OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral

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ORPHA:295112
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Overview

Congenital absence or hypoplasia (underdevelopment) of the thumb on both hands is a rare condition present at birth where a baby is born with both thumbs either missing entirely or significantly smaller and less developed than normal. This condition is sometimes called bilateral thumb aplasia or bilateral thumb hypoplasia. Because the entry is marked as 'OBSOLETE' in Orphanet, it means this specific classification is no longer actively used and has likely been reclassified under broader categories of limb malformations or radial ray deficiencies. The thumbs play a critical role in hand function, especially for gripping and pinching. When both thumbs are affected, children may have significant difficulty with everyday tasks such as grasping objects, writing, buttoning clothes, and feeding themselves. The severity can range from a slightly small but functional thumb to a completely absent thumb. This condition can occur as an isolated finding or as part of a broader syndrome, such as Holt-Oram syndrome, Fanconi anemia, thrombocytopenia-absent radius (TAR) syndrome, or VACTERL association. Treatment depends on the severity and may include surgical reconstruction of the thumb, pollicization (surgically converting the index finger into a thumb), or use of adaptive devices. Early evaluation by a hand surgeon and geneticist is important to determine the underlying cause and plan appropriate treatment.

Also known as:

Key symptoms:

Missing thumbs on both hands at birthVery small or underdeveloped thumbs on both handsDifficulty gripping or holding objectsWeak pinch strengthTrouble with fine motor tasks like writing or buttoningShortened or absent forearm bone (radius) in some casesPossible abnormalities of the wrist bonesFloppy or unstable thumb joint if thumb is present but underdevelopedDifficulty feeding independently as a toddlerPossible associated heart defects depending on underlying syndromePossible low blood counts if associated with Fanconi anemia

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: Congenital absence/hypoplasia of thumb, bilateral.

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No actively recruiting trials found for OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral at this time.

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No specialists are currently listed for OBSOLETE: Congenital absence/hypoplasia of thumb, 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: Congenital absence/hypoplasia of thumb, bilateral.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Is my child's thumb condition isolated or part of a larger syndrome?,What genetic tests should be done to find the underlying cause?,Is pollicization surgery recommended, and at what age should it be done?,What hand function can we expect after surgery and therapy?,Are there any associated health problems we need to screen for, such as heart defects or blood disorders?,Should other family members be tested or evaluated?,What adaptive tools or therapies can help my child in the meantime?

Common questions about OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral

What is OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral?

Congenital absence or hypoplasia (underdevelopment) of the thumb on both hands is a rare condition present at birth where a baby is born with both thumbs either missing entirely or significantly smaller and less developed than normal. This condition is sometimes called bilateral thumb aplasia or bilateral thumb hypoplasia. Because the entry is marked as 'OBSOLETE' in Orphanet, it means this specific classification is no longer actively used and has likely been reclassified under broader categories of limb malformations or radial ray deficiencies. The thumbs play a critical role in hand functi

At what age does OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral typically begin?

Typical onset of OBSOLETE: Congenital absence/hypoplasia of thumb, bilateral is neonatal. Age of onset can vary across affected individuals.