Mayer-Rokitansky-Küster-Hauser syndrome type II: A rare case report

Autor: Kaouthar Sfar, Kaoutar Imrani, Fatima Chait, Sara Essetti, Kaoutar Maslouhi, Nabil Moatassim Billah, Ittimade Nassar
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: SAGE Open Medical Case Reports, Vol 12 (2024)
Druh dokumentu: article
ISSN: 2050-313X
2050313X
DOI: 10.1177/2050313X241265047
Popis: MRKH syndrome, or Mayer-Rokitansky-Küster-Hauser syndrome, a rare congenital disease, manifests as a complete or partial aplasia of the uterus and the vagina’s upper two-thirds with normal external genitalia and functioning ovaries. Mayer-Rokitansky-Küster-Hauser syndrome can occur in isolation (type I) or in conjunction with other congenital extragenital deformities affecting the kidneys, skeleton, heart, eyes, or auditory system (type II). The diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome typically relies on imaging studies, with transabdominal ultrasonography serving as the primary modality. However, magnetic resonance imaging is considered the gold standard for detailed assessment of internal genital anatomy. We present the case of an 18-year-old woman without any notable medical history who exhibited primary amenorrhea. Mayer-Rokitansky-Küster-Hauser syndrome type II was suspected on pelvic ultrasound and subsequently confirmed via magnetic resonance imaging. The patient was provided with psychological assistance and planned for vaginoplasty.
Databáze: Directory of Open Access Journals