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.
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Bicornuate uterus.
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Specialists
View all specialists →No specialists are currently listed for Bicornuate uterus.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Bicornuate uterus.
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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.