Pelvic dysplasia-arthrogryposis of lower limbs syndrome

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ORPHA:2840OMIM:602484Q68.8
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Overview

Pelvic dysplasia-arthrogryposis of lower limbs syndrome is an extremely rare condition that affects the development of the pelvis (hip bones) and causes stiffness and limited movement in the joints of the lower legs. The term "arthrogryposis" refers to joints that are fixed or locked in certain positions, making it difficult or impossible to move them normally. In this syndrome, the pelvis does not form correctly (pelvic dysplasia), and the joints of the hips, knees, and ankles may be stiff or contracted from birth. Babies born with this condition typically show obvious signs at birth, including abnormal positioning of the legs, limited ability to bend or straighten the lower limb joints, and an unusually shaped pelvis that can be seen on X-rays. The upper body and arms are usually not affected, which helps distinguish this condition from other forms of arthrogryposis that involve the whole body. Because this syndrome is so rare, there is very limited information about its full range of symptoms and long-term outcomes. Treatment is mainly supportive and focuses on improving joint movement and function through physical therapy, bracing, and sometimes surgery. There is currently no cure or specific medication for this condition. A team of specialists including orthopedic surgeons, physical therapists, and geneticists typically work together to manage care.

Also known as:

Key symptoms:

Stiff or locked joints in the legsAbnormal shape or development of the pelvisLimited movement at the hipsLimited movement at the kneesLimited movement at the anklesAbnormal positioning of the feetDifficulty walking or inability to walk normallyHip dislocation or instabilityMuscle weakness in the lower limbsShortened or underdeveloped leg bones

Clinical phenotype terms (10)— hover any for plain English
Slender long boneHP:0003100Hypoplastic pelvisHP:0008839Sacrococcygeal pilonidal abnormalityHP:0010767
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 Pelvic dysplasia-arthrogryposis of lower limbs syndrome.

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No actively recruiting trials found for Pelvic dysplasia-arthrogryposis of lower limbs syndrome at this time.

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No specialists are currently listed for Pelvic dysplasia-arthrogryposis of lower limbs 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 Pelvic dysplasia-arthrogryposis of lower limbs syndrome.

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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.How severe is my child's condition, and which joints are most affected?,What does the pelvic X-ray show, and how might this affect my child's ability to walk?,What physical therapy program do you recommend, and how often should sessions occur?,Will my child need surgery, and if so, when is the best time?,Is genetic testing available to help us understand the cause and the chance of this happening in future pregnancies?,What assistive devices or braces might help my child?,Are there any clinical trials or research studies we could participate in?

Common questions about Pelvic dysplasia-arthrogryposis of lower limbs syndrome

What is Pelvic dysplasia-arthrogryposis of lower limbs syndrome?

Pelvic dysplasia-arthrogryposis of lower limbs syndrome is an extremely rare condition that affects the development of the pelvis (hip bones) and causes stiffness and limited movement in the joints of the lower legs. The term "arthrogryposis" refers to joints that are fixed or locked in certain positions, making it difficult or impossible to move them normally. In this syndrome, the pelvis does not form correctly (pelvic dysplasia), and the joints of the hips, knees, and ankles may be stiff or contracted from birth. Babies born with this condition typically show obvious signs at birth, includ

How is Pelvic dysplasia-arthrogryposis of lower limbs syndrome inherited?

Pelvic dysplasia-arthrogryposis of lower limbs syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pelvic dysplasia-arthrogryposis of lower limbs syndrome typically begin?

Typical onset of Pelvic dysplasia-arthrogryposis of lower limbs syndrome is neonatal. Age of onset can vary across affected individuals.