Mayer–Rokitansky–Kuster–Hauser syndrome (MRKH) and Ovarian Cyst : A Rare Case


  • Susan Meuthia Obstetrics and Gynecology Department, Faculty of Medicine, Andalas University, Dr. M. Djamil Central General Hospital Padang, West Sumatera, Indonesia
  • Yulias Margaretta Sari Division of Urogynecology and Reconstructure Aesthetic, Department of Obstetrics and Gynecology, RSUP Dr M. Djamil, Unversitas Andalas, Padang, Indonesia



Mayer–Rokitansky–Kuster–Hauser syndrome, primary amenorrhea, ovarian cyst


Background: In the most recent publications on Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, the uterine remnants and ovaries in patients may develop uterine remnant leiomyoma, adenomyosis, or ovarian tumor, and this can lead to problems in differential diagnosis. Here we summarize the diagnosis methods and available interventions for ovarian tumor in MRKH syndrome, with emphasis on the relevant clinical findings and illustrative relevant case. According to the clinical findings and illustrative relevant case, with the help of imaging techniques, ovarian tumors can be detected in the pelvis in patients with MRKH syndrome and evaluated in terms of size. Laparoscopy could further differentiate ovarian tumors into different pathological types. In addition, laparoscopic surgery not only is helpful for the diagnosis of MRKH combined ovarian tumor, but also has a good treatment role for excising ovarian tumor at the same time. Moreover, laparoscopic removals of ovarian tumor can be considered as a safe and reliable treatment for conservative management.

Case report: 25-year-old nulliparous woman with female phenotype and normal secondary sex development presented with complaints of primary amenorrhea and difficulty in sexual intercourse. Physical examination revealed that there were no vaginal and uterus structure. A transvaginal ultrasound examination showed absent of uterine . normal right ovariesand cystic mass in left adnexa . Laparoscopy cystectomy and amniograft vaginoplasty  was performed in this patient. Histopatology result showed Cystadenoma srous ovarii with borderline serous ovarii.

Conclusion: Rarely ovarian cyst was associated with MRKH . Although malformation are associated with MRKH syndrome. Association  beetween MRKH syndrome and ovarian cyst can be found 


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