Didelphys uterus

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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:

Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

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

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

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No specialists are currently listed for Didelphys uterus.

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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 Didelphys uterus.

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Community

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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.