Marfanoid syndrome, De Silva type

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ORPHA:2464OMIM:223330Q87.4
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Overview

Marfanoid syndrome, De Silva type (also sometimes referred to as De Silva syndrome) is a very rare genetic condition that shares some physical features with Marfan syndrome — a well-known connective tissue disorder — but is considered a distinct condition. People with this syndrome tend to have a tall, slender body build with unusually long limbs, fingers, and toes, giving them a 'marfanoid' appearance. However, unlike classic Marfan syndrome, De Silva type is also associated with intellectual disability and developmental delays, meaning that children may reach milestones like walking and talking later than expected. The condition affects multiple body systems. In addition to the distinctive body shape, individuals may experience joint looseness (hypermobility), muscle weakness, and facial features that look different from other family members. Learning difficulties and cognitive challenges are a central part of this condition and can range from mild to moderate. Because this condition is extremely rare, the full picture of how it affects people over a lifetime is not completely understood. There is currently no cure. Care focuses on supporting development through therapies such as speech therapy, physical therapy, and educational support, along with regular monitoring of the heart and skeleton. A team of specialists working together gives the best outcomes for affected individuals and their families.

Key symptoms:

Tall, slender body build with long arms and legsUnusually long fingers and toes (arachnodactyly)Intellectual disability or learning difficultiesDelayed developmental milestones (walking, talking later than expected)Loose, flexible joints (joint hypermobility)Muscle weakness or low muscle toneDistinctive facial featuresCurved spine (scoliosis) in some individualsFlat feet or other foot abnormalities

Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Marfanoid syndrome, De Silva type.

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No actively recruiting trials found for Marfanoid syndrome, De Silva type at this time.

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Search ClinicalTrials.gov ↗Join the Marfanoid syndrome, De Silva type community →

No specialists are currently listed for Marfanoid syndrome, De Silva type.

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 Marfanoid syndrome, De Silva type.

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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

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.What genetic tests should we do to confirm this diagnosis, and what might the results tell us?,Does my child need a heart scan (echocardiogram), and how often should this be repeated?,What therapies — physical, speech, or occupational — would benefit my child most right now?,Are there any signs of complications we should watch for at home that would require emergency care?,How can we get an individualized education plan (IEP) set up for school?,Are there any research studies or patient registries we could join to help advance understanding of this condition?,Should other family members be tested or evaluated for this condition?

Common questions about Marfanoid syndrome, De Silva type

What is Marfanoid syndrome, De Silva type?

Marfanoid syndrome, De Silva type (also sometimes referred to as De Silva syndrome) is a very rare genetic condition that shares some physical features with Marfan syndrome — a well-known connective tissue disorder — but is considered a distinct condition. People with this syndrome tend to have a tall, slender body build with unusually long limbs, fingers, and toes, giving them a 'marfanoid' appearance. However, unlike classic Marfan syndrome, De Silva type is also associated with intellectual disability and developmental delays, meaning that children may reach milestones like walking and talk

How is Marfanoid syndrome, De Silva type inherited?

Marfanoid syndrome, De Silva type follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Marfanoid syndrome, De Silva type typically begin?

Typical onset of Marfanoid syndrome, De Silva type is childhood. Age of onset can vary across affected individuals.