Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Mostafa Mahmoud El Feky

Presentation

1ry Amenorrhea.

Patient Data

Age: 20
Gender: Female
MRI

MRI pelvis

The uterus is not visualized.

The right ovary is located in the inguinal canal.

The left ovary is intra-pelvic just deep to the internal inguinal ring.

Both ovary show otherwise normal MRI features.

MRI

MRI Abdomen

Solitary left kidney showing compensatory hypertrophy. Right renal agenesis.

Case Discussion

Mayer-Rokitansky-Küster-Hauser sundrome belongs to class I Müllerian duct anomalies (uterine agenesis). In this case; the uterus is missing and upper segment of vagina with presence of both ovaries, the right ovary is extra-pelvic at the inguinal region. This is associated with right renal agenesis. There is known uncommon association with renal agenesis and extrapelvic ovary that are demonstrated in this case. Primary amenorrhea is the commonest presentation as the presence of functioning ovaries and normal hormonal levels preserve the external genital development and puberty.

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Case information

rID: 55291
Case created: 28th Aug 2017
Last edited: 6th Sep 2017
Inclusion in quiz mode: Included

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