Mayer Rokitansky Küster Hauser Syndrome as a Cause of Infertility and Primary Amenorrhoea: A Case Report  

作　　者：Tidiani Traoré Hamidou Toungara Famakan Kané Kassoum Sidibé Oumar S. Coulibaly Seydou Z. Dao Mamadou Touré Seydou Traoré Brahima Donigolo Babou Traoré Adama Coulibaly Abdourahamane Diarisso Birama Traoré Alima Sidibé Apha Sanogo Amadou Bocoum Seydou Fané Youssouf Traoré Ibrahim Teguété Niani Mounkoro Amadou Dolo Tidiani Traoré;Hamidou Toungara;Famakan Kané;Kassoum Sidibé;Oumar S. Coulibaly;Seydou Z. Dao;Mamadou Touré;Seydou Traoré;Brahima Donigolo;Babou Traoré;Adama Coulibaly;Abdourahamane Diarisso;Birama Traoré;Alima Sidibé;Apha Sanogo;Amadou Bocoum;Seydou Fané;Youssouf Traoré;Ibrahim Teguété;Niani Mounkoro;Amadou Dolo(Ministry of Health in Bamako, Nianakoro Fomba Hospital, Segou, Mali;Regional Health Department of Segou, District Hospital, Bla, Mali;Regional Health Department of Bamako, Reference Health Center, Commune II, Bamako, Mali;Regional Health Department of Koutiala, Reference Health Center, Koutiala, Mali;Regional Health Department of Segou, District Hospital, San, Mali;Ministry of Health in Bamako, Fousseyni Daou Hospital, Kayes, Mali;Ministry of Health in Bamako, Gabriel Toure University Hospital Center, Bamako, Mali)

机构地区：[1]Ministry of Health in Bamako, Nianakoro Fomba Hospital, Segou, Mali [2]Regional Health Department of Segou, District Hospital, Bla, Mali [3]Regional Health Department of Bamako, Reference Health Center, Commune II, Bamako, Mali [4]Regional Health Department of Koutiala, Reference Health Center, Koutiala, Mali [5]Regional Health Department of Segou, District Hospital, San, Mali [6]Ministry of Health in Bamako, Fousseyni Daou Hospital, Kayes, Mali [7]Ministry of Health in Bamako, Gabriel Toure University Hospital Center, Bamako, Mali

出　　处：《Open Journal of Obstetrics and Gynecology》2024年第12期1801-1806,共6页妇产科期刊(英文)

摘　　要：Mayer Rokitansky Küster Hauser (MRKH) syndrome is a rare congenital disorder that occurs during foetal development. MRKH is characterised by agenesis of the uterus and part of the vagina. Its exact aetiology is still unknown due to the complexity of the genetic pathways involved in the embryogenetic development of the Müllerian ducts. Although these women have a female phenotype and functional ovaries, they do not have menstrual cycles. We report the rare case of a 23-year-old married woman who consulted us for primary amenorrhoea and desire for pregnancy. On assessment and investigation, it was confirmed that the patient had no uterus and a poorly developed vagina. However, she had normal functioning ovaries, and no abnormalities in other organs were detected. A multidisciplinary team provided advice on the nature of the anomaly, its implications for sex life, infertility and possible child adoption alternatives.Mayer Rokitansky Küster Hauser (MRKH) syndrome is a rare congenital disorder that occurs during foetal development. MRKH is characterised by agenesis of the uterus and part of the vagina. Its exact aetiology is still unknown due to the complexity of the genetic pathways involved in the embryogenetic development of the Müllerian ducts. Although these women have a female phenotype and functional ovaries, they do not have menstrual cycles. We report the rare case of a 23-year-old married woman who consulted us for primary amenorrhoea and desire for pregnancy. On assessment and investigation, it was confirmed that the patient had no uterus and a poorly developed vagina. However, she had normal functioning ovaries, and no abnormalities in other organs were detected. A multidisciplinary team provided advice on the nature of the anomaly, its implications for sex life, infertility and possible child adoption alternatives.

关 键 词：Rokitansky Syndrome Müllerian Ducts Uterine Agenesis 

分 类 号：R73[医药卫生—肿瘤]