Craniosynostosis-intracranial calcifications syndrome

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ORPHA:52054OMIM:608432Q87.0
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Overview

Craniosynostosis-intracranial calcifications syndrome, also sometimes called Craniosynostosis with intracranial calcifications or CIC syndrome, is a very rare condition that affects the skull and brain. Craniosynostosis means that the bones of the skull fuse together too early, before the brain has finished growing. This can cause the head to have an unusual shape and can put pressure on the growing brain. In this syndrome, there are also calcium deposits that build up inside the brain, which is called intracranial calcification. These calcium deposits can interfere with how the brain works normally. The combination of early skull fusion and calcium deposits in the brain can lead to a range of problems. Children with this condition may have an unusually shaped head, increased pressure inside the skull, developmental delays, intellectual disability, and seizures. Some children may also have vision or hearing problems. The face may look different from typical, and some children have other physical features that are part of the syndrome. Treatment focuses on managing symptoms rather than curing the underlying condition. Surgery is often needed to correct the skull shape and relieve pressure on the brain. Seizures may be managed with anti-seizure medications. Children typically need support from many different specialists and may benefit from therapies to help with development and learning. Because this syndrome is so rare, treatment plans are highly individualized.

Also known as:

Key symptoms:

Skull bones fusing too early (craniosynostosis), causing an abnormal head shapeCalcium deposits building up inside the brain (intracranial calcifications)Intellectual disability or developmental delaysSeizures or epilepsyIncreased pressure inside the skull (intracranial hypertension)Unusual facial featuresVision problemsHearing difficultiesSlow growth or short statureFeeding difficulties in infancy

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 Craniosynostosis-intracranial calcifications syndrome.

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No actively recruiting trials found for Craniosynostosis-intracranial calcifications syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Craniosynostosis-intracranial calcifications syndrome community →

No specialists are currently listed for Craniosynostosis-intracranial calcifications 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 Craniosynostosis-intracranial calcifications 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

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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 craniosynostosis does my child have, and does surgery need to happen soon?,What genetic testing do you recommend to find the cause of this syndrome in our family?,How often should we monitor the calcium deposits in the brain, and can they grow over time?,What type of seizures does my child have, and which medication is best for them?,What developmental therapies should we start, and how soon?,What signs should prompt us to go to the emergency room?,Are there any specialists or centers with specific experience in this syndrome that you would recommend?

Common questions about Craniosynostosis-intracranial calcifications syndrome

What is Craniosynostosis-intracranial calcifications syndrome?

Craniosynostosis-intracranial calcifications syndrome, also sometimes called Craniosynostosis with intracranial calcifications or CIC syndrome, is a very rare condition that affects the skull and brain. Craniosynostosis means that the bones of the skull fuse together too early, before the brain has finished growing. This can cause the head to have an unusual shape and can put pressure on the growing brain. In this syndrome, there are also calcium deposits that build up inside the brain, which is called intracranial calcification. These calcium deposits can interfere with how the brain works no

How is Craniosynostosis-intracranial calcifications syndrome inherited?

Craniosynostosis-intracranial calcifications syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Craniosynostosis-intracranial calcifications syndrome typically begin?

Typical onset of Craniosynostosis-intracranial calcifications syndrome is neonatal. Age of onset can vary across affected individuals.