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

Yıl 2018, Cilt: 2 Sayı: 2, 192 - 196, 31.08.2018

Reha Girgin

Öz

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.

Anahtar Kelimeler

Congenital anomaly, primary amenorrhea, incontinence

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

Öz

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.

Anahtar Kelimeler

Konjenital anomali, primer amenore, inkontinan

Kaynakça

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