Unicervical bicornuate uterus

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Overview

Unicervical bicornuate uterus, also known as bicornuate uterus or bicornis unicollis uterus, is a congenital uterine anomaly classified as a Müllerian duct malformation. In this condition, the uterus has two separate horn-shaped cavities (cornua) that share a single cervix. It results from incomplete fusion of the two Müllerian (paramesonephric) ducts during embryonic development, typically between the 6th and 9th weeks of gestation. The external surface of the uterus shows a heart-shaped or deeply indented fundus, while internally the two uterine cavities may communicate to varying degrees near the cervix. This anomaly primarily affects the female reproductive system. Many women with a bicornuate uterus are asymptomatic and may only be diagnosed incidentally during imaging for other reasons or during pregnancy evaluation. However, the condition can be associated with significant reproductive complications including recurrent miscarriages, preterm labor, preterm delivery, malpresentation of the fetus (such as breech presentation), cervical incompetence, and intrauterine growth restriction. Some women may also experience dysmenorrhea (painful menstruation) or abnormal uterine bleeding. 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 accurate classification. Treatment is not always necessary, particularly in asymptomatic women or those without reproductive difficulties. For women experiencing recurrent pregnancy loss or other significant reproductive complications attributed to the anomaly, surgical correction through a procedure called Strassman metroplasty may be considered. This procedure involves unifying the two uterine horns into a single cavity. Cervical cerclage may also be employed in cases of cervical incompetence during pregnancy. Management is individualized based on the patient's symptoms and reproductive goals.

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 Unicervical bicornuate uterus.

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No actively recruiting trials found for Unicervical bicornuate uterus at this time.

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No specialists are currently listed for Unicervical 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 Unicervical bicornuate uterus.

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Community

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Common questions about Unicervical bicornuate uterus

What is Unicervical bicornuate uterus?

Unicervical bicornuate uterus, also known as bicornuate uterus or bicornis unicollis uterus, is a congenital uterine anomaly classified as a Müllerian duct malformation. In this condition, the uterus has two separate horn-shaped cavities (cornua) that share a single cervix. It results from incomplete fusion of the two Müllerian (paramesonephric) ducts during embryonic development, typically between the 6th and 9th weeks of gestation. The external surface of the uterus shows a heart-shaped or deeply indented fundus, while internally the two uterine cavities may communicate to varying degrees ne

How is Unicervical bicornuate uterus inherited?

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

At what age does Unicervical bicornuate uterus typically begin?

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