Bicornuate uterus

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:180134Q51.3
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

Bicornuate uterus is a congenital uterine anomaly classified as a Müllerian duct malformation, in which the uterus has two horn-shaped cavities (cornua) that share a single cervix, resulting from incomplete fusion of the two Müllerian (paramesonephric) ducts during embryonic development. It is categorized as a Class IV uterine anomaly under the American Fertility Society classification. The condition affects the female reproductive system and is typically present from birth, though it is often not discovered until adolescence or adulthood, frequently during evaluation for reproductive difficulties or through incidental imaging findings. Many women with a bicornuate uterus are asymptomatic and may have normal reproductive outcomes. However, the condition can be associated with an increased risk of adverse obstetric outcomes, including recurrent pregnancy loss, preterm labor, preterm delivery, malpresentation (such as breech presentation), cervical incompetence, and intrauterine growth restriction. Some women may also experience dysmenorrhea (painful menstruation) or abnormal uterine bleeding. The degree of separation between the two uterine horns can vary, ranging from a partial bicornuate uterus (with a minor indentation at the fundus) to a complete bicornuate uterus (with a deep cleft extending nearly to the cervix). Diagnosis is typically made through imaging modalities such as pelvic ultrasound, hysterosalpingography, magnetic resonance imaging (MRI), or three-dimensional ultrasound, with MRI considered the gold standard for definitive classification. Treatment is not always necessary, particularly in asymptomatic women or those without reproductive complications. When surgical intervention is indicated — usually for recurrent pregnancy loss attributed to the uterine anomaly — a metroplasty (Strassman procedure) may be performed to unify the two uterine cavities. Cervical cerclage may be considered in cases complicated by cervical incompetence during pregnancy. Management is individualized and often involves close obstetric monitoring during pregnancy.

Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Bicornuate uterus.

View clinical trials →

No actively recruiting trials found for Bicornuate uterus at this time.

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

Search ClinicalTrials.gov ↗Join the Bicornuate uterus community →

No specialists are currently listed for Bicornuate uterus.

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 Bicornuate uterus.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Bicornuate uterusForum →

No community posts yet. Be the first to share your experience with Bicornuate uterus.

Start the conversation →

Latest news about Bicornuate uterus

No recent news articles for Bicornuate uterus.

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.

Common questions about Bicornuate uterus

What is Bicornuate uterus?

Bicornuate uterus is a congenital uterine anomaly classified as a Müllerian duct malformation, in which the uterus has two horn-shaped cavities (cornua) that share a single cervix, resulting from incomplete fusion of the two Müllerian (paramesonephric) ducts during embryonic development. It is categorized as a Class IV uterine anomaly under the American Fertility Society classification. The condition affects the female reproductive system and is typically present from birth, though it is often not discovered until adolescence or adulthood, frequently during evaluation for reproductive difficul

How is Bicornuate uterus inherited?

Bicornuate uterus follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Bicornuate uterus typically begin?

Typical onset of Bicornuate uterus is adult. Age of onset can vary across affected individuals.