Journal of Human Reproductive Science
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CASE REPORT Table of Contents   
Year : 2015  |  Volume : 8  |  Issue : 4  |  Page : 242-244
A rare case of Mayer-Rokitansky-Kuster-Hauser syndrome with multiple leiomyomas in hypoplastic uterus


1 Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, Maharashtra, India
2 Department of Obstetrics and Gynaecology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, Maharashtra, India

Correspondence Address:
Maithili Mandar Kulkarni
Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, General Hospital, Narhe, Pune - 411 041, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-1208.170418

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Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in development of mullerian ducts. Women with this syndrome are characterized by the presence of 46 XX karyotype, normal female secondary sex characters, normal ovarian functions, and underdeveloped vagina. The presence of leiomyoma in MRKH syndrome is very rare, and only few cases have been reported in the literature. Here, we report a case of MRKH syndrome with multiple leiomyomas originating from the rudimentary horn of uterus in 25 years married, phenotypically female patient.


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