Lethal multiple pterygium syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:33108OMIM:253290Q79.8
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

Lethal multiple pterygium syndrome (LMPS) is a very rare and severe condition that affects a baby before birth. It is sometimes called lethal pterygium syndrome or multiple pterygium syndrome, lethal type. The word 'pterygium' refers to webs or folds of skin that form across the joints, such as the neck, elbows, knees, and armpits. In this condition, these skin webs are so extensive that they severely restrict movement of the limbs and joints while the baby is still developing in the womb. Because the baby cannot move normally, many other problems develop, including fluid buildup (called hydrops fetalis), underdeveloped lungs, and abnormal facial features. The condition is almost always fatal either before birth (stillbirth) or very shortly after birth, which is why it is called 'lethal.' There is currently no cure or treatment that can reverse the condition. Care is focused on supporting families through the diagnosis, providing genetic counseling, and helping parents understand the risk of the condition occurring in future pregnancies. Families who receive this diagnosis are encouraged to work closely with a clinical geneticist and a maternal-fetal medicine specialist.

Also known as:

Key symptoms:

Webbing of skin across multiple joints (neck, elbows, knees, armpits, fingers)Severe restriction of joint movementFluid buildup throughout the baby's body (hydrops fetalis)Underdeveloped or poorly formed lungsAbnormal facial features including a small jaw and low-set earsCleft palate (opening in the roof of the mouth)Curved or fixed spine (scoliosis or rigid spine)Absent or reduced fetal movement in the wombSwelling of the skin (edema)Abnormally small head size (microcephaly) in some casesShortened or abnormally shaped limbsHeart defects in some casesStillbirth or death shortly after birth

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 Lethal multiple pterygium syndrome.

View clinical trials →

No actively recruiting trials found for Lethal multiple pterygium syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Lethal multiple pterygium syndrome community →

No specialists are currently listed for Lethal multiple pterygium 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 Lethal multiple pterygium syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Lethal multiple pterygium syndromeForum →

No community posts yet. Be the first to share your experience with Lethal multiple pterygium syndrome.

Start the conversation →

Latest news about Lethal multiple pterygium syndrome

No recent news articles for Lethal multiple pterygium 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 gene or genes caused this condition in our baby, and what does that mean for future pregnancies?,What is the chance that this could happen again in a future pregnancy?,What prenatal testing options are available to us if we decide to try for another pregnancy?,Is preimplantation genetic testing (PGT) during IVF an option for our family?,Should other family members be tested to see if they carry the same gene change?,What support services — such as grief counseling or support groups — are available to us?,Are there any research studies or registries we should know about for this condition?

Common questions about Lethal multiple pterygium syndrome

What is Lethal multiple pterygium syndrome?

Lethal multiple pterygium syndrome (LMPS) is a very rare and severe condition that affects a baby before birth. It is sometimes called lethal pterygium syndrome or multiple pterygium syndrome, lethal type. The word 'pterygium' refers to webs or folds of skin that form across the joints, such as the neck, elbows, knees, and armpits. In this condition, these skin webs are so extensive that they severely restrict movement of the limbs and joints while the baby is still developing in the womb. Because the baby cannot move normally, many other problems develop, including fluid buildup (called hydro

How is Lethal multiple pterygium syndrome inherited?

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

At what age does Lethal multiple pterygium syndrome typically begin?

Typical onset of Lethal multiple pterygium syndrome is neonatal. Age of onset can vary across affected individuals.