Müllerian duct anomalies

Last revised by Ashesh Ishwarlal Ranchod on 31 Jan 2024

Müllerian duct anomalies (MDAs) are congenital abnormalities that occur when the Müllerian ducts (paramesonephric ducts) do not develop correctly. This may be as a result of complete agenesis, defective vertical or lateral fusion, or resorption failure.

MDAs are estimated to occur in 1-5% of all women. There is a higher rate of women with repeated miscarriages (3-15%) 2,5

Despite these anomalies being common 1, the majority are asymptomatic. Obstruction of the Müllerian duct may occur, and patients present with an abdominal mass and dysmenorrhea. Delayed treatment may result in severe consequences and potentially infertility. Patients may also present with recurrent miscarriages and infertility 2,5

The Müllerian duct anomaly classification system divides them according to clinical manifestations, prognosis, and treatment. Accurate diagnosis is essential since management varies according to the type of malformation.

Renal anomalies are frequently associated, most commonly renal agenesis but also crossed fused renal ectopia, and duplex kidney 2

  • should be performed initially
  • confirms any structural abnormalities of the genital tract
  • sometimes cannot help to identify the type of MDA (especially on 2D imaging alone)
  • 3D coronal transvaginal imaging has a high degree of diagnostic accuracy and ideally should be performed in the secretory phase of the menstrual cycle
  • valuable noninvasive technique
  • evaluation of the female pelvic anatomy
  • accurate MDA classification

Many patients are asymptomatic and require no treatment. However, where obstruction occurs, surgical intervention is usually required and may result in permanent infertility - counseling is required.

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Cases and figures

  • Figure 1: diagram: Müllerian duct development
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  • Figure 2: uterine anatomical abnormalities (illustrations)
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  • Case 1: bicornuate Uterus
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  • Case 2: bicornuate uterus
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  • Case 3: septate uterus
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  • Case 4: arcuate uterus
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  • Case 6: class II Mullerian duct anomaly
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  • Case 7: unicornuate uterus
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  • Case 8: unicornuate uterus- type B
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  • Case 9: Bicornuate uterus with triamniotic dichorionic triplet pregnancy (3D ultrasound)
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  • Case 10: Mayer-Rokitansky-Küster-Hauser syndrome
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