Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:1101Q87.8
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

Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome is an extremely rare genetic condition that affects multiple body systems from birth. The name describes its main features: anophthalmia (absence of one or both eyes), megalocornea (an abnormally large cornea in the eye that is present), heart defects (cardiopathy), and problems with bone and skeletal development. This syndrome is sometimes referred to by its Orphanet designation OMCS syndrome. Babies born with this condition typically show obvious eye abnormalities at birth, which may include one eye being completely absent while the other eye has an unusually large cornea. Heart defects can range from mild to severe and may include structural problems that affect how blood flows through the heart. Skeletal anomalies can involve abnormalities of the limbs, spine, or other bones, potentially affecting growth and movement. Because this syndrome is so rare, with only a handful of cases described in the medical literature, there is no specific cure or targeted treatment. Management focuses on addressing each symptom individually. This may include surgery for heart defects, prosthetic eyes for cosmetic and developmental purposes, orthopedic care for skeletal problems, and supportive therapies to help with development. A team of specialists working together is essential for providing the best possible care.

Also known as:

Key symptoms:

Absence of one or both eyes (anophthalmia)Abnormally large cornea in the remaining eyeHeart defects present at birthSkeletal and bone abnormalitiesAbnormal limb developmentShort stature or growth delaysJoint problems or limited joint movementPossible vision impairment or blindnessFacial differences related to missing eye(s)Possible developmental delays

Clinical phenotype terms (28)— hover any for plain English
MegalocorneaHP:0000485AniridiaHP:0000526AnophthalmiaHP:0000528Abnormality of the earHP:0000598Tricuspid valve prolapseHP:0001704
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome.

View clinical trials →

No actively recruiting trials found for Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome community →

No specialists are currently listed for Anophthalmia-megalocornea-cardiopathy-skeletal anomalies 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 Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndromeForum →

No community posts yet. Be the first to share your experience with Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome.

Start the conversation →

Latest news about Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome

No recent news articles for Anophthalmia-megalocornea-cardiopathy-skeletal anomalies 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.How severe is my child's heart defect, and will surgery be needed?,When should we start fitting a prosthetic eye, and how often will it need to be replaced?,What skeletal problems should we watch for as my child grows?,Is genetic testing available to confirm the diagnosis and help with family planning?,What early intervention services should we start right away?,How often will my child need follow-up appointments with each specialist?,Are there any clinical trials or research studies we could participate in?

Common questions about Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome

What is Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome?

Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome is an extremely rare genetic condition that affects multiple body systems from birth. The name describes its main features: anophthalmia (absence of one or both eyes), megalocornea (an abnormally large cornea in the eye that is present), heart defects (cardiopathy), and problems with bone and skeletal development. This syndrome is sometimes referred to by its Orphanet designation OMCS syndrome. Babies born with this condition typically show obvious eye abnormalities at birth, which may include one eye being completely absent wh

How is Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome inherited?

Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome typically begin?

Typical onset of Anophthalmia-megalocornea-cardiopathy-skeletal anomalies syndrome is neonatal. Age of onset can vary across affected individuals.