OBSOLETE: Split hand, unilateral

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ORPHA:295120
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8Treatment centers

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Overview

Split hand, unilateral (also called unilateral ectrodactyly or lobster claw hand on one side) is a rare congenital limb malformation in which one hand has missing central fingers, creating a deep cleft or split in the middle of the hand. The condition affects only one hand, unlike bilateral forms that involve both hands or hands and feet. The hand may appear to have a V-shaped gap where the middle fingers should be, and the remaining fingers may be fused or abnormally shaped. This condition is present at birth and is typically an isolated finding, meaning it occurs on its own without other major health problems in most cases. The severity can vary widely. Some individuals have only a mild cleft with minor finger differences, while others may have significant absence of fingers and changes in hand structure. Despite the visible difference, many people with unilateral split hand retain good hand function, especially with early therapy and, in some cases, surgical reconstruction. Note: This entry is classified as 'OBSOLETE' in the Orphanet database, meaning it may have been reclassified or merged into a broader category of split hand/foot malformations (ectrodactyly). Patients and families should discuss the most current classification with their geneticist. Treatment is primarily supportive and may include occupational therapy to maximize hand function, prosthetic devices, and reconstructive surgery depending on the severity. Genetic counseling is recommended to understand recurrence risks.

Also known as:

Key symptoms:

Missing central fingers on one handDeep cleft or split in the middle of the handV-shaped or lobster claw appearance of the handFused fingers on the affected handShortened or underdeveloped fingersReduced grip strength in the affected handAbnormally shaped remaining fingersDifficulty with fine motor tasks using the affected hand

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: Split hand, unilateral.

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No actively recruiting trials found for OBSOLETE: Split hand, unilateral at this time.

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No specialists are currently listed for OBSOLETE: Split hand, unilateral.

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: Split hand, unilateral.

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Community

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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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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 split hand isolated, or could it be part of a broader genetic syndrome?,Should we pursue genetic testing, and what would the results mean for our family?,Would surgery improve hand function or appearance, and when is the best time for it?,What kind of occupational therapy is recommended, and how often?,What is the chance that future children could also be affected?,Are there adaptive devices that could help with daily tasks?,Can you refer us to a support group for families of children with limb differences?

Common questions about OBSOLETE: Split hand, unilateral

What is OBSOLETE: Split hand, unilateral?

Split hand, unilateral (also called unilateral ectrodactyly or lobster claw hand on one side) is a rare congenital limb malformation in which one hand has missing central fingers, creating a deep cleft or split in the middle of the hand. The condition affects only one hand, unlike bilateral forms that involve both hands or hands and feet. The hand may appear to have a V-shaped gap where the middle fingers should be, and the remaining fingers may be fused or abnormally shaped. This condition is present at birth and is typically an isolated finding, meaning it occurs on its own without other maj

At what age does OBSOLETE: Split hand, unilateral typically begin?

Typical onset of OBSOLETE: Split hand, unilateral is neonatal. Age of onset can vary across affected individuals.