IŞIK ÜSTÜNER, Levent KESKİN, Özge ÖZTÜRK, Eda ÖZYİĞİT, A. Filiz AVŞAR

Mayer-Rokitansky-küster-hauser (MRKH) sendromu

Mayer-Rokitansky-Küster-Hauser (MRKH) sendromu nadir görülen, Mülleriyen kanallarının embriyonik gelişimindeki duraksama sonucunda ortaya çıkan, uterus ve üst 2/3 vaginanın konjenital aplazisidir. Bu yazıda bir MRKH sendromlu olgu sunularak, güncel literatür eşliğinde tanı ve tedavi seçeneklerinin tartışılması amaçlandı. Kliniğimize primer amenore şikâyetiyle başvuran ve MRKH tip I sendromu tanısı alan 16 yaşındaki olgu sunulmuştur. MRKH sendromunda tanının doğruluğu; psikolojik destek sağlanması, normal seksüel fonksiyonların kazanılması için tedavi ve yardımla üreme tekniklerine yönelik danışmanlık verilmesi açısından önem arz etmektedir. Bu olgularda, eşlik eden diğer sistem anomalilerinin varlığına yönelik ayrıntılı bir sistemik değerlendirme ihmal edilmemelidir.

Anahtar Kelimeler:

Ergen, Kadın, Müller kanalı, Ultrasonografi, Amenore

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: A case report

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by interruption of the embryonic development of müllerian ducts causing congenital aplasia of the uterus and the upper part (2/3) of the vagina. Herein we will present a case with MRKH syndrome especially focusing on the diagnosis and treatment options in the light of the current literature. The case of a 16- year old female who presented with primary amenorrhea and diagnosed with MRKH type I syndrome is presented. Correct diagnosis in MRKH syndrome is essential so that targeted counseling can be initiated to achieve normal sexual functions, psychological help and if needed provide assisted reproductive technology options. Through systemic evaluation to detect other associated system anomalies should not be neglected in these cases.

Keywords:

Adolescent, Female, Mullerian Ducts, Ultrasonography, Amenorrhea,

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