Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome

Korean J Radiol. 2013 Mar-Apr;14(2):233-9. doi: 10.3348/kjr.2013.14.2.233. Epub 2013 Feb 22.

Abstract

Objective: To analyze magnetic resonance imaging (MRI) findings of Müllerian remnants in young females clinically suspected of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome in a primary amenorrhea workup.

Materials and methods: Fifteen young females underwent multiplanar T2- and transverse T1-weighted MRI at either a 1.5T or 3.0T MR imager. Two gynecologic radiologists reached consensus decisions for the evaluation of Müllerian remnants, vagina, ovaries, and associated findings.

Results: All cases had bilateral uterine buds in the pelvic cavity, with unilateral cavitation in two cases. The buds had an average long-axis diameter of 2.64 ± 0.65 cm. In all cases, bilateral buds were connected with fibrous band-like structures. In 13 cases, the band-like structures converged at the midline or a paramedian triangular soft tissue lying above the bladder dome. The lower one-third of the vagina was identified in 14 cases. Fourteen cases showed bilateral normal ovaries near the uterine buds. One unilateral pelvic kidney, one unilateral renal agenesis, one mild scoliosis, and three lumbar sacralization cases were found as associated findings.

Conclusion: Typical Müllerian remnants in MRKH syndrome consist of bilateral uterine buds connected by the fibrous band-like structures, which converge at the midline triangular soft tissue lying above the bladder dome.

Keywords: Amenorrhea; Magnetic resonance imaging; Mayer-Rokitansky-Küster-Hauser syndrome; Müllerian; Remnant.

MeSH terms

  • 46, XX Disorders of Sex Development
  • Abnormalities, Multiple / pathology*
  • Adolescent
  • Adult
  • Congenital Abnormalities
  • Female
  • Humans
  • Kidney / abnormalities
  • Kidney / pathology
  • Magnetic Resonance Imaging / methods*
  • Middle Aged
  • Mullerian Ducts / abnormalities
  • Mullerian Ducts / pathology
  • Retrospective Studies
  • Somites / abnormalities
  • Somites / pathology
  • Spine / abnormalities
  • Spine / pathology
  • Uterus / abnormalities
  • Uterus / pathology
  • Vagina / abnormalities
  • Vagina / pathology

Supplementary concepts

  • Mullerian aplasia