The Coexistence of Gonadal Dysgenesis With Mayer-rokitansky-küster-hauser Syndrome, and Dandy-Walker Variant

Background: Gonadal dysgenesis, the most common cause of primary amenorrhea, is characterized by absent or underdeveloped ovaries. Although coexistence gonadal dysgenesis and Mayer-Rokitansky-Küster-Hauser (MRKH) has been reported, it still quite infrequent. To extent that authors searched, just one study reported association between Rokitansky sequence Dandy-Walker malformation. Clinical Presentation Intervention: We aimed to report a case with MRKH, variant. In this care report, 15-year-old girl amenorrhea secondary sexual properties. Her karyotype was 46, XX. The abdominopelvic MRI without contrast demonstrated bilateral ovarian agenesis no uterus cervix. Vagina normal in length. Brain consistent Conclusion: some affected chromosomal regions have identified, further genetic analyses should be performed elucidate probable these anomalies.