Overview
Didelphys uterus, also known as uterus didelphys or double uterus, is a congenital Müllerian duct anomaly in which the two Müllerian (paramesonephric) ducts fail to fuse during embryonic development, resulting in the formation of two separate uterine bodies, each with its own cervix. In many cases, a longitudinal vaginal septum is also present, effectively creating a double vagina. This condition belongs to the broader category of Müllerian duct anomalies and is classified as a Class III uterine anomaly under the American Fertility Society classification system. Many individuals with uterus didelphys are asymptomatic and may not be diagnosed until puberty, during gynecological examination, or during pregnancy. When symptoms do occur, they may include dysmenorrhea (painful periods), dyspareunia (painful intercourse), and in some cases, obstructed hemivagina if a vaginal septum is present, which can lead to hematocolpos (accumulation of menstrual blood). The condition primarily affects the female reproductive system. Fertility is often preserved, though there is an increased risk of obstetric complications including recurrent miscarriage, preterm labor, malpresentation (such as breech presentation), and intrauterine growth restriction due to the reduced capacity of each individual uterine cavity. Treatment is generally not required in asymptomatic individuals. When a vaginal septum causes obstruction or symptoms, surgical excision of the septum may be performed. In cases of recurrent pregnancy loss or significant obstetric complications, surgical metroplasty (Strassman procedure) to unify the two uterine horns has been described, though this is rarely necessary. Management during pregnancy typically involves close obstetric monitoring. Cervical cerclage may be considered in cases of cervical insufficiency. Overall, reproductive outcomes are generally favorable with appropriate surveillance and management.
Also known as:
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Didelphys uterus.
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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 Didelphys uterus
What is Didelphys uterus?
Didelphys uterus, also known as uterus didelphys or double uterus, is a congenital Müllerian duct anomaly in which the two Müllerian (paramesonephric) ducts fail to fuse during embryonic development, resulting in the formation of two separate uterine bodies, each with its own cervix. In many cases, a longitudinal vaginal septum is also present, effectively creating a double vagina. This condition belongs to the broader category of Müllerian duct anomalies and is classified as a Class III uterine anomaly under the American Fertility Society classification system. Many individuals with uterus d
How is Didelphys uterus inherited?
Didelphys uterus follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.