Overview
Bicervical bicornuate uterus with patent cervix and vagina is a congenital uterine malformation classified among Müllerian duct anomalies. In this condition, the uterus has two separate uterine horns (bicornuate) and two distinct cervices (bicervical), while both cervical canals remain open (patent) and the vagina is single and normally formed. This anomaly results from incomplete fusion of the paired Müllerian (paramesonephric) ducts during embryonic development, typically between the 6th and 9th weeks of gestation. The condition primarily affects the female reproductive system. Many individuals with this uterine anomaly may be asymptomatic and the condition may only be discovered incidentally during imaging studies, gynecological examination, or evaluation for reproductive difficulties. However, some affected women may experience adverse reproductive outcomes including recurrent miscarriage, preterm labor, malpresentation of the fetus, and other obstetric complications. Menstrual irregularities may also occur in some cases. The condition is typically diagnosed through imaging modalities such as pelvic ultrasound, hysterosalpingography, MRI, or direct visualization during laparoscopy or hysteroscopy. Treatment depends on the clinical presentation and reproductive goals of the patient. Many women with this condition can have successful pregnancies with appropriate obstetric monitoring, and no surgical intervention may be necessary. In cases of recurrent pregnancy loss attributed to the uterine anomaly, surgical metroplasty (Strassman procedure) may be considered to unify the two uterine horns, though this is not routinely performed. Management is individualized and typically involves close collaboration between gynecologists and reproductive medicine specialists.
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 Bicervical bicornuate uterus with patent cervix and vagina.
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Specialists
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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
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Common questions about Bicervical bicornuate uterus with patent cervix and vagina
What is Bicervical bicornuate uterus with patent cervix and vagina?
Bicervical bicornuate uterus with patent cervix and vagina is a congenital uterine malformation classified among Müllerian duct anomalies. In this condition, the uterus has two separate uterine horns (bicornuate) and two distinct cervices (bicervical), while both cervical canals remain open (patent) and the vagina is single and normally formed. This anomaly results from incomplete fusion of the paired Müllerian (paramesonephric) ducts during embryonic development, typically between the 6th and 9th weeks of gestation. The condition primarily affects the female reproductive system. Many individ
How is Bicervical bicornuate uterus with patent cervix and vagina inherited?
Bicervical bicornuate uterus with patent cervix and vagina follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Bicervical bicornuate uterus with patent cervix and vagina typically begin?
Typical onset of Bicervical bicornuate uterus with patent cervix and vagina is adult. Age of onset can vary across affected individuals.