Microcephaly-cleft palate-abnormal retinal pigmentation syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:2521Q87.8
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

Microcephaly-cleft palate-abnormal retinal pigmentation syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. The three main features are microcephaly (a head that is smaller than expected), a cleft palate (an opening in the roof of the mouth), and unusual pigmentation patterns in the retina (the light-sensing tissue at the back of the eye). Because the brain is smaller than typical, children with this condition often experience intellectual disability and developmental delays. The cleft palate can cause difficulties with feeding, speech, and hearing. The abnormal retinal pigmentation may affect vision to varying degrees. This syndrome was first described in a small number of families, and very few cases have been reported in the medical literature. Because it is so rare, the full range of symptoms and the natural course of the disease are not completely understood. Some affected individuals may also have short stature, seizures, or other neurological problems. There is currently no cure for this condition. Treatment focuses on managing each symptom individually. Surgical repair of the cleft palate is typically performed in infancy. Children benefit from early intervention programs including speech therapy, occupational therapy, and special education support. Regular eye examinations are important to monitor vision. A team of specialists working together provides the best care for affected individuals and their families.

Key symptoms:

Abnormally small head (microcephaly)Opening in the roof of the mouth (cleft palate)Unusual pigmentation in the back of the eyeIntellectual disabilityDelayed developmentFeeding difficulties in infancySpeech problemsShort statureVision problemsPossible seizuresHearing difficultiesLearning challenges

Clinical phenotype terms (12)— hover any for plain English
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Microcephaly-cleft palate-abnormal retinal pigmentation syndrome.

View clinical trials →

No actively recruiting trials found for Microcephaly-cleft palate-abnormal retinal pigmentation syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Microcephaly-cleft palate-abnormal retinal pigmentation syndrome community →

No specialists are currently listed for Microcephaly-cleft palate-abnormal retinal pigmentation 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 Microcephaly-cleft palate-abnormal retinal pigmentation syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Microcephaly-cleft palate-abnormal retinal pigmentation syndromeForum →

No community posts yet. Be the first to share your experience with Microcephaly-cleft palate-abnormal retinal pigmentation syndrome.

Start the conversation →

Latest news about Microcephaly-cleft palate-abnormal retinal pigmentation syndrome

No recent news articles for Microcephaly-cleft palate-abnormal retinal pigmentation 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 is the severity of my child's microcephaly, and what does brain imaging show?,When should cleft palate repair surgery be scheduled, and what can we expect?,How will the abnormal retinal pigmentation affect my child's vision over time?,What early intervention therapies should we start right away?,Should we pursue genetic testing such as whole exome sequencing to look for a specific cause?,Are there any signs of seizures we should watch for at home?,What is the chance that future children could also be affected?

Common questions about Microcephaly-cleft palate-abnormal retinal pigmentation syndrome

What is Microcephaly-cleft palate-abnormal retinal pigmentation syndrome?

Microcephaly-cleft palate-abnormal retinal pigmentation syndrome is an extremely rare genetic condition that affects several parts of the body at the same time. The three main features are microcephaly (a head that is smaller than expected), a cleft palate (an opening in the roof of the mouth), and unusual pigmentation patterns in the retina (the light-sensing tissue at the back of the eye). Because the brain is smaller than typical, children with this condition often experience intellectual disability and developmental delays. The cleft palate can cause difficulties with feeding, speech, and

How is Microcephaly-cleft palate-abnormal retinal pigmentation syndrome inherited?

Microcephaly-cleft palate-abnormal retinal pigmentation syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Microcephaly-cleft palate-abnormal retinal pigmentation syndrome typically begin?

Typical onset of Microcephaly-cleft palate-abnormal retinal pigmentation syndrome is neonatal. Age of onset can vary across affected individuals.