Isolated absence of both forearm and hand

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Overview

Isolated absence of both forearm and hand is a rare congenital limb deficiency present at birth, in which a baby is born missing both the forearm (the part between the elbow and wrist) and the hand on one or both arms. This condition is also known as bilateral transverse upper limb deficiency at the forearm level, or bilateral congenital below-elbow amputation. The term 'isolated' means it occurs without other major birth defects or syndromes. This condition happens during early pregnancy when the limbs are forming. In most cases, the exact cause is unknown. It may occur randomly (sporadically) without a clear genetic explanation, though in rare instances environmental factors during pregnancy or genetic changes may play a role. The upper arms and elbows are typically normal, and the child's overall health and intelligence are usually unaffected. Treatment focuses on helping the child achieve the best possible function and independence. This often includes prosthetic devices (artificial limbs), occupational therapy, and adaptive equipment. Early intervention programs can help children learn to perform daily tasks. Advances in prosthetic technology, including myoelectric (muscle-controlled) prostheses, have greatly improved outcomes. Surgical options are rarely needed but may be considered to improve the residual limb for better prosthetic fitting. With proper support, most individuals lead full, active, and independent lives.

Also known as:

Key symptoms:

Absence of both forearms and hands from birthShortened arms ending at or near the elbowDifficulty grasping and holding objectsChallenges with self-care tasks like dressing and eatingDifficulty with fine motor activities like writingPossible differences in balance due to altered arm lengthResidual limb may have small finger-like buds (nubbins) at the end

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Isolated absence of both forearm and hand.

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No actively recruiting trials found for Isolated absence of both forearm and hand at this time.

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No specialists are currently listed for Isolated absence of both forearm and hand.

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 Isolated absence of both forearm and hand.

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Community

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Caregiver Resources

NORD Caregiver Resources

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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 this condition truly isolated, or should we test for any underlying genetic syndromes?,At what age should my child start using prosthetic limbs, and what type is best to start with?,How often will prosthetics need to be replaced as my child grows?,What occupational therapy services are available to help my child become more independent?,Are there specialized limb deficiency clinics or multidisciplinary teams we should be referred to?,What is the chance of this condition occurring again in future pregnancies?,Are there support groups or organizations for families of children with limb differences?

Common questions about Isolated absence of both forearm and hand

What is Isolated absence of both forearm and hand?

Isolated absence of both forearm and hand is a rare congenital limb deficiency present at birth, in which a baby is born missing both the forearm (the part between the elbow and wrist) and the hand on one or both arms. This condition is also known as bilateral transverse upper limb deficiency at the forearm level, or bilateral congenital below-elbow amputation. The term 'isolated' means it occurs without other major birth defects or syndromes. This condition happens during early pregnancy when the limbs are forming. In most cases, the exact cause is unknown. It may occur randomly (sporadicall

How is Isolated absence of both forearm and hand inherited?

Isolated absence of both forearm and hand follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Isolated absence of both forearm and hand typically begin?

Typical onset of Isolated absence of both forearm and hand is neonatal. Age of onset can vary across affected individuals.