MAYER-ROKITANSKY-KÜSTER-HAUSER SYNDROME WITH GONADAL DYSGENESIS: CASE REPORT

Dr. Narinder Singh, Dr. Shikha Sharma, *Dr. Aditi Ranaut and Dr. Anita Sharma

ABSTRACT

This is a report of 18 year old teenage girl with primary amenorrhoea and underdeveloped secondary sexual characters. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with gonadal dysgenesis, also referred as müllerian agenesis or aplasia is caused by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. It is characterized by congenital aplasia of the uterus and the upper two thirds of the vagina in women showing under development of secondary sexual characteristics and a normal 46, XX karyotype.1-2 The incidence of Mayer-Rokitansky-Kuster-Hauser syndrome was not clearly established, but studies indicate a variation of 1/4,000 and 1/5,000 live births of the female sex.[2]

Keywords: Mayer-Rokitansky-Küster-Hauser, Mullerian duct, Primary amenorrhoea.