Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:168552OMIM:607543Q77.8
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

Spondylometaphyseal dysplasia with bowed forearms and facial dysmorphism syndrome is an extremely rare genetic bone disorder that affects the growth and development of the skeleton. The name describes its main features: 'spondylo' refers to the spine, 'metaphyseal' refers to the growing ends of long bones, and 'dysplasia' means abnormal development. People with this condition typically have abnormalities in their vertebrae (bones of the spine), unusual changes at the ends of their long bones (metaphyseal irregularities), characteristic bowing of the forearm bones, and distinctive facial features. The condition is usually noticed in early childhood when skeletal differences become apparent. Key features may include short stature, bowed forearms, a flattened appearance of the vertebrae (platyspondyly), and facial features such as a flat midface or broad forehead. Because the bones are affected throughout the body, children may experience difficulty with movement and may be shorter than expected for their age. There is currently no cure for this condition. Treatment focuses on managing symptoms and may include orthopedic interventions to address bone deformities, physical therapy to maintain mobility, and regular monitoring of growth and skeletal development. Pain management and supportive care are also important parts of the treatment plan. Because this is such a rare condition, management is typically guided by a team of specialists who tailor care to each individual's needs.

Key symptoms:

Short statureBowed forearmsAbnormal curvature or flattening of the spine bonesDistinctive facial featuresFlat or broad faceIrregularities at the ends of long bonesLimited range of motion in the armsJoint stiffnessWaddling gait or difficulty walkingLeg bowingShort limbs relative to the trunkBroad forehead

Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome.

View clinical trials →

No actively recruiting trials found for Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome community →

No specialists are currently listed for Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism 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 Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndromeForum →

No community posts yet. Be the first to share your experience with Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome.

Start the conversation →

Latest news about Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome

No recent news articles for Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism 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 specific skeletal abnormalities does my child have, and how might they change over time?,Is genetic testing available to confirm the diagnosis, and should other family members be tested?,What orthopedic treatments or surgeries might be needed as my child grows?,How often should we schedule follow-up X-rays and specialist visits?,Are there physical activities my child should avoid to protect their bones?,What support services are available for growth and developmental concerns?,Are there any clinical trials or research studies we could participate in?

Common questions about Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome

What is Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome?

Spondylometaphyseal dysplasia with bowed forearms and facial dysmorphism syndrome is an extremely rare genetic bone disorder that affects the growth and development of the skeleton. The name describes its main features: 'spondylo' refers to the spine, 'metaphyseal' refers to the growing ends of long bones, and 'dysplasia' means abnormal development. People with this condition typically have abnormalities in their vertebrae (bones of the spine), unusual changes at the ends of their long bones (metaphyseal irregularities), characteristic bowing of the forearm bones, and distinctive facial featur

How is Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome inherited?

Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome typically begin?

Typical onset of Spondylometaphyseal dysplasia-bowed forearms-facial dysmorphism syndrome is childhood. Age of onset can vary across affected individuals.