Aphalangy-syndactyly-microcephaly syndrome

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ORPHA:1113OMIM:600384Q87.2
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Overview

Aphalangy-syndactyly-microcephaly syndrome is an extremely rare genetic condition that affects the development of the hands, feet, and brain. The name describes its three main features: aphalangy means the absence of one or more finger or toe bones (phalanges), syndactyly means webbing or fusion of the fingers or toes, and microcephaly means a smaller-than-expected head size, which often reflects reduced brain growth. Children born with this condition typically have noticeable differences in their hands and feet at birth, along with a head circumference that is smaller than average for their age. Because the brain may be underdeveloped, some affected individuals may experience intellectual disability or developmental delays. Additional features that have been reported in some cases include unusual facial features and other skeletal abnormalities. Because this syndrome is so rare, with only a handful of cases described in the medical literature, the full range of symptoms and the best approaches to treatment are not yet well understood. There is currently no cure, and treatment focuses on managing individual symptoms through surgery, therapy, and supportive care. A team of specialists is usually needed to address the different aspects of this condition.

Key symptoms:

Missing finger or toe bones (absent phalanges)Webbed or fused fingers or toesSmaller than normal head sizeIntellectual disability or learning difficultiesDevelopmental delaysUnusual facial featuresShort or absent nailsSkeletal abnormalities in the hands and feetPossible speech delays

Clinical phenotype terms (13)— hover any for plain English
Hypoplastic fingernailHP:0001804Aplasia/Hypoplasia of the distal phalanges of the toesHP:0010185Absent toenailHP:0001802Postaxial foot polydactylyHP:0001830Split footHP:0001839
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 Aphalangy-syndactyly-microcephaly syndrome.

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No actively recruiting trials found for Aphalangy-syndactyly-microcephaly syndrome at this time.

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No specialists are currently listed for Aphalangy-syndactyly-microcephaly syndrome.

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 Aphalangy-syndactyly-microcephaly syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the severity of my child's hand and foot differences, and what surgical options are available?,How will microcephaly affect my child's development, and what therapies should we start?,Should we pursue genetic testing, and what can it tell us about the cause?,What early intervention services should my child receive?,Are there risks for future children in our family having the same condition?,How often should we schedule follow-up appointments with specialists?,Are there any research studies or registries we can participate in?

Common questions about Aphalangy-syndactyly-microcephaly syndrome

What is Aphalangy-syndactyly-microcephaly syndrome?

Aphalangy-syndactyly-microcephaly syndrome is an extremely rare genetic condition that affects the development of the hands, feet, and brain. The name describes its three main features: aphalangy means the absence of one or more finger or toe bones (phalanges), syndactyly means webbing or fusion of the fingers or toes, and microcephaly means a smaller-than-expected head size, which often reflects reduced brain growth. Children born with this condition typically have noticeable differences in their hands and feet at birth, along with a head circumference that is smaller than average for their a

How is Aphalangy-syndactyly-microcephaly syndrome inherited?

Aphalangy-syndactyly-microcephaly syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Aphalangy-syndactyly-microcephaly syndrome typically begin?

Typical onset of Aphalangy-syndactyly-microcephaly syndrome is neonatal. Age of onset can vary across affected individuals.