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. 2011 Jan 1;5(1):1–8. doi: 10.3941/jrcr.v5i1.572

Table 1.

Summary table for uterus didelphys variations

Etiology Complete nonfusion of uterovaginal horns
Incidence 1 per 1000 live births
Age Predilection Uterus didelphys with obstruction in a patient presenting at menarche with cyclical dysmenorrhea and pelvic mass
Risk Factors Genetics
Majority of cases sporadic or multifactorial in origin
May be caused by an autosomal dominant or recessive gene in certain cases
Hepatocyte nuclear factor (HNF-1) β mutations reported in association with renal anomalies and diabetes
Anatomic Types American Fertility Society classification
Class I: Segmental agenesis, hypoplasia
Occurs in a variety of forms
Complete form most common (Mayer-Rokitansky-Kuster-Hauser syndrome)
Class II: Unicornuate uterus
May have rudimentary horn, which may or may not communicate with main uterine cavity
Class III: Uterus didelphys
Class IV: Bicornuate uterus
Class V: Septate uterus
Class VI: Arcuate uterus
Class VII: DES exposure
Treatment Expectant
Metroplasty leaving duplicated cervix intact in selected patients with recurrent spontaneous abortions and premature deliveries
Traditionally performed abdominally via a Pfannenstiel approach (e.g., Strassman metroplasty)
Currently performed by a combined hysteroscopic and laparoscopic approach
Benefits of metroplasty unclear
Hysteroscopic resection of vaginal septum in patients with an obstructing vaginal septum
Prognosis Generally good
≈ 25% of women with uterine anomalies have reproductive problems
≈ 3% of women with repeated pregnancy loss
Findings on Imaging MR Findings
  • Not recommended in first trimester

  • Unknown effects on organogenesis

  • Study of choice for evaluating non-pregnant patient

  • Accuracy approaching 100%

  • Image plane parallel to long axis of uterus

  • Optimal assessment of fundal contour

  • T2WI

  • High signal endometrium

  • Low signal junctional zone

  • Intermediate signal myometrium

  • T1WI occasionally useful

  • High signal fat may outline low signal uterus

Ultrasonographic Findings
  • Primary modality of evaluating uterine duplication in pregnancy

  • Reported accuracy 90–92%

  • 3D ultrasound provides improved spatial delineation

  • Sensitivity 93%, specificity 100%