Ovarian torsion - Mayer-Rokitansky-Küster-Hauser Syndrome

Case contributed by Dr Eid Kakish

Presentation

Sudden onset acute lower abdominal and left iliac fossa pain, gradually increasing in severity over the past week. Patient presented with primary amenorrhea at the age of 13.

Patient Data

Age: 25 years
Gender: Female

Massively enlarged left ovary, measuring around 280 cm³ in volume, located in the midline of the pelvis, associated with extensive stromal edema, vascular pedicle twisting and a large amount of pelvic free fluid. Findings are consistent with left ovarian torsion.

Two well-defined rim-enhancing relatively large cystic lesions are seen within the left ovary, measuring around 4.2 x 3.5 cm and 3.7 x 3.5 cm in maximal dimensions, containing irregular septations and fluid-blood levels, consistent with hemorrhagic left ovarian cysts. Multiple other tiny peripherally arranged left ovarian follicles of variable signal intensity are noted.

The uterus is absent. Instead, two rudimentary uterine horns are identified just caudal to each ovary, larger on the right side, associated with agenesis of the uterine cervix. Only the lower third of the vaginal canal can be identified, best seen on Axial T2FS and the enhanced study. The left renal bed is empty, with evidence of an ectopic left pelvic kidney. 

An orthotopic right ovary is identified. 

Ultrasound was performed in retrospect, confirming the findings described on MRI. 

No internal venous flow was identified on color Doppler.

The last image stack nicely demonstrates the whirlpool sign seen in ovarian torsion, which is characteristic for a twisted ovarian pedicle. 

Annotated image

Annotated MRI

  1. red asterisk in the lower vaginal canal
  2. yellow circle around the lower vaginal canal on axial T2FS
  3. arrow pointing to the left ovarian pedicle
  4. arrows following a twisted left ovarian pedicle
  5. rudimentary uteri (red circles) caudal to both ovaries (yellow asterisks)

Case Discussion

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) or Müllerian agenesis is a Class I Mullerian duct anomaly characterized by the absence of the paramesonephric (Müllerian) ducts, resulting in agenesis of the uterus, cervix, fallopian tubes and upper 2/3 of the vagina. Patients usually also have other anomalies of the genitourinary tract. 

Patients with MRKH syndrome have fully developed secondary sexual characteristics, presenting in adolescence with primary amenorrhea. 

A few studies have described an association between Müllerian duct agenesis and an increased risk of Ovarian torsion, which is believed to be caused by increased ovarian mobility secondary to abnormal embryonic development, resulting in uterine agenesis and absence of the utero-ovarian ligament.

MRI is an effective tool in the diagnosis of MRKH syndrome, with sensitivity and specificity reaching 100%. Findings seen on MRI are coherent with those of laparoscopy in assessing pelvic organs. 

It is commonly believed that patients with MRKH syndrome have a completely absent uterus. However, rudimentary uterine buds are usually seen in most of the cases. 

The normal uterine layer morphology encountered on MRI is variably seen in the rudimentary uterine buds, ranging from 1-3 layers.

Various studies found a fixed caudal rudimentary uterus in relation to the ovary. This relationship was deemed very useful in identifying small uterine buds in cases with ectopic ovaries, which can be present in up to 40% of patients. 

This patient was diagnosed with MRKH syndrome at the age of 13 following a presentation of primary amenorrhea. The diagnosis was confirmed on laparoscopy.

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Case information

rID: 86541
Published: 6th Feb 2021
Last edited: 9th Mar 2021
System: Gynaecology
Inclusion in quiz mode: Included
Institution: MedRay - Jordan