Korean J Radiol.  2013 Apr;14(2):233-239. 10.3348/kjr.2013.14.2.233.

Magnetic Resonance Evaluation of Mullerian Remnants in Mayer-Rokitansky-Kuster-Hauser Syndrome

• Yoo RE
• Cho JY
• Kim SY
• Kim SH

• KMID: 1482782
• DOI: http://doi.org/10.3348/kjr.2013.14.2.233

Abstract

OBJECTIVE
To analyze magnetic resonance imaging (MRI) findings of Mullerian remnants in young females clinically suspected of Mayer-Rokitansky-Kuster-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 Mullerian 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 Mullerian 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.

Keyword

Amenorrhea; Magnetic resonance imaging; Mayer-Rokitansky-Kuster-Hauser syndrome; Mullerian; Remnant

MeSH Terms

Abnormalities, Multiple/*pathology
Adolescent
Adult
Female
Humans
Kidney/abnormalities/pathology
Magnetic Resonance Imaging/*methods
Middle Aged
Mullerian Ducts/abnormalities/pathology
Retrospective Studies
Somites/abnormalities/pathology
Spine/abnormalities/pathology
Uterus/abnormalities/pathology
Vagina/abnormalities/pathology

Figure

• 

  Fig. 1 Representative case of MRKH syndrome in 34-year-old woman. A. On transverse T2WI, bilateral soft tissues suspected as uterine buds (thick arrows) are found on each side of lateral pelvic walls. B-E. Serial coronal T2WIs show fibrous band-like structures (B-D: thick arrows) connecting bilateral uterine buds (B, C: dotted arrows) and converging at midline soft tissue (E: arrowhead). F. On sagittal T2WI, instead of normal uterine structure, triangular soft tissue (thick arrow) is found at midline. Lower one-third of vagina appears normal (thin arrows). G-I. Bilateral ovaries (G, H: thick arrows) are also found in pelvic cavity and appear normal on transverse T2WI. Coronal T2WI shows low signal intensity band (I: thin arrow) between left ovary and uterine bud (right side not shown). MRKH = Mayer-Rokitansky-Küster-Hauser, T2WI = T2-weighted image

• 

  Fig. 2 MRKH syndrome in 18-year-old woman. A, B. Bilateral uterine buds (A, B: thick arrows) are connected only with fibrous band-like structures (B: arrowheads) on transverse T2WI. Prominent cavitation (B: thin arrow) is noted in right uterine bud. C. Coronal T2WI shows bilateral normal ovaries (thick arrows) abutting uterine buds (thin arrows) at superior aspect. MRKH = Mayer-Rokitansky-Küster-Hauser, T2WI = T2-weighted image

• 

  Fig. 3 Schematic drawings of typical Müllerian remnants. A. Fibrous band-like structures connecting bilateral uterine buds converge at midline triangular soft tissue. Cavitation may be present in uterine buds (shown as triangular area within left bud). Ovaries abutting uterine buds are also shown. B. Sagittal T2-weighted images shows triangular soft tissue lying above bladder dome. Soft tissue is continuous with vagina showing atrophy in upper two-thirds.

Reference

1. Giusti S, Fruzzetti E, Perini D, Fruzzetti F, Giusti P, Bartolozzi C. Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging. 2011. 36:753–755.

2. Lamarca M, Navarro R, Ballesteros ME, García-Aguirre S, Conte MP, Duque JA. Leiomyomas in both uterine remnants in a woman with the Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2009. 91:931.e13–931.e15.

3. Pompili G, Munari A, Franceschelli G, Flor N, Meroni R, Frontino G, et al. Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 2009. 114:811–826.

4. Reinhold C, Hricak H, Forstner R, Ascher SM, Bret PM, Meyer WR, et al. Primary amenorrhea: evaluation with MR imaging. Radiology. 1997. 203:383–390.

5. Zhou JH, Sun J, Yang CB, Xie ZW, Shao WQ, Jin HM. Long-term outcomes of transvestibular vaginoplasty with pelvic peritoneum in 182 patients with Rokitansky's syndrome. Fertil Steril. 2010. 94:2281–2285.

6. Jurkiewicz B, Matuszewski L, Cisłak R, Rybak D. Rokitansky-Kustner-Hauser syndrome - a case report. Eur J Pediatr Surg. 2006. 16:135–137.

7. Chandiramani M, Gardiner CA, Padfield CJ, Ikhena SE. Mayer - Rokitansky - Kuster - Hauser syndrome. J Obstet Gynaecol. 2006. 26:603–606.

8. Deligeoroglou E, Kontoravdis A, Makrakis E, Christopoulos P, Kountouris A, Creatsas G. Development of leiomyomas on the uterine remnants of two women with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2004. 81:1385–1387.

9. Govindarajan M, Rajan RS, Kalyanpur A, Ravikumar . Magnetic resonance imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. J Hum Reprod Sci. 2008. 1:83–85.

10. Jadoul P, Pirard C, Squifflet J, Smets M, Donnez J. Pelvic mass in a woman with Mayer-Rokitansky-Kuster-Hauser syndrome. Fertil Steril. 2004. 81:203–204.

11. Lanowska M, Favero G, Schneider A, Köhler C. Laparoscopy for differential diagnosis of a pelvic mass in a patient with Mayer-Rokitanski-Küster-Hauser (MRKH) syndrome. Fertil Steril. 2009. 91:931.e17–931.e18.

12. Papa G, Andreotti M, Giannubilo SR, Cesari R, Ceré I, Tranquilli AL. Case report and surgical solution for a voluminous uterine leiomyoma in a woman with complicated Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2008. 90:2014.e5–2014.e6.

13. Sönmezer M, Atabekoglu C, Dökmeci F. Laparoscopic excision of symmetric uterine remnants in a patient with mayer-rokitansky-küster-hauser syndrome. J Am Assoc Gynecol Laparosc. 2003. 10:409–411.

14. Chandler TM, Machan LS, Cooperberg PL, Harris AC, Chang SD. Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol. 2009. 82:1034–1042.

15. Reichman DE, Laufer MR. Mayer-Rokitansky-Küster-Hauser syndrome: fertility counseling and treatment. Fertil Steril. 2010. 94:1941–1943.