Osteosclerosis-developmental delay-craniosynostosis syndrome

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Overview

Osteosclerosis-developmental delay-craniosynostosis syndrome is an extremely rare genetic condition that affects bone development, brain development, and skull formation. The name describes its three main features: osteosclerosis (abnormally dense and hardened bones), developmental delay (slower than expected progress in learning, movement, or speech), and craniosynostosis (premature fusion of the skull bones in infants, which can restrict normal brain growth). In this condition, the bones throughout the body become denser than normal, which can be seen on X-rays. The early closure of skull sutures can lead to an abnormally shaped head and may increase pressure inside the skull if not treated. Children with this syndrome typically show delays in reaching developmental milestones such as sitting, walking, and talking. Some individuals may also have distinctive facial features and other skeletal abnormalities. Because this syndrome is so rare, there is no specific cure. Treatment focuses on managing individual symptoms. Surgery may be needed to correct craniosynostosis and relieve pressure on the brain. Early intervention programs including physical therapy, occupational therapy, and speech therapy can help children reach their developmental potential. A team of specialists is usually needed to provide comprehensive care.

Key symptoms:

Abnormally dense or hardened bonesEarly fusion of skull bones (craniosynostosis)Abnormal head shapeDevelopmental delayDelayed speech and language skillsDelayed motor milestones like sitting and walkingIntellectual disabilityUnusual facial featuresSkeletal abnormalitiesPossible increased pressure inside the skullShort stature or growth problems

Clinical phenotype terms (16)— hover any for plain English
Thickened calvariaHP:0002684Increased bone mineral densityHP:0011001Broad jawHP:0012802
Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Osteosclerosis-developmental delay-craniosynostosis syndrome.

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No actively recruiting trials found for Osteosclerosis-developmental delay-craniosynostosis syndrome at this time.

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No specialists are currently listed for Osteosclerosis-developmental delay-craniosynostosis 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 Osteosclerosis-developmental delay-craniosynostosis syndrome.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's craniosynostosis, and will surgery be needed?,What developmental therapies should we start, and how often?,How will you monitor for increased pressure in the skull?,What signs should I watch for at home that would need urgent medical attention?,Is genetic testing available to identify the exact cause of this condition?,What is the expected developmental outlook for my child?,Are there any clinical trials or research studies we could participate in?

Common questions about Osteosclerosis-developmental delay-craniosynostosis syndrome

What is Osteosclerosis-developmental delay-craniosynostosis syndrome?

Osteosclerosis-developmental delay-craniosynostosis syndrome is an extremely rare genetic condition that affects bone development, brain development, and skull formation. The name describes its three main features: osteosclerosis (abnormally dense and hardened bones), developmental delay (slower than expected progress in learning, movement, or speech), and craniosynostosis (premature fusion of the skull bones in infants, which can restrict normal brain growth). In this condition, the bones throughout the body become denser than normal, which can be seen on X-rays. The early closure of skull s

At what age does Osteosclerosis-developmental delay-craniosynostosis syndrome typically begin?

Typical onset of Osteosclerosis-developmental delay-craniosynostosis syndrome is neonatal. Age of onset can vary across affected individuals.