Tawam Hospital in affiliation with Johns Hopkins Medical Center, Al Ain, United Arab Emirates.
Fertil Steril. 2011 Mar 1;95(3):1119.e1-4. doi: 10.1016/j.fertnstert.2010.09.065. Epub 2010 Oct 30.
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.
Case report and literature review.
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.
报告一例 Mayer-Rokitansky-Küster-Hauser 综合征（MRKH）合并子宫-卵巢腹股沟疝病例，并对相关文献进行回顾。
病例报告和文献回顾。
医院诊所。
一名 31 岁已婚女性。
超声、磁共振成像、静脉肾盂造影、激素分析、外周染色体核型分析、卵巢活检、子宫、输卵管和卵巢复位、疝修补和 Williams 阴道成形术。
诊断准确性、卵巢功能保留、疝修复和新阴道的建立。
我们的患者为成人 MRKH 综合征合并肾脏异常和子宫-卵巢腹股沟疝。我们对类似的有详细记录的病例进行了英文文献检索，仅发现两例。我们的治疗方案包括彻底的咨询，以及成功的一步卵巢活检、子宫、输卵管和卵巢复位、疝修补和 Williams 阴道成形术。
这是第三例成人 46,XX MRKH 合并子宫-卵巢腹股沟疝的英文文献病例。为避免扭转和保留卵巢功能，必须进行生殖器复位。疝修补和阴道成形术或任何其他手术类型的选择应根据患者的身体状况个体化。
