Chondroectodermal dysplasia with night blindness

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Overview

Chondroectodermal dysplasia with night blindness is an extremely rare genetic condition that combines features of Ellis-van Creveld syndrome (a type of skeletal dysplasia) with difficulty seeing in low light or darkness (night blindness). Chondroectodermal dysplasia affects the growth of bones and cartilage, leading to short stature, particularly with shortened limbs. It also affects the development of teeth, nails, and sometimes the heart. When combined with night blindness, this condition also involves problems with the retina — the light-sensing tissue at the back of the eye — making it hard to see in dim lighting conditions. Key features of this condition include short arms and legs, extra fingers or toes (polydactyly), abnormal teeth and nails, and sometimes heart defects present at birth. The night blindness component means that affected individuals have trouble with vision in low-light environments, which can worsen over time. Some patients may also experience other eye problems. There is currently no cure for this condition. Treatment focuses on managing individual symptoms. Orthopedic care can help with bone and joint problems, dental treatment addresses tooth abnormalities, and heart defects may require surgical correction. Eye specialists can help manage the night blindness and monitor for progressive vision changes. Early diagnosis and a coordinated team of specialists are important for the best possible outcomes.

Key symptoms:

Short stature with shortened limbsExtra fingers or toes at birthSmall or missing fingernails and toenailsAbnormal or missing teethDifficulty seeing in dim light or darknessNarrow chestHeart defects present at birthKnock kneesShort ribsFusion of bones in the wristUpper lip abnormalitiesProgressive vision problemsThin or sparse hair

Clinical phenotype terms (27)— hover any for plain English
Metaphyseal dysplasiaHP:0100255Hyperconvex fingernailsHP:0001812Epiphyseal dysplasiaHP:0002656Metaphyseal irregularityHP:0003025Abnormal patella morphologyHP:0003045Recurrent long bone fracturesHP:0003084Wide humerusHP:0003886
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 Chondroectodermal dysplasia with night blindness.

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No actively recruiting trials found for Chondroectodermal dysplasia with night blindness at this time.

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No specialists are currently listed for Chondroectodermal dysplasia with night blindness.

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 Chondroectodermal dysplasia with night blindness.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Does my child have a heart defect, and if so, will it need surgery?,How severe is the night blindness, and is it expected to get worse over time?,What genetic testing should be done, and should other family members be tested?,What orthopedic treatments or surgeries might be needed as my child grows?,Are there any clinical trials or new treatments being studied for this condition?,What school accommodations should I request for my child?,How often should we see each specialist, and what monitoring is recommended?

Common questions about Chondroectodermal dysplasia with night blindness

What is Chondroectodermal dysplasia with night blindness?

Chondroectodermal dysplasia with night blindness is an extremely rare genetic condition that combines features of Ellis-van Creveld syndrome (a type of skeletal dysplasia) with difficulty seeing in low light or darkness (night blindness). Chondroectodermal dysplasia affects the growth of bones and cartilage, leading to short stature, particularly with shortened limbs. It also affects the development of teeth, nails, and sometimes the heart. When combined with night blindness, this condition also involves problems with the retina — the light-sensing tissue at the back of the eye — making it har

How is Chondroectodermal dysplasia with night blindness inherited?

Chondroectodermal dysplasia with night blindness follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Chondroectodermal dysplasia with night blindness typically begin?

Typical onset of Chondroectodermal dysplasia with night blindness is neonatal. Age of onset can vary across affected individuals.