Mayer rokitansky küster hauser syndrome - type b

Case contributed by Utkarsh Kabra
Diagnosis certain

Presentation

Primary amenorrhea

Patient Data

Age: 25
Gender: Female

Non-visualization of the uterus, cervix, upper 2/3rd of the vagina. Only part of lower 3rd of the vagina is visualized.

Right ovary is normal in location. Small biloculated cyst is seen adjacent to it, possibly representing a para-ovarian cyst or exophytic ovarian cyst.

Left ovary is seen in left iliac fossa.

Left kidney is seen in the pelvis.

 

 

Case Discussion

 

Overall findings are suggestive of Mullerian agenesis, Mayer-Rokitansky-Küster-Hauser syndrome with an ectopic pelvic left kidney.  This makes it a type b subtype due to the presence of a renal anomaly.

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