Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome; Case Report and Literature Review
Journal Title: IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) - Year 2017, Vol 16, Issue 3
Abstract
Introduction: Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome is a rare congenital absence of uterus and the upper two-thirds of the vagina in women who have normal development of secondary sexual characteristics and normal 46 XX karyotyping. Case report: We here report on a 17 years old girl with type I MRKH syndrome. Discussion: Laboratory studies should include, Chromosomal analysis to exclude karyotypic abnormalities of the X chromosome and androgen insensitivity syndrome (AIS). Normal levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) indicate appropriate ovarian function. On the other hand, Ultrasound and MRI plays a pivotal role in highlighting the exact anatomical details. Conclusion: absence of normal menstruation in young normal looking adolescent female should raise suspicion of MRKH syndrome. Management should be tailored to the individual needs and motivation of the patient.
Authors and Affiliations
Dr Mohammad Othman, Dr Ahmed Hassan Alghamdi
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