Isolated acheiria

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:294983Q71.3
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Isolated acheiria is a rare congenital limb deficiency in which a baby is born without one or both hands. The term 'acheiria' comes from Greek and literally means 'without hand.' In this condition, the hand is completely absent at the wrist level, while the rest of the arm (forearm, upper arm, and shoulder) is typically formed normally. The word 'isolated' means that this condition occurs on its own, without other birth defects or as part of a larger syndrome. This condition is present at birth and is usually noticed immediately during the newborn examination or on prenatal ultrasound. It can affect one side (unilateral) or both sides (bilateral), though one-sided involvement is more common. The exact cause of isolated acheiria is not fully understood in most cases. It is thought to result from a disruption in blood flow or other developmental events during early pregnancy that interfere with normal limb formation, rather than being inherited from parents. There is no cure that can restore the missing hand, but treatment focuses on helping the child function as independently as possible. This includes prosthetic devices, occupational therapy, and adaptive techniques. Many children with acheiria adapt remarkably well and lead full, active lives. Early intervention with therapy and prosthetics can help children develop fine motor skills and confidence. Surgical options may sometimes be considered to improve the shape of the limb stump for better prosthetic fitting.

Also known as:

Key symptoms:

Complete absence of one or both hands at birthArm ends at the wrist levelNormal formation of the forearm, upper arm, and shoulderDifficulty grasping or holding objectsChallenges with tasks requiring two handsPossible differences in arm length if growth plates are affectedNo other associated birth defects in the isolated form

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 acheiria.

View clinical trials →

No actively recruiting trials found for Isolated acheiria at this time.

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

Search ClinicalTrials.gov ↗Join the Isolated acheiria community →

No specialists are currently listed for Isolated acheiria.

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 acheiria.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Isolated acheiriaForum →

No community posts yet. Be the first to share your experience with Isolated acheiria.

Start the conversation →

Latest news about Isolated acheiria

No recent news articles for Isolated acheiria.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 my child's condition truly isolated, or should we test for any associated syndromes?,At what age should we start fitting a prosthetic device?,What type of prosthetic would be best for my child at this stage?,How often will my child need occupational therapy, and what goals should we set?,Are there any surgical options that could improve function or prosthetic fit?,What adaptive tools or resources are available for school and daily activities?,Is there any chance this condition could affect future children in our family?

Common questions about Isolated acheiria

What is Isolated acheiria?

Isolated acheiria is a rare congenital limb deficiency in which a baby is born without one or both hands. The term 'acheiria' comes from Greek and literally means 'without hand.' In this condition, the hand is completely absent at the wrist level, while the rest of the arm (forearm, upper arm, and shoulder) is typically formed normally. The word 'isolated' means that this condition occurs on its own, without other birth defects or as part of a larger syndrome. This condition is present at birth and is usually noticed immediately during the newborn examination or on prenatal ultrasound. It can

How is Isolated acheiria inherited?

Isolated acheiria follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Isolated acheiria typically begin?

Typical onset of Isolated acheiria is neonatal. Age of onset can vary across affected individuals.