Cerebral gigantism-jaw cysts syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:2081
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Cerebral gigantism-jaw cysts syndrome, also known as Sotos syndrome with jaw cysts or sometimes referenced in relation to Gorlin-like features combined with overgrowth, is an extremely rare condition that combines features of excessive growth (cerebral gigantism) with the development of cysts in the jawbones. Children with this condition typically show rapid growth before and after birth, resulting in a large head size, tall stature, and distinctive facial features including a prominent forehead and a long face. The jaw cysts, which are fluid-filled sacs that develop in the bones of the jaw, can cause dental problems, jaw swelling, and pain. Some affected individuals may also experience learning difficulties or mild intellectual disability, delayed motor development, and behavioral challenges. Because this condition is so rare, the treatment approach is largely supportive and symptom-based. Growth and development are monitored closely throughout childhood. Jaw cysts may require surgical removal, and dental care is an important part of ongoing management. Genetic counseling is recommended for affected families to understand the inheritance pattern and recurrence risk.

Also known as:

Key symptoms:

Excessive growth and tall stature in childhoodLarge head size (macrocephaly)Prominent foreheadLong face with distinctive facial featuresCysts in the jawbonesJaw swelling or painDental abnormalitiesMild to moderate intellectual disabilityDelayed motor milestones such as walkingSpeech and language delaysAdvanced bone age on X-raysBehavioral difficultiesLarge hands and feetPoor coordination or clumsiness

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Cerebral gigantism-jaw cysts syndrome.

View clinical trials →

No actively recruiting trials found for Cerebral gigantism-jaw cysts syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Cerebral gigantism-jaw cysts syndrome community →

No specialists are currently listed for Cerebral gigantism-jaw cysts 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 Cerebral gigantism-jaw cysts syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Cerebral gigantism-jaw cysts syndromeForum →

No community posts yet. Be the first to share your experience with Cerebral gigantism-jaw cysts syndrome.

Start the conversation →

Latest news about Cerebral gigantism-jaw cysts syndrome

No recent news articles for Cerebral gigantism-jaw cysts syndrome.

Follow this condition to be notified when news becomes available.

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 specific genetic testing should my child have to confirm this diagnosis?,How often should my child have jaw imaging to check for new cysts?,What developmental therapies would benefit my child the most right now?,Are there any signs or symptoms I should watch for that would need urgent medical attention?,Will my child's growth rate slow down as they get older?,What is the chance that future children in our family could have this condition?,Are there any specialists or centers with experience managing this specific condition?

Common questions about Cerebral gigantism-jaw cysts syndrome

What is Cerebral gigantism-jaw cysts syndrome?

Cerebral gigantism-jaw cysts syndrome, also known as Sotos syndrome with jaw cysts or sometimes referenced in relation to Gorlin-like features combined with overgrowth, is an extremely rare condition that combines features of excessive growth (cerebral gigantism) with the development of cysts in the jawbones. Children with this condition typically show rapid growth before and after birth, resulting in a large head size, tall stature, and distinctive facial features including a prominent forehead and a long face. The jaw cysts, which are fluid-filled sacs that develop in the bones of the jaw, c

How is Cerebral gigantism-jaw cysts syndrome inherited?

Cerebral gigantism-jaw cysts syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Cerebral gigantism-jaw cysts syndrome typically begin?

Typical onset of Cerebral gigantism-jaw cysts syndrome is childhood. Age of onset can vary across affected individuals.