Short stature-webbed neck-heart disease syndrome

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Overview

Short stature-webbed neck-heart disease syndrome is an extremely rare genetic condition that shares some features with Turner syndrome and Noonan syndrome but is considered a distinct disorder. People with this condition are notably shorter than average, have a webbed neck (extra folds of skin stretching from the sides of the neck to the shoulders), and are born with heart defects. The heart problems most commonly involve the valves or the structure of the heart itself, such as pulmonary valve stenosis or other congenital heart malformations. Additional features may include a broad or shield-shaped chest, low-set ears, and subtle differences in facial appearance. Because this syndrome is so rare, the full range of symptoms and their severity can vary from person to person. There is currently no cure for this condition. Treatment focuses on managing individual symptoms, particularly the heart defects, which may require surgical correction or ongoing monitoring by a cardiologist. Growth hormone therapy may be considered to help with short stature, though its effectiveness in this specific syndrome is not well established. Regular follow-up with multiple specialists is important to address the various aspects of the condition and to ensure the best possible quality of life.

Also known as:

Key symptoms:

Short stature or growth delayWebbed neck with extra skin foldsCongenital heart defectsBroad or shield-shaped chestLow-set earsWide-spaced nipplesSwelling of the hands or feet at birthUnusual facial featuresDelayed puberty or hormonal differencesSkeletal abnormalitiesMild learning difficulties in some cases

Clinical phenotype terms (7)— hover any for plain English
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 Short stature-webbed neck-heart disease syndrome.

View clinical trials →

No actively recruiting trials found for Short stature-webbed neck-heart disease syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Short stature-webbed neck-heart disease syndrome community →

No specialists are currently listed for Short stature-webbed neck-heart disease 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 Short stature-webbed neck-heart disease syndrome.

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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 type of heart defect does my child have, and will it need surgery?,Should we consider growth hormone therapy, and what are the potential benefits and risks?,How often should my child have heart check-ups?,Are there any physical activities my child should avoid?,Should we pursue genetic testing to look for a specific cause?,What developmental or learning support services should we look into?,Are there any related conditions we should screen for over time?

Common questions about Short stature-webbed neck-heart disease syndrome

What is Short stature-webbed neck-heart disease syndrome?

Short stature-webbed neck-heart disease syndrome is an extremely rare genetic condition that shares some features with Turner syndrome and Noonan syndrome but is considered a distinct disorder. People with this condition are notably shorter than average, have a webbed neck (extra folds of skin stretching from the sides of the neck to the shoulders), and are born with heart defects. The heart problems most commonly involve the valves or the structure of the heart itself, such as pulmonary valve stenosis or other congenital heart malformations. Additional features may include a broad or shield-s

At what age does Short stature-webbed neck-heart disease syndrome typically begin?

Typical onset of Short stature-webbed neck-heart disease syndrome is neonatal. Age of onset can vary across affected individuals.