¨A síndrome Mayer-Rokitansky-Kuster-Hauser (MRKH) é caracterizada por aplasia congênita do útero e dois terços superiores da vagina em mulheres com cariótipo e características sexuais secundárias normais, apresentando incidência de 1:4.500 meninas. É a segunda causa mais frequente de amenorreia primária, após as disgenesias gonadais A MRKH pode ocorrer de forma isolada (tipo I) ou associada à malformação renal, vertebral e, em alguns casos, defeitos cardíacos ou auditivos, configurando a MRKH tipo II ou associação de MURCS, com incidência de 1:50.000 meninas. A ausência de dois terços proximais da vagina e ausência ou hipoplasia uterina ocorre em 96% dos casos, agenesia unilateral ou ectopia de um ou ambos os rins (88% dos casos); hidronefrose e rim em ferradura também podem ser encontrados. Já as anomalias de esqueleto, como defeito vertebral, envolvendo segmento cervical e/ou torácico, especialmente entre C5-T1, ocorrem em 80% dos casos, assim como assimetria, fusão de vértebras ou vértebra em cunha, escoliose e anomalia de Klippel-Feil (fusão de vértebras cervicais). A constituição cromossômica é 46,XX, embora tenham sido descritos casos de disgenesia gonadal XY ou monossomia X com os defeitos congênitos anteriormente descritos. A etiologia permanece desconhecida¨
■ Co-occurrence of Mayer-Rokitansky-Küster-Hauser syndrome and ovarian cancer: a case report and review of the literature
Roberta Villa, Jacopo Azzollini, Bernard Peissel, Siranoush Manoukian
Gynecol Oncol Rep. 2019 May; 28: 68–70
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431729/
■ A case of Mayer-Rokitansky-Küster-Hauser syndrome in a low-resource tertiary hospital in Douala, Cameroon
Thomas Obinchemti Egbe, Fidelia Mbi Kobenge, Metogo Mbengono Junette Arlette et al
SAGE Open Med Case Rep. 2019; 7: 2050313X19830817
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378465/
■ Diagnosis and management of ovarian tumor in Mayer-Rokitansky-Küster-Hauser syndrome
Yali Miao, Jirui Wen, Liwei Huang, Jiang Wu, Zhiwei Zhao
Biomed Res Int. 2018; 2018: 2369430
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5867664/
■ Genetic analysis of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) through ascertainment of a large cohort of families
Lacey S. Williams, Durkadin Demir Eksi, Yiping Shen et al
Fertil Steril. 2017 Jul; 108(1): 145–151.e2
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5770980/
■ Mayer-Rokitansky-Küster-Hauser syndrome associated with rectovestibular fistula
Charu Tiwari, Hemanshi Shah, Mukta Waghmare, Kiran Khedkar
Turk J Obstet Gynecol. 2017 Mar; 14(1): 70–73
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5558322/
■ Sexual life of women with Mayer-Rokitansky-Küster-Hauser syndrome after laparoscopic Vecchietti vaginoplasty
Zlatko Pastor, Jiří Froněk, Marta Nováčková, Roman Chmel
Sex Med. 2017 Jun; 5(2): e106–e113
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5440630/
■ Two sisters with Mayer-Rokitansky-Küster-Hauser syndrome and serous adenocarcinoma of the ovary
Sarah P. Huepenbecker, Laura Divine, Christina S. Chu, David G. Mutch
Gynecol Oncol Rep. 2017 Nov; 22: 13–15
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5596329/
■ Recent advances in the molecular mechanisms of Mayer-Rokitansky-Küster-Hauser syndrome
Keiko Watanabe, Yusuke Kobayashi, Kouji Banno et al
Biomed Rep. 2017 Aug; 7(2): 123–127
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5526143/
■ Treatment management during the adolescent transition period of girls and young women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS): a systematic literature review
Anke Wagner, Sara Yvonne Brucker, Esther Ueding et al
Orphanet J Rare Dis. 2016; 11: 15
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5112722/
■ An unusual cause of urinary incontinence: urethral coitus in a case of Mayer-Rokitansky-Küster-Hauser syndrome
Farzaneh Sharifiaghdas, Azar Daneshpajooh, Samira Sohbati, Mahboubeh Mirzaei
Investig Clin Urol. 2016 Sep; 57(5): 367–371
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5017564/
■ Unusual association of Turner syndrome and Mayer-Rokitansky-Küster-Hauser syndrome
Alpana Meena, Mradul Kumar Daga, Rashmi Dixit
BMJ Case Rep. 2016; 2016: bcr2015212634.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4885519/
■ Vertebral defect., anal atresia, cardiac defect., tracheoesophageal fistula/esophageal atresia, renal defect., a limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature
Thomas Bjørsum-Meyer, Morten Herlin, Niels Qvist, Michael B. Petersen
J Med Case Rep. 2016; 10: 374.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5178070/
■ Mayer-Rokitansky-Kuster-Hauser syndrome: a review
L Londra, F S Chuong, L Kolp
Int J Women Health 2015, vol 7: 965-870
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4636170/
■ Rim na síndrome de Mayer-Rokitansky-Kuster-Hauser
G L Breda, H G Ascenço, M Carvalho
Revista Médica da UFPR 2014, vol 1 (1)
https://revistas.ufpr.br/revmedicaufpr/article/view/40685/pdf_40685
■ Síndrome de Mayer-Rokitansky-Kuster-Hauser: uma revisão da literatura
D F Rezende, A V Rezende Filho, G N Oliveira et al
Revista Medicina e Saúde de Brasília 2013, vol 2 (2)
https://portalrevistas.ucb.br/index.php/rmsbr/article/view/4089/2672
■ Large leiomyoma in a woman with Mayer-Kokitansky-Kuster-Hauser syndrome
Kishan S Rawat, TBS Buxi, Anurag Yadav, Samarjit S ghuman, Shashi Dhawan
J Radiol Case Rep. 2013 Mar; 7(3): 39–46.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3661426/
■ Mayer-Rokitansky-Kuster-Hauser syndrome: embryology, genetics, and clinical and surgical treatment
Alfonsa Pizzo, Antonio Simone Laganà, Emanuele Sturlese et al
ISRN Obstet Gynecol. 2013; 2013: 628717
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3575620/
■ Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging
M A Hall-Craggs, C E Williams, S H Pattison et al
Radiology 2013
https://pubs.rsna.org/doi/full/10.1148/radiol.13130211
■ Forma atípica da síndrome de Mayer-Rokitansky-Kuster-Hauser com malformação renal e displasia cervicotorácica (associação de MURCS).
Borges, Mariana de Almeida Pinto et al.
Rev. Bras. Ginecol. Obstet., Mar 2012, vol.34, no.3, p.133-138
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032012000300008&lng=en&nrm=iso
■ Síndrome de Mayer-Rokitansky-Kuster-Hauser: relato de caso e revisão da literatura
Gutsche, Rodrigo Manfroi et al.
Radiol Bras, Jun 2011, vol.44, no.3, p.192-194
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-39842011000300014&lng=en&nrm=iso
■ Presurgical management of dysmenorrhea and endomtriosis in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome
J E Elliot, H Abduliabar, M Morris
Fertility and Sterity 2011, vol 96 (2)
https://www.fertstert.org/article/S0015-0282(11)00925-3/fulltext
■ Neovaginoplastia com membrana amniótica na síndrome de Mayer-Rokitansky-Küster-Hauser
Carvalho, Bruno Ramalho de et al.
Rev. Bras. Ginecol. Obstet., Dez 2007, vol.29, no.12, p.619-624
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032007001200004&lng=en&nrm=iso
■ Dr Paulo Fernando Leite
Cardiologia/Prevenção Cardiovascular
Consultório: Rua Padre Rolim 815/sala 815
Tel: 33245518 (Consulta Particular/Unimed)
– Centro Médico Unimed BH/Contagem/Pedro I
Belo Horizonte/MG/Brasil
CRMMG: 7026
Email: pfleite1873@gmail.com