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Mayer Rokitansky Kuster Hauser Syndrome: A Case Study of Mullerian Agenesis in a Patient


S. Ghazali
H. Umaru-Sule
A.G. Adesiyun
N. Ameh
H.U. Sulayman
S. Avidime
F. Bakari
U. Mohammed
B. Olatubosun

Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is an uncommon congenital malformation characterised by agenesis or hypoplasia of  the vagina and uterus. It occurs due to failure of development of the Mullerian duct. Its incidence is 1 per 4500 female births. Mostly,  girls present with primary amenorrhoea. The diagnosis of MRKH has a significant impact on the lives of women especially in the way they  perceive themselves as healthy and functioning women. We present a case of MayerRokitansky-Kuster-Hauser (MRKH) syndrome. A  23year old single nullipara presented with failure to initiate menstruation since puberty. She has normal secondary sexual characteristics  however she had a completely sealed portio-vaginalis and absent vagina. Abdominopelvic ultrasound showed a hypoplastic uterus and  her karyotype is XX. A diagnostic laparoscopy revealed rudimentary fallopian tubes, hypoplastic uterus, absent cervix, a single ovary in  the midline, with a grossly normal urinary bladder. The experience of failure to initiate menstruation in individuals with MRKH syndrome   negatively affects their quality of life. Treatment includes psychological counselling and vaginoplasty.


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eISSN: 2635-3490
print ISSN: 2476-8316