Mayer-Rokitansky-Küster-Hauser syndrome

Authors

  • Arga Kafi Perdana Kusuma Obstetrics and Gynecology Department, Faculty of Medicine, Andalas University, Dr. M. Djamil Central General Hospital Padang, West Sumatera, Indonesia
  • Bobby Indra Utama Division of Urogynecology and Reconstructure Aesthetic, Department of Obstetrics and Gynecology, RSUP Dr M. Djamil, Unversitas Andalas, Padang, Indonesia

DOI:

https://doi.org/10.25077/aoj.7.2.488-494.2023

Keywords:

MRKH syndrome, mullerian duct anomalies, vaginoplasty

Abstract

Mayer–Rokitansky–Küster–Hauser syndrome (MRKH) is a syndrome characterized by uterine, cervix, and the two third of upper vagina aplasia which is the cause of incomplete development of the Müllerian duct. Reported a case of woman 28 years old, patient has not menstruated until now. The phenotype of the patient appears to be female, and with normal stature. Breast, axilla dan pubic hair distribution, fatty in buttocks and thigh developed normally. Fallopian tubes, uterine and 2/3 upper part of vagina were not formed. On gynecological clinical examination, found vaginal introitus with a vaginal sonde was 2 cm. On abdominal ultrasound examination, the uterus was seen as a line, right ovary measuring 2.93 x 2.59 cm and left ovary measuring 2.52 x 2.28 cm. The patient then underwent a diagnostic laparoscopic procedure, and found both right and left ovaries within normal limits, intact tubes, but no uterus was visible. Patient are planned for vaginoplasty.

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Published

2023-07-30