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Uterus Didelphys (Double Uterus)

Complete Müllerian Duct Duplication

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Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Kadın Hastalıkları ve Doğum department. Book Appointment →

What is Uterus Didelphys (Double Uterus)?

Uterus didelphys (American Society for Reproductive Medicine class III; ESHRE/ESGE U3) is a complete failure of Müllerian duct fusion, resulting in two separate uterine cavities, two cervices, and often a longitudinal vaginal septum.

Embryology: occurs at 6-13 weeks gestation when paramesonephric (Müllerian) ducts fail to fuse in midline.

Prevalence: 0.1-0.5% of women; 8% of all Müllerian anomalies.

Associated anomalies: 25% have ipsilateral renal agenesis; OHVIRA syndrome (Obstructed HemiVagina with Ipsilateral Renal Agenesis) is a specific variant requiring urgent surgical management.

Distinct from septate uterus (residual midline septum after fused ducts) and bicornuate uterus (partial fusion failure with single cervix).

Generally compatible with successful pregnancy in one or both horns; obstetric outcomes inferior to normal uterus but better than untreated septate.

Symptoms

Often asymptomatic and discovered incidentally on imaging or pelvic examination.
Hematocolpos and cyclic pelvic pain in OHVIRA syndrome (obstructed hemivagina).
Dyspareunia (longitudinal vaginal septum).
Recurrent miscarriage (less common than septate; 25-30% pregnancy loss rate).
Preterm birth (15-25%).
Intrauterine growth restriction (IUGR).
Malpresentation (breech, transverse).
Primary infertility (less common than septate).
Cesarean delivery rates higher (40-60%).

Risk Factors

Family history of Müllerian anomalies (rare).
Maternal teratogen exposure during embryogenesis.
Idiopathic in most cases.
Genetic conditions: rare syndromic associations (e.g., Bardet-Biedl, McKusick-Kaufman).

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Cyclic pelvic pain in adolescent girl with normal menses (suspicion for OHVIRA).
  • Recurrent miscarriage with imaging suggesting Müllerian anomaly.
  • Pregnancy with imaging showing two separate uterine horns.
  • Severe dyspareunia with vaginal septum.
  • Preterm birth or recurrent malpresentation history.
  • Renal anomaly identified, considering screening for associated Müllerian anomaly.

Treatment Methods

01
Diagnosis: 3D transvaginal ultrasound, MRI pelvis (gold standard for complex anomalies), examination, hysteroscopy, laparoscopy.
02
Renal imaging: ultrasound or MRI to screen for renal agenesis or other urinary tract anomalies.
03
Most cases: no surgical intervention required; expectant pregnancy management with close monitoring.
04
OHVIRA syndrome: urgent vaginal septum incision/excision to relieve hematocolpos; preserve fertility.
05
Vaginal septum resection for dyspareunia or obstruction (transvaginal incision and reapproximation).
06
Strassmann metroplasty (abdominal unification): rarely indicated; high morbidity, indicated only for severe recurrent pregnancy loss in selected cases.
07
Pregnancy management: serial ultrasound for fetal growth and cervical length, screening for preterm labor.
08
Cervical cerclage if cervical incompetence develops.
09
Mode of delivery: cesarean indicated for malpresentation or obstetric reasons; vaginal delivery possible.
10
Postpartum: monitor for retained placenta in non-pregnant horn (rare).
11
Genetic counseling for fertility planning, with particular attention to renal screening of offspring.
12
Long-term: monitor for endometrial pathology (in both horns), adequate Pap smear coverage of both cervices.

Which Department to Visit?

You can visit our Kadın Hastalıkları ve Doğum department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.