Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome

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ORPHA:459061OMIM:620062Q87.8
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8Treatment centers

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Overview

Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome is an extremely rare genetic condition that affects multiple body systems. The name describes its main features: abnormal development of the skull and face (craniofacial dysplasia), shorter-than-expected height (short stature), problems with structures that develop from the outer layer of the embryo such as skin, hair, teeth, and nails (ectodermal anomalies), and difficulties with thinking and learning (intellectual disability). People with this syndrome typically show distinctive facial features that may include an unusually shaped skull, widely spaced eyes, a flat or underdeveloped midface, and abnormalities of the jaw. The ectodermal problems can lead to thin or sparse hair, abnormal or missing teeth, dry skin, and nail changes. Growth is often slower than expected from early childhood, resulting in short stature. Intellectual disability can range from mild to moderate, affecting learning, speech development, and daily functioning. Because this condition is so rare, there is currently no specific cure or targeted treatment. Management focuses on addressing individual symptoms through a team of specialists. This may include growth monitoring, dental care, skin treatments, educational support, and therapies to help with speech and development. Early intervention and supportive care can help improve quality of life for affected individuals.

Also known as:

Key symptoms:

Abnormal skull and face shapeShort stature or slow growthThin or sparse hairAbnormal or missing teethNail abnormalitiesDry or unusual skinIntellectual disabilityDelayed speech and languageLearning difficultiesWidely spaced eyesFlat or underdeveloped midfaceDelayed developmental milestones

Clinical phenotype terms (40)— hover any for plain English
Posterior fossa cystHP:0007291CamptodactylyHP:0012385
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 Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome.

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No actively recruiting trials found for Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome at this time.

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No specialists are currently listed for Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability 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 Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome.

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Community

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Latest news about Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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Family & Caregiver Grants

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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 has been done, and are there additional tests that could help identify the exact cause?,What specialists should my child see, and how often?,Is growth hormone therapy appropriate for my child's short stature?,What educational and developmental support services should we pursue?,Are there any clinical trials or research studies we could participate in?,What should we watch for as warning signs of complications?,Can you help us connect with other families affected by similar conditions?

Common questions about Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome

What is Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome?

Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome is an extremely rare genetic condition that affects multiple body systems. The name describes its main features: abnormal development of the skull and face (craniofacial dysplasia), shorter-than-expected height (short stature), problems with structures that develop from the outer layer of the embryo such as skin, hair, teeth, and nails (ectodermal anomalies), and difficulties with thinking and learning (intellectual disability). People with this syndrome typically show distinctive facial features that m

How is Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome inherited?

Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome typically begin?

Typical onset of Craniofacial dysplasia-short stature-ectodermal anomalies-intellectual disability syndrome is neonatal. Age of onset can vary across affected individuals.