Mayer-Rokitansky-Küster-Hauser syndrome


  • 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



MRKH syndrome, mullerian duct anomalies, vaginoplasty


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.


Sultan C, Lauber AB, Philibert P. Mayer- Rokitansky-Kuster-Hauser Syndrome: Recent Clinical and Genetic Findings. Journal Gynecological Endocrinology. 2009;25(1):8-11.

Morcel K, Camborieux L, Guerrier D. Mayer- Rokitansky-Kuster-Hauser (MRKH) Syndrome. Orphanet Journal of Rare Diseases. 2007;2(13):1- 9.

Wiyasa A, Mukminah A. Penatalaksanaan Sindroma Mayer Rokitansky Kuster Hauser (Sindroma MRKH) Dengan Sindroma Klippel Feil. Jurnal Kedokteran Brawijaya. 2004;20(2):92-95.

Ekici A, Strissel P, Oppelt P. HOXA10 and HOXA13 Sequence Variations in Human Female Genital Malformations Including Congenital Absence of The Uterus and Vagina. Gene 2013. 2013;518(2):267-272.

Guerrier D, Mouchel T, Pasquier L, Pellerin I. The Mayer-Rokitansky-Küster-Hauser syndrome (congenital absence of uterus and vagina) – phenotypic manifestations and genetic approaches. Journal of Negative Results in Biomedicine. 2006;5:1-1.

Oppelt P, Renner S, Kellermann A. Clinical Aspects of Mayer–Rokitansky–Kuester–Hauser Syndrome: Recommendations for Clinical Diagnosis and Staging. Human Reproduction. 2006;21(3):792-797.

Hendarto H.Gangguan Haid/ Perdarahan Uterus Abnormal.In:Anwar M,Baziad A,Prabowo RP, editors. Ilmu Kandungan. 3th ed. Jakarta: Bina Pustaka Sarwono Prawirohardjo;2011.p.161-183.

Paraton H. Embriologi Sistem Alat-alat Urogenital. In: Anwar M, Baziad A, Prabowo RP, editors. Ilmu Kandungan. 3th ed. Jakarta: Bina Pustaka Sarwono Prawirohardjo; 2011.p.33-46.

Dhaheri SA, Kaabi JA, Aswad S, Souid AK, Langer RD. Mullerian Agenesis: Presenting As An Isolated Rudimentary Uterus. Oman Medical Journal. 2014;29(5):1-2.

Gari A. McIndoe Neovagina in Patients With Mullerian Agenesis: A Sigle Center Experience. Pak Journal Med Science. 2017;33(1):236-240.

Yakasai I, Daneji S, Natasha A, Yunus E. Late Presentation of Mayer-Rokitansky-Kuster-Hauser Syndrome in The Tropics-Northern Nigeria. British Journal of Medicine and Medical Research. 2015;5(8):1077-1082.

Medical and Science. 2014;2(1):106-108.

Pizzo A, Laganà AS, Sturlese E, Retto G, Retto A, Dominici RD, et al. Mayer-Rokitansky- Kuster

Hauser Syndrome: Embryology, Genetics and Clinical and Surgical Treatment. Obstetrics and Gynecology. ;2013:1-10