Characteristic features of Rokitansky -- Kuster Hauser syndrome are all of the following except :
Anovulation Mayer Rokitansky Kuser Syndrome (MRKH) is the complete failure in the development of the mullerian ducts resulting in absence of the fallopian tubes, uterus and most of vaginadeg (as of vagina is formed by Mullerian duct). Mayer Rokitansky-Kuster Hauser syndrome Karyotype = 46 XXdeg Phenotype = Female Associated abnormalities are : Renal ectopy and agenesis (Horse shoe shaped kidney)deg Skeletal abnormalitiesdeg (most common - scoliosis) Cardiac anomalies.deg Clinical features : Patient present between 15-18 years of age with primary amenorrhoea.deg (eugonadotropic amenorrhea). Secondary sexual characteristics are normaldeg as ovaries are normal (because of their separate embryonic origin, so ovulation is also normal.) P/V = Vagina is felt like a blind pouch and uterus is absent.deg Findings are confirmed by USG.deg Ideal method for demonstrating Uterine malformations is MRI.deg Management : Repair of vaginal agenesis done either by frank dilatation or vaginoplasty. Vaginoplast should onl be performed when the girls is just married or about to be marripri Frank Dilatation : This non-surgical procedure consists of a woman applying gradual pressure with progressively increasing dilators over the mullerian pit for 15 minutes twice a day. An indentation is created by the end of 3 to 6 month. Some have satisfactory intercourse, but in many, vaginal size is inadequate and they need a surgical procedure eventually. Surgical management : Vaginoplasty either by Mc Indoe operation Differential Diagnosis : Testicular feminization syndrome. Mayer Rokitansky-Kuster Hauser syndrome can be differentiated from testicular feminizing syndrome on the basis of :
Absent uterus
Absent vagina
Anovulation
46 -- XX