Inguinal uterus, fallopian tube, and ovary associated with adult Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE

To report a case of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) with a utero-ovarian inguinal hernia, and to present a review of the pertinent literature.

DESIGN

Case report and literature review.

SETTING

Hospital clinic.

PATIENT(S): A 31-year-old married woman.

INTERVENTION(S): Ultrasound, magnetic resonance imaging, intravenous pyelography, hormone analysis, peripheral karyotyping, ovarian biopsy, repositioning of the uterus, fallopian tube, and ovary, herniorrhaphy, and Williams vulvovaginoplasty.

MAIN OUTCOME MEASURE(S): Accuracy of diagnosis, preservation of ovarian function, hernia repair and creation of neovagina.

RESULT(S): Our patient represented an adult case of MRKH syndrome associated with renal abnormality and utero-ovarian inguinal hernia. Our English-language literature search for similar well-documented cases revealed only two. Our management consisted of thorough counseling, and successful one-step ovarian biopsy, repositioning of the uterus, fallopian tube, and ovary, herniorrhaphy, and Williams vulvovaginoplasty.

CONCLUSION(S): This is the third case of adult 46,XX MRKH with utero-ovarian inguinal hernia in the English literature. Genital repositioning is mandatory to avoid torsion and preserve ovarian function. The choice of the type of hernia repair and vaginoplasty or any other procedure should be individualized to the patient's medical status.