Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Utkarsh Kabra
Diagnosis almost certain

Presentation

Primary amenorrhea

Patient Data

Age: 18 years
Gender: Female

Non-visualization of uterus, cervix, upper 2/3 rd of vagina. Possibly only part of lower 3 rd of vagina is visualized.
Bilateral ovaries are seen higher up in bilateral iliac fossa. Right ovary measures approx. 29 x 19mm and left ovary measures approx. 26 x 18mm.

Left kidney is seen in pelvis on left side. No obvious dilatation of pelvicalyceal system is seen.

Case Discussion

Above findings are suggestive of Mullerian agenesis, Mayer-Rokitansky- Küster-Hauser syndrome (Type B)

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