Presentation
Primary amenorrhea to rule out pelvic abnormality.
Patient Data
The uterus, cervix and upper two-thirds of the vagina are not seen, in keeping with class I Müllerian duct anomaly.
Each ovary is seen in ipsilateral iliac fossa region, mildly extending to inguinal canals with mild right canal of Nuck hydrocele.
Right ovarian cystic lesion, with thin imperceptible wall, no mural nodules, in keeping with follicular cyst.
Bilateral fused pelvic renal ectopia (pancake kidney) opposite to L4 down to S1.
Mild pelvic free fluid is seen.
Case Discussion
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea after gonadal dysgenesis/Turner syndrome.
Two types of MRHK syndrome have been identified:
typical (type I): isolated agenesis of uterus and vagina
atypical (type II) or MURCS: Müllerian duct aplasia, renal aplasia/ectopia, cervical vertebral and thoracic abnormalities
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