Hydrocephaly-tall stature-joint laxity syndrome

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ORPHA:2181OMIM:236660Q87.8
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8Treatment centers

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Overview

Hydrocephaly-tall stature-joint laxity syndrome is a very rare condition that affects multiple parts of the body. It is sometimes referred to by its three main features: fluid buildup in the brain (hydrocephalus), unusually tall height, and overly flexible joints. Hydrocephalus happens when cerebrospinal fluid — the liquid that cushions the brain and spinal cord — builds up inside the brain's cavities, putting pressure on brain tissue. This can affect brain development and function. The tall stature means affected individuals tend to grow significantly taller than expected for their age and family background. Joint laxity means the joints are looser and more flexible than normal, which can cause instability, pain, or an increased risk of dislocations. This syndrome is extremely rare, and only a small number of cases have been reported in the medical literature. Because so few cases are known, doctors are still learning about the full range of symptoms and how the condition progresses over time. The cause at the genetic level is not yet fully understood. Treatment focuses on managing individual symptoms — for example, surgery or a shunt device may be used to relieve pressure from hydrocephalus, while physical therapy can help with joint problems. There is currently no cure for this syndrome.

Also known as:

Key symptoms:

Fluid buildup in the brain (hydrocephalus)Unusually tall height for ageOverly flexible or loose jointsLarge head size (macrocephaly)Developmental delaysIntellectual disability in some casesIncreased risk of joint dislocationsPossible vision problems due to brain pressureCoordination difficulties

Clinical phenotype terms (15)— hover any for plain English
Disproportionate tall statureHP:0001519HemiplegiaHP:0002301Shoulder dislocationHP:0003834
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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hydrocephaly-tall stature-joint laxity syndrome.

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No actively recruiting trials found for Hydrocephaly-tall stature-joint laxity syndrome at this time.

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No specialists are currently listed for Hydrocephaly-tall stature-joint laxity 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 Hydrocephaly-tall stature-joint laxity syndrome.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What type of treatment do you recommend for my child's hydrocephalus, and what are the risks and benefits?,How often should we have brain imaging done to monitor the hydrocephalus?,What signs should prompt me to go to the emergency room immediately?,Should we pursue genetic testing, and could this condition be inherited by other family members?,What therapies — such as physical or occupational therapy — would help with joint laxity?,Are there any specialists or centers with experience in this specific syndrome that you would recommend?,What support services are available for school and development?

Common questions about Hydrocephaly-tall stature-joint laxity syndrome

What is Hydrocephaly-tall stature-joint laxity syndrome?

Hydrocephaly-tall stature-joint laxity syndrome is a very rare condition that affects multiple parts of the body. It is sometimes referred to by its three main features: fluid buildup in the brain (hydrocephalus), unusually tall height, and overly flexible joints. Hydrocephalus happens when cerebrospinal fluid — the liquid that cushions the brain and spinal cord — builds up inside the brain's cavities, putting pressure on brain tissue. This can affect brain development and function. The tall stature means affected individuals tend to grow significantly taller than expected for their age and fa

At what age does Hydrocephaly-tall stature-joint laxity syndrome typically begin?

Typical onset of Hydrocephaly-tall stature-joint laxity syndrome is neonatal. Age of onset can vary across affected individuals.