Mayer-Rokitansky-Küster-Hauser Syndrome: Admission With An Atypical Chief Complaint

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital abnormality with absence of uterus, cervix and vagina, together with normal sex development and external genitalia. It’s a rare case of girls seen in every 4,000 to 10,000 births. The syndrome, usually identified during the investigation of primary amenorrhea in puberty, may have additional abnormalities, that also can cause a variety of clinical complaints. However, in the literature there is no patients diagnosed as a result of admission with complaints of micturition. Urinary incontinence is common among women but while they do not seek for medical help and while they think that it’s a natural process though the situation affects their quality of life, may delay the diagnosis of important factors that may lie beneath. For the correct identification of these patients in clinical practice all female patients admitted to the clinic for any reason incontinence should be noted by physicians.

Keywords

• Congenital anomaly,primary amenorrhea,incontinence

Mayer-Rokitansky-Küster-Hauser Sendromu: Atipik Bir Şikayet ile Başvuru

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) sendromu, normal sekonder seks karakter ve dış genital gelişimi ile birlikte uterus, serviks ve vajina yokluğu ile karakterize konjenital bir anomalidir. Dişi cinsiyette 4000 ile 10.000 doğumda bir görülen nadir bir sendromdur. Genellikle puberte döneminde primer amenore etiyolojisinin araştırılması sırasında tanımlanan sendrom, çeşitli klinik şikayetlere de neden olabilir. Ancak literatürde, işeme şikayetleriyle kliniğe başvurarak teşhis edilen vaka bildirilmemiştir. Üriner inkontinans kadınlarda yaygındır, ancak yaşam kalitesini etkilese de bu durumun doğal bir süreç olduğu düşünülerek tıbbi yardım arayışına girilmez ise altta yatan önemli faktörlerin tanısında gecikme yaşanabilir. Klinik pratikte bu hastaların doğru tanınması için, herhangi bir nedenle kliniğe başvuran hastalarda inkontinans varlığı hekimler tarafından mutlaka sorgulanmalıdır.

Keywords

• Konjenital anomali,primer amenore,inkontinan

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