Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Mohamed Mahmoud Elthokapy

Presentation

Primary amenorrhea. Ultrasound study revealed normal ovaries and no uterus as well as single ectopic right kidney.

Patient Data

Age: 18 years
Gender: Female

There is absence of the uterus and upper two-thirds of the vagina. Normal appearances of the lower third of the vagina and both ovaries.

Single ectopic pelvic right kidney with duplicated renal collecting system versus crossed fused renal ectopia (pancake kidney).

The urinary bladder and rectum are unremarkable.

No pelvic free fluid or lymphadenopathy.

Case Discussion

Imaging features are impressive of Mayer-Rokitansky-Küster-Hauser syndrome type 1. MRI, in this case, clarifies that agenesis of the uterus with renal anomalies is the atypical and rare form of MRKH syndrome and differentiates from other differentials including MRKH type 2 and hematocolpometra due to an imperforate hymen. 

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