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Introduction

MRKH ( Mayer–Rokitansky–Küster–Hauser)is a congenital condition in females due to which a a female is born without vagina and the uterus is not developed.Reproductive age of girls is generally marked by the onset of first menstrual cycle.But females suffering from MRKH syndrome do not have a developed reproductive system. It is a rare disease which occurs one in 4500 females.

There is a lot of psychological distress in females with MRKH syndrome. so it is essential for the patient and its family to go for a counselling before the treatment.

DISEASE NAME AND SYNONYMS:

This syndrome is subdivided into two types :
 type I (isolated) or Rokitansky sequence (OMIM 277000), and type II or MURCS association (MÜllerian duct aplasia, Renal dysplasia and Cervical Somite anomalies) (OMIM 601076).It is also referred as
CAUV (Congenital Absence of the Uterus and Vagina), MA (Müllerian Aplasia) or GRES (Genital Renal Ear Syndrome). It would thus be preferable that all entries (MRKH type I and type II, MURCS association, CAUV, MA and GRES) refer to the unique OMIM number 601076.onset of MRKH is mostly by single cell mutation.

DIAGNOSIS:

It is mostly done by ultrasound and magnetic resonance imaging. Ultrasound is mostly preferred due to easy availability , but it is not always effective in identifying underdeveloped mullerian structures and ovaries, which are usually located high in the pelvis, often at the level of the pelvic brim.

MRI is more useful than ultrasound but it is more expensive as compared to Ultrasound.

TREATMENT:

It includes both surgical and non surgical treatment.

SURGICAL TREATMENT:

  • Frank method
  • Vaginal dilators with progressive caliber
  • Ingram method
  • Vaginal dilators attached to a seat

NON SURGICAL TREATMENT:

  • Traction-based methods
  • Vechietti (laparotomic or laparoscopic)
  • Graft-based methods
  • Abbe-McIndoe (vaginal approach, various tissues used, skin graft most common)
  • Intestinal (combined vaginal and laparotomic or laparoscopic approach)
  • Davydov (combined vaginal and laparoscopic approach using peritoneum)


A Rare Case of Mayer-Rokitansky-Kuster-Hauser Syndrome Presenting with Acute Psychosis https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4866227/

Reference:

1:Laura londra, Farah S Chuong and Lisa Kolp( 2015 Nov 2)Mayer-Rokitansky-Kuster-Hauser syndrome: a review Retrieved from http://ncbi.nlm.nih.gov

2: Kamal Nath, Bhanita Boro, Subrata Naskar ( 2016 Apr 1 )A Rare Case of Mayer-Rokitansky-Kuster-Hauser Syndrome Presenting with Acute Psychosis Retrieved from http://ncbi.nlm.nih.gov

3: Keiko Watanabe, Yusuke Kobayashi, Kouji Banno, Yusuke Matoba, Haruko Kunitomi, Kanako Nakamura, Masataka Adachi, Kiyoko Umene, Iori Kisu, Eiichiro Tominaga, and Daisuke Aoki( 2017 Jun 21 ) Recent advances in the molecular mechanisms of Mayer-Rokitansky-Küster-Hauser syndrome
Retrieved from http://ncbi.nlm.nih.gov

4: Sussane Ladig and Peter Wieacker( 2018 Feb 21 ) Clinical and genetic aspects of Mayer–Rokitansky–Küster–Hauser syndrome
Retrieved from http://ncbi.nlm.nih.gov

5: Camborieux L; Programme de Recherches sur les Aplasies Müllériennes, Guerrier D ,Morcel K (  2007 Mar 14 )Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome Retrieved from http://ncbi.nlm.nih.gov

6: Both S, Kluivers k , Ten Kate-Booij M, Weijenborg P( 2018 Jul 11) Sexual response in women with Mayer-Rokitansky-Küster-Hauser syndrome with a nonsurgical neovagina Retrieved from http://ncbi.nlm.nih.gov