Lethal Larsen-like syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:2371OMIM:245650Q74.8
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 Larsen-like syndrome is an extremely rare and severe skeletal disorder that affects how bones and joints form before birth. It belongs to a group of conditions called skeletal dysplasias, which means the bones develop abnormally during fetal development. The condition shares some features with Larsen syndrome — such as dislocated joints and unusual facial features — but is far more severe and is typically fatal either before birth or very shortly after birth, which is why it is called 'lethal.' The syndrome is also sometimes referred to as lethal Larsen syndrome or a lethal variant of Larsen syndrome. Babies affected by this condition are usually found to have multiple dislocated joints, abnormal bone development throughout the skeleton, and serious problems with the spine. The face may look different from typical, with a flat midface, wide-set eyes, and a prominent forehead. Because the condition is so severe, the lungs and other vital organs often cannot develop properly, making survival outside the womb extremely unlikely. There is currently no cure or disease-modifying treatment for lethal Larsen-like syndrome. Care is focused on supporting families through diagnosis, genetic counseling, and bereavement. Prenatal imaging and genetic testing play a key role in identifying the condition before birth.

Key symptoms:

Multiple dislocated joints present at birthAbnormal bone formation throughout the skeletonFlat or underdeveloped midfaceWide-set eyesProminent foreheadSevere spinal abnormalitiesShort limbsUnderdeveloped or poorly formed lungsStillbirth or death shortly after birthAbnormal skull shapeCleft palate in some cases

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 ↗

FDA & Trial Timeline

10 events
Mar 2026

KETAMINE HYDROCHLORIDE: New indication approved

FDAcompleted
Mar 2026KETALAR: New indication approved
FDAcompleted
Mar 2026

KETALAR: New indication approved

FDAcompleted
Oct 2025KETALAR: New indication approved
FDAcompleted
Jan 2023

KETAMINE HYDROCHLORIDE: FDA approved

FDAcompleted
Aug 2022KETALAR: New indication approved
FDAcompleted
Jun 2022KETALAR: New indication approved
FDAcompleted
Aug 2020KETALAR: New indication approved
FDAcompleted
Aug 2018KETALAR: New indication approved
FDAcompleted
Apr 2017KETALAR: New indication approved
FDAcompleted

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Lethal Larsen-like syndrome.

View clinical trials →

No actively recruiting trials found for Lethal Larsen-like syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Lethal Larsen-like syndrome community →

No specialists are currently listed for Lethal Larsen-like 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 Larsen-like syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Lethal Larsen-like syndromeForum →

No community posts yet. Be the first to share your experience with Lethal Larsen-like syndrome.

Start the conversation →

Latest news about Lethal Larsen-like syndrome

No recent news articles for Lethal Larsen-like 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 genetic testing should we have done to confirm the diagnosis and find the exact cause?,What is the chance this condition could happen again in a future pregnancy?,Are there prenatal tests available to check for this condition in future pregnancies?,Should other family members be tested for the gene change?,Can you refer us to a bereavement counselor or support group for families who have experienced this?,Are there any research studies or registries we should know about for this condition?,What specialists should be involved in our care going forward?

Common questions about Lethal Larsen-like syndrome

What is Lethal Larsen-like syndrome?

Lethal Larsen-like syndrome is an extremely rare and severe skeletal disorder that affects how bones and joints form before birth. It belongs to a group of conditions called skeletal dysplasias, which means the bones develop abnormally during fetal development. The condition shares some features with Larsen syndrome — such as dislocated joints and unusual facial features — but is far more severe and is typically fatal either before birth or very shortly after birth, which is why it is called 'lethal.' The syndrome is also sometimes referred to as lethal Larsen syndrome or a lethal variant of L

How is Lethal Larsen-like syndrome inherited?

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

At what age does Lethal Larsen-like syndrome typically begin?

Typical onset of Lethal Larsen-like syndrome is neonatal. Age of onset can vary across affected individuals.