Congenital limbs-face contractures-hypotonia-developmental delay syndrome

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ORPHA:562528OMIM:616266Q87.8
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Overview

Congenital limbs-face contractures-hypotonia-developmental delay syndrome is a very rare genetic condition that is present from birth. It affects multiple parts of the body, including the limbs, face, muscles, and brain development. The name describes the main features: contractures (stiff, fixed joints that cannot move freely), unusual facial features, low muscle tone (hypotonia), and delays in reaching developmental milestones like sitting, walking, and talking. Children born with this condition often have joints in their arms and legs that are bent or fixed in unusual positions, making movement difficult. Their facial appearance may look different from other family members, and their muscles may feel floppy or weak. Because the brain is also affected, children may take longer to learn new skills, and some may have intellectual disability. There is currently no cure for this syndrome. Treatment focuses on managing symptoms and helping children reach their full potential. This includes physical therapy to improve movement, occupational therapy to help with daily tasks, speech therapy for communication, and special education support. A team of specialists works together to support both the child and their family throughout life.

Also known as:

Key symptoms:

Stiff or fixed joints in the arms and legs (contractures) present at birthLow muscle tone (floppy muscles, also called hypotonia)Unusual facial features such as a small jaw, widely spaced eyes, or a flat nasal bridgeDelays in reaching milestones like sitting, walking, and talkingIntellectual disability or learning difficultiesFeeding difficulties in infancyShort stature or slow growthCurved or abnormally shaped fingers or toesLimited range of motion in multiple jointsPossible breathing difficulties due to weak muscles

Clinical phenotype terms (50)— hover any for plain English
CamptodactylyHP:0012385Pursed lipsHP:0000205Ulnar deviation of the hand or of fingers of the handHP:0001193Short columellaHP:0002000
Inheritance

Autosomal dominant

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

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 Congenital limbs-face contractures-hypotonia-developmental delay syndrome.

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No actively recruiting trials found for Congenital limbs-face contractures-hypotonia-developmental delay syndrome at this time.

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No specialists are currently listed for Congenital limbs-face contractures-hypotonia-developmental delay 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 Congenital limbs-face contractures-hypotonia-developmental delay syndrome.

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Community

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Latest news about Congenital limbs-face contractures-hypotonia-developmental delay syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

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 genetic tests should my child have, and how long will results take?,Which therapies are most important to start right away, and how often should my child receive them?,Are there any breathing or swallowing concerns I should watch for at home?,What does my child's developmental outlook look like, and what milestones should I realistically expect?,Are there any clinical trials or research studies my child might be eligible for?,How should I coordinate care between all the different specialists my child needs?,Are there patient support groups or family networks for this condition that you can connect us with?

Common questions about Congenital limbs-face contractures-hypotonia-developmental delay syndrome

What is Congenital limbs-face contractures-hypotonia-developmental delay syndrome?

Congenital limbs-face contractures-hypotonia-developmental delay syndrome is a very rare genetic condition that is present from birth. It affects multiple parts of the body, including the limbs, face, muscles, and brain development. The name describes the main features: contractures (stiff, fixed joints that cannot move freely), unusual facial features, low muscle tone (hypotonia), and delays in reaching developmental milestones like sitting, walking, and talking. Children born with this condition often have joints in their arms and legs that are bent or fixed in unusual positions, making mov

How is Congenital limbs-face contractures-hypotonia-developmental delay syndrome inherited?

Congenital limbs-face contractures-hypotonia-developmental delay syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Congenital limbs-face contractures-hypotonia-developmental delay syndrome typically begin?

Typical onset of Congenital limbs-face contractures-hypotonia-developmental delay syndrome is neonatal. Age of onset can vary across affected individuals.