Cleidorhizomelic syndrome

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ORPHA:1453OMIM:119650Q77.8
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8Treatment centers

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Overview

Cleidorhizomelic syndrome is an extremely rare skeletal disorder that affects bone development. The name describes its key features: 'cleido' refers to the clavicles (collarbones), and 'rhizomelic' refers to the bones closest to the body in the arms and legs (the upper arms and thighs). People with this condition are born with absent or underdeveloped collarbones and shortened upper limbs, particularly affecting the humerus (upper arm bone) and femur (thigh bone). This pattern of bone shortening is called rhizomelic shortening because it primarily affects the proximal (closer to the body) segments of the limbs. The condition is present from birth and can be identified through physical examination and skeletal X-rays. Affected individuals may have a characteristic appearance with narrow or drooping shoulders due to the collarbone abnormalities, and disproportionately short upper arms and thighs compared to the forearms and lower legs. Additional skeletal findings may include other bone abnormalities. Because this syndrome is so rare, with very few cases described in the medical literature, the treatment landscape is limited to supportive and symptomatic care. There is no specific cure or targeted therapy. Management typically involves orthopedic monitoring, physical therapy, and addressing any functional limitations that arise from the skeletal differences. Genetic counseling is recommended for affected families to understand the inheritance pattern and recurrence risk.

Also known as:

Key symptoms:

Absent or underdeveloped collarbonesShortened upper arm bonesShortened thigh bonesDrooping or narrow shouldersShort statureDisproportionate limb shortening affecting upper arms and thighs more than forearms and lower legsPossible wrist or hand bone abnormalitiesLimited range of motion in affected jointsAbnormal gait or walking pattern

Clinical phenotype terms (7)— hover any for plain English
Short middle phalanx of the 5th fingerHP:0004220Diaphyseal undertubulationHP:0005019
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 Cleidorhizomelic syndrome.

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

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No specialists are currently listed for Cleidorhizomelic 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 Cleidorhizomelic syndrome.

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Community

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Caregiver Resources

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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 bones are affected in my child, and how might this change as they grow?,Should we pursue genetic testing to identify the exact cause, and would this help other family members?,What physical therapy or occupational therapy programs would be most helpful?,Are there any complications we should watch for as my child gets older?,What is the expected adult height, and are there any growth-related treatments to consider?,How can we best support my child's participation in school and physical activities?,What is the chance of this condition occurring again in future pregnancies?

Common questions about Cleidorhizomelic syndrome

What is Cleidorhizomelic syndrome?

Cleidorhizomelic syndrome is an extremely rare skeletal disorder that affects bone development. The name describes its key features: 'cleido' refers to the clavicles (collarbones), and 'rhizomelic' refers to the bones closest to the body in the arms and legs (the upper arms and thighs). People with this condition are born with absent or underdeveloped collarbones and shortened upper limbs, particularly affecting the humerus (upper arm bone) and femur (thigh bone). This pattern of bone shortening is called rhizomelic shortening because it primarily affects the proximal (closer to the body) segm

How is Cleidorhizomelic syndrome inherited?

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

At what age does Cleidorhizomelic syndrome typically begin?

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