Mesoaxial synostotic syndactyly with phalangeal reduction

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ORPHA:157801OMIM:609432Q70.0Q70.2
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Overview

Mesoaxial synostotic syndactyly with phalangeal reduction is an extremely rare condition that affects the development of the hands and sometimes the feet before birth. In this condition, certain fingers (typically the middle fingers or central digits) are fused together — a feature called syndactyly. The term 'synostotic' means that the bones themselves are fused, not just the skin and soft tissue. Additionally, some of the small bones in the fingers (called phalanges) may be missing or reduced in number, which is referred to as phalangeal reduction. The word 'mesoaxial' refers to the central part of the hand being primarily affected, as opposed to the thumb side or pinky side. This condition is present at birth and is usually noticed immediately by the appearance of the hands. The severity can vary, with some individuals having mild fusion and others having more significant bone involvement. Because this is such a rare condition, the treatment approach is highly individualized. Surgery may be considered to separate fused fingers and improve hand function. Occupational therapy can help children learn to use their hands effectively. There is no cure that addresses the underlying cause, but surgical and therapeutic interventions can significantly improve quality of life and hand function. Genetic counseling is recommended for affected families to understand the chance of the condition occurring in future children.

Also known as:

Key symptoms:

Fingers fused together (syndactyly), especially the central fingersBones of the fingers joined together (bony fusion)Missing or shortened finger bonesReduced number of finger segmentsAbnormal appearance of the handsPossible reduced grip strengthDifficulty with fine motor tasks like grasping small objectsPossible involvement of the toes in some cases

Clinical phenotype terms (11)— hover any for plain English
2-3 toe syndactylyHP:0004691Aplasia/Hypoplasia of the halluxHP:0008362Metacarpal synostosisHP:0009701Aplasia/Hypoplasia of the middle phalanges of the handHP:0009843Short halluxHP:0010109
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Mesoaxial synostotic syndactyly with phalangeal reduction.

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No actively recruiting trials found for Mesoaxial synostotic syndactyly with phalangeal reduction at this time.

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No specialists are currently listed for Mesoaxial synostotic syndactyly with phalangeal reduction.

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 Mesoaxial synostotic syndactyly with phalangeal reduction.

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is the bone fusion in my child's hands, and which fingers are most affected?,Is surgery recommended, and if so, at what age should it be done?,What improvements in hand function can we realistically expect after surgery?,Should we start occupational therapy now, and how often?,Is genetic testing recommended for our family, and what would the results mean for future pregnancies?,Are there any other parts of the body we should check for related problems?,Can you refer us to a hand surgeon who has experience with complex syndactyly?

Common questions about Mesoaxial synostotic syndactyly with phalangeal reduction

What is Mesoaxial synostotic syndactyly with phalangeal reduction?

Mesoaxial synostotic syndactyly with phalangeal reduction is an extremely rare condition that affects the development of the hands and sometimes the feet before birth. In this condition, certain fingers (typically the middle fingers or central digits) are fused together — a feature called syndactyly. The term 'synostotic' means that the bones themselves are fused, not just the skin and soft tissue. Additionally, some of the small bones in the fingers (called phalanges) may be missing or reduced in number, which is referred to as phalangeal reduction. The word 'mesoaxial' refers to the central

How is Mesoaxial synostotic syndactyly with phalangeal reduction inherited?

Mesoaxial synostotic syndactyly with phalangeal reduction follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Mesoaxial synostotic syndactyly with phalangeal reduction typically begin?

Typical onset of Mesoaxial synostotic syndactyly with phalangeal reduction is neonatal. Age of onset can vary across affected individuals.