Raisa Altaf Malik


ABSTRACT: Mayer-rokitansky-kuster-hauser syndrome is a rare congenital syndrome occurring in approximately 1out of 4500 to 1 out of 5000 women. (4,7) There is a defect in the embryonic development of para mesonephric ducts leading to aplasia or hypoplasia of the uterus and proximal 2/3 of vagina, despite normal hormonal status and female karyotype (3,4). It is divided into three types:Type 1 an isolated deformity of the uterus and cervixType 2 is associated with renal and ovarian disorders (atypical form)Type 3 shows anomalies including anal atresia, tracheoesophageal fistula, renal, cardiac and vertebral deformities (1,3,4,7). Typical MRI findings of this disorder can be helpful in the diagnosis of this syndrome. Therefore, we have presented here a case of an 8-year-old who was diagnosed with Mayer-rokitansky-kuster-hauser syndrome and her MRI pelvis findings along with other investigations. KEYWORDS: Mayer-Rokitansky-Küster-Hauser syndrome, uterine hypoplasia, congenital disorder, MRI findings.

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