Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case
Mayer-Rokitansky -Küster -Hauser syndrome (MRKH) is a rare cause of primary amenorrhea. It is defined as congenital aplasia of the uterus and of the upper two thirds of the vagina in women with normal development of secondary sexual characteristics. Diagnosis is essentially based on magnetic resonan...
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| Veröffentlicht in: | The Pan African medical journal 2021-12, Vol.40, p.260 |
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| creator | Abdellaoui, Mohamed Fenni, Jamal El Edderai, Meryem |
| description | Mayer-Rokitansky -Küster -Hauser syndrome (MRKH) is a rare cause of primary amenorrhea. It is defined as congenital aplasia of the uterus and of the upper two thirds of the vagina in women with normal development of secondary sexual characteristics. Diagnosis is essentially based on magnetic resonance imaging (MRI). We here report the case of a 17-year-old girl presenting with primary amenorrhea with well developed secondary sexual characteristics. Laboratory tests revealed normal ovarian function as well as gonadotropic axis. Pelvic ultrasonography and magnetic resonance imaging revealed complete uterine agenesis of the uterus, the upper two-thirds of the vagina and left kidney, allowing to confirm the diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type II. This study highlights the importance of suspecting MRKH in young patients with well-developed sexual characteristics and any type of primary amenorrhea as well as of performing MRI in order to detect any specific sign of this disorder. |
| doi_str_mv | 10.11604/pamj.2021.40.260.29181 |
| format | Article |
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It is defined as congenital aplasia of the uterus and of the upper two thirds of the vagina in women with normal development of secondary sexual characteristics. Diagnosis is essentially based on magnetic resonance imaging (MRI). We here report the case of a 17-year-old girl presenting with primary amenorrhea with well developed secondary sexual characteristics. Laboratory tests revealed normal ovarian function as well as gonadotropic axis. Pelvic ultrasonography and magnetic resonance imaging revealed complete uterine agenesis of the uterus, the upper two-thirds of the vagina and left kidney, allowing to confirm the diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type II. 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This study highlights the importance of suspecting MRKH in young patients with well-developed sexual characteristics and any type of primary amenorrhea as well as of performing MRI in order to detect any specific sign of this disorder.</description><subject>46, XX Disorders of Sex Development - complications</subject><subject>46, XX Disorders of Sex Development - diagnosis</subject><subject>Adolescent</subject><subject>Amenorrhea - diagnosis</subject><subject>Amenorrhea - etiology</subject><subject>Congenital Abnormalities - diagnostic imaging</subject><subject>Female</subject><subject>Humans</subject><subject>Mullerian Ducts - abnormalities</subject><subject>Vagina - abnormalities</subject><issn>1937-8688</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1T0tOwzAUtJAQLYUrgC_g4Gc7rs0OVdAiipAQXVcvsS3ako_sZJG7seNiBCiL0YxmRk9vCLkGngForm5arPaZ4AIyxTOhR1gwcEKmYOWcGW3MhJyntOdcayP5GZnIXOSgcjUlm2ccfGSvzWHXYZ0OA3v6-kzdaK2wTz7SNNQuNpW_pUjLH4s2gbZxV2EcKFa-bmJ89zjGRdN3v6XkL8hpwI_kL488I5uH-7fFiq1flo-LuzVrgZuOgQYrzLwoVC6UCs4iRw1GYhmcy8F7WwSDo1KGc-kDLx1oBBvQKeGElDNy9Xe37YvKu-3xr-3_PvkNjhRTwg</recordid><startdate>20211223</startdate><enddate>20211223</enddate><creator>Abdellaoui, Mohamed</creator><creator>Fenni, Jamal El</creator><creator>Edderai, Meryem</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope></search><sort><creationdate>20211223</creationdate><title>Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case</title><author>Abdellaoui, Mohamed ; Fenni, Jamal El ; Edderai, Meryem</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p108t-1619287bb45244fd9a0a6183acfdd51ee9bf8ad5148003ef0cd16a19fad42d233</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>fre</language><creationdate>2021</creationdate><topic>46, XX Disorders of Sex Development - complications</topic><topic>46, XX Disorders of Sex Development - diagnosis</topic><topic>Adolescent</topic><topic>Amenorrhea - diagnosis</topic><topic>Amenorrhea - etiology</topic><topic>Congenital Abnormalities - diagnostic imaging</topic><topic>Female</topic><topic>Humans</topic><topic>Mullerian Ducts - abnormalities</topic><topic>Vagina - abnormalities</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Abdellaoui, Mohamed</creatorcontrib><creatorcontrib>Fenni, Jamal El</creatorcontrib><creatorcontrib>Edderai, Meryem</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><jtitle>The Pan African medical journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Abdellaoui, Mohamed</au><au>Fenni, Jamal El</au><au>Edderai, Meryem</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case</atitle><jtitle>The Pan African medical journal</jtitle><addtitle>Pan Afr Med J</addtitle><date>2021-12-23</date><risdate>2021</risdate><volume>40</volume><spage>260</spage><pages>260-</pages><eissn>1937-8688</eissn><abstract>Mayer-Rokitansky -Küster -Hauser syndrome (MRKH) is a rare cause of primary amenorrhea. 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| ispartof | The Pan African medical journal, 2021-12, Vol.40, p.260 |
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| source | MEDLINE; DOAJ Directory of Open Access Journals; PubMed Central Open Access; African Journals Online (Open Access); EZB-FREE-00999 freely available EZB journals; PubMed Central |
| subjects | 46, XX Disorders of Sex Development - complications 46, XX Disorders of Sex Development - diagnosis Adolescent Amenorrhea - diagnosis Amenorrhea - etiology Congenital Abnormalities - diagnostic imaging Female Humans Mullerian Ducts - abnormalities Vagina - abnormalities |
| title | Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case |
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