Current Pediatric Research

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Research Article - Current Pediatric Research (2021) Volume 25, Issue 11

Mayer-Rokitansky-Kuster-Hauser Syndrome: Typical and atypical MRI findings

Background: The Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is a reproductive tract congenital malformation, characterized by the absence or hypo plastic uterus and upper two thirds of the vagina in an otherwise normal female. Aims: This study is to analyze the MRI findings in group of MRKHS patients of both typical and atypical type. Methods: A cross sectional study, held in Mosul city, over a period of a year from October 2020 to October 2021, Female patients who was having MRI (Magnetic Resonance Imaging), done to them as a part of radio diagnosis workup of primary amenorrhea, were included in the study 1.5 tesla MRI scanner was used. Results: The studied patients are 23 females, age range (16 years-23 years), average 17.4 years, Std. Deviation 2.03 years. MRKHS type I is noted in (16/23) patients (69.5%) and MRKHS type II in (7/23) patients (30.4%), no MURCS subtype was identified. All the patients (100%) have absent uterus, cervix and upper vagina; small uterine buds are preserved in 17 patients (73.9%). Distended uterine buds with ipsilateral tubal dilatation is noted in 6 cases (26%) (Unilateral in 13% and bilateral in 13%). The lower vagina and external genetalia are not affected by the anomalies, however the lower vagina are very small (less than 1cm), in 4 cases (17%). The ovaries and the fallopian tubes appear to be unaffected by the malformation in 18 cases (78.2%), the extragenital associated findings were include renal anomalies in 3 cases (13%), in a form of ectopic kidney and renal agenesis, simple ovarian cysts were evident in 6 cases (26%), no detectable skeletal congenital abnormalities. Conclusion: MRI plays an important role in the evaluation of primary amenorrhea; it can detect congenital abnormalities of mullarian duct as those caused by MRKHS. It also can help in the diagnosis of extragenital renal or skeletal abnormalities, thus MRI exam should include the abdomen as well.

Author(s): Mohamed Muyaser* Naif Rasha Nadeem Ahmed, Abeer Wali Ahmed

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