known as:

Key symptoms:

Shortened or absent thigh bones on both sidesNoticeably short legs at birthDifficulty standing or walkingHip joint abnormalitiesKnee joint instability or stiffnessUneven leg lengths compared to the trunkLimited range of motion in the hips or kneesAbnormal gait or inability to walk without assistancePossible associated foot or ankle abnormalitiesPossible pelvic abnormalities

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: Femoral agenesis/hypoplasia, bilateral.

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

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

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Community

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Latest news about OBSOLETE: Femoral 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.How severe is my child's femoral deficiency, and what does that mean for their mobility?,What surgical options are available, and when is the best time to consider them?,Should we pursue genetic testing to look for an underlying cause or syndrome?,What type of prosthetic devices would be most appropriate at this stage?,How often will my child need orthopedic follow-up appointments?,What physical therapy program do you recommend?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about OBSOLETE: Femoral agenesis/hypoplasia, bilateral

What is OBSOLETE: Femoral agenesis/hypoplasia, bilateral?

Bilateral femoral agenesis or hypoplasia is a rare congenital condition in which both thigh bones (femurs) are either completely absent (agenesis) or significantly underdeveloped (hypoplasia). This condition is present at birth and affects the structure and function of both legs. The term 'OBSOLETE' in the Orphanet classification means this specific entry has been retired or merged into a broader or updated category, but the condition itself still exists and is recognized under related classifications such as proximal femoral focal deficiency (PFFD) or femoral hypoplasia. Babies born with thi

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

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