Uterus didelphys

Uterus didelphys is a type of Müllerian duct anomaly (class III) where there is a complete duplication of uterine horns as well as duplication of the cervix, with no communication between them.

Epidemiology

Didelphic uteri account for approximately ~8% (range 5-11%) of Müllerian duct anomalies.

Associations

• renal agenesis
• vaginal septum which can include a transverse vaginal septum
  • there is a vaginal septum in 75% of cases, and obstruction to one horn is possible from occasional transverse septa

Clinical presentation

Many patients are asymptomatic although some may occasionally experience dyspareunia as a result of the vaginal septum.

Pathology

It results from failed ductal fusion that occurs between the 12 and 16 week of pregnancy and is characterized by two symmetric, widely divergent uterine horns and two cervices. The uterine volume in each duplicated segment is reduced. As with most uterine anatomical anomalies, there is an increased incidence of fertility issues, and Müllerian abnormalities, in general, are over-represented in infertile women. The chance of seeing a pregnancy to term is significantly reduced, down to only 20%, with a third of pregnancies ending in abortion and over half in premature deliveries. Only 40% of pregnancies resulted in living children .

Along with unicornuate uterus, uterus didelphys has the greatest impact on reproductive performance .

Radiographic features

Classically shows two widely spaced uterine corpora, each with a single Fallopian tube. Separate divergent uterine horns with large fundal cleft (as distinct from a septate uterus)

Uterus didelphys should be differentiated from a bicornuate uterus (separation of horns only) and a septate uterus (midline uterine septum).

Hysterosalpingogram (HSG)

HSG demonstrates two separate endocervical canals that open into separate fusiform endometrial cavities, with no communication between the two horns. Each endometrial cavity ends in a solitary fallopian tube.

If the anomaly is associated with an obstructed longitudinal vaginal septum, only one cervical os may be depicted, and it may be cannulated with the endometrial configuration mimicking a unicornuate uterus.

Ultrasound

Separate divergent uterine horns are identified with a large fundal cleft. Endometrial cavities are uniformly separate, with no evidence of communication. Two separate cervices need to be documented.

MRI

MR imaging demonstrates two separate uteri with widely divergent apices, two separate cervices, and usually an upper vaginal longitudinal septum. In each uterus, normal uterine zonal anatomy is preserved.

Treatment and prognosis

Complications

• infertility

• unilateral hydrocolpos/hematocolpos (if a vaginal septum is present)

• endometriosis

See also

• Müllerian duct anomaly classification
• Herlyn-Werner-Wunderlich syndrome

Siehe auch:

• Endometriose
• Fehlbildungen der Gebärmutter
• einseitige Nierenagenesie
• Müllerian duct anomaly classification
• Hydrokolpos
• Uterus septus
• Uterus bicornis
• uterine anomalies
• transverses Vaginalseptum
• Uterus unicornis
• obstruierte Hemivagina und ipsilaterale renale Anomalie (OHVIRA) Syndrom
• Hämatokolpos
• Uterus didelphys Schwangerschaft

und weiter:

• Hydrometra
• Hydrometrokolpos
• Hämatometra
• haematometrocolpos
• uterus didelphys with haematometrocolpos
• primary amenhorrea due to Müllerian duct anomalies
• Duplikatur des Uterus
• uterus didelphys with pregnancy