Stromme syndrome

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ORPHA:506307OMIM:243605Q13.8
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Overview

Stromme syndrome is a very rare genetic condition that affects multiple parts of the body, especially the eyes, intestines, and brain. It is sometimes called CILD-like syndrome or referred to by its genetic cause, CENPF-related disorder. The condition is caused by changes (mutations) in a gene called CENPF, which plays an important role in how cells divide and develop before and after birth. Children with Stromme syndrome are typically born with a combination of problems. These often include missing or underdeveloped parts of the eye (called microphthalmia or iris coloboma), a blockage or gap in the small intestine (called jejunal atresia), and a smaller-than-usual head size (microcephaly). Brain development is also affected, which can lead to intellectual disability and developmental delays. Some children may also have kidney problems or heart defects. Because Stromme syndrome is so rare, there is no specific cure. Treatment focuses on managing each symptom individually. Intestinal problems often require surgery shortly after birth. Eye problems may need glasses, patching, or surgery. Developmental delays are supported through physical, occupational, and speech therapy. A team of specialists is usually needed to care for children with this condition.

Also known as:

Key symptoms:

Small or underdeveloped eyes (microphthalmia)Gaps or holes in the iris or other eye structures (coloboma)Blockage or missing section of the small intestine (jejunal atresia)Smaller-than-normal head size (microcephaly)Intellectual disability or learning difficultiesDelayed development of motor and speech skillsKidney abnormalitiesHeart defectsAbnormal brain structure seen on brain scansFeeding difficulties in infancyLow muscle tone (hypotonia)

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 Stromme syndrome.

View clinical trials →

No actively recruiting trials found for Stromme syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Stromme syndrome community →

No specialists are currently listed for Stromme 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 Stromme syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Stromme syndrome

No recent news articles for Stromme syndrome.

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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.Which specialists does my child need to see, and how often?,What genetic testing should be done to confirm the diagnosis, and should other family members be tested?,What early intervention therapies are most important to start right away?,What signs or symptoms should prompt me to seek emergency care?,Are there any clinical trials or research studies we could participate in?,What can we expect for my child's development and independence as they grow older?,What support services or patient organizations are available for families affected by Stromme syndrome?

Common questions about Stromme syndrome

What is Stromme syndrome?

Stromme syndrome is a very rare genetic condition that affects multiple parts of the body, especially the eyes, intestines, and brain. It is sometimes called CILD-like syndrome or referred to by its genetic cause, CENPF-related disorder. The condition is caused by changes (mutations) in a gene called CENPF, which plays an important role in how cells divide and develop before and after birth. Children with Stromme syndrome are typically born with a combination of problems. These often include missing or underdeveloped parts of the eye (called microphthalmia or iris coloboma), a blockage or gap

How is Stromme syndrome inherited?

Stromme syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Stromme syndrome typically begin?

Typical onset of Stromme syndrome is neonatal. Age of onset can vary across affected individuals.