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vaginal agenesis (a-jen-uh-sis) is a rare disorder that occurs when the vagina doesnt develop, and the womb (uterus) may only develop partially or not at all. This condition is present before birth, and may also be associated with kidney, heart or skeletal abnormalities. Müllerian agenesis, also known as mayerrokitanskyküsterhauser syndrome (mrkh) or vaginal agenesis, is a congenital malformation characterized by a failure of the müllerian duct to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Müllerian agenesis (including absence of the uterus, cervix andor vagina) is the cause in 15 of. Vaginal agenesis is a birth defect that affects few women (1 out of 5,000). But unless it is fixed, it can make sex and having a baby impossible. Some girls may have a shorter vagina, a remnant of one, or lack one altogether. vaginal atresia is a congenital defect resulting in uterovaginal outflow tract obstruction. It occurs when the caudal portion of the vagina, contributed by the urogenital sinus, fails to form. This caudal portion of the vagina is replaced with fibrous tissue. Vagina atresia and agenesis are congenital anomalies of the female genitourinary tract. This study of 90 cases of vaginal agenesia showed the following results 50 of the patients sought medical advice when between 16 and 20 years of age 71. 1 of the patients were not married amenorrhea was present in 100 of the cases, with hypogastric pains in 20 the uterus was not palpable clinically in 77. Jorge enrique aristizbal-duque, joan camilo piedrahta-meja, seguridad y eficacia de la vaginoplastia de vecchietti en agenesia vaginal estudio de cohorte, en medelln, colombia, 2007 a 2012, revista colombiana de obstetricia y ginecologa, 10. Vaginal atresia refers to a spectrum of anomalies comprising of failure to form a part or all of the vagina. Epidemiology it is considered the second most common cause of primary amenorrhea. Lower vaginal atresia is a type of vagina atresia where the the lower 3rd of the vagina fails to develop. Clinical presentation patients may present with primary amenorrhea with progressive cyclical abdominal pain. Vaginal agenesis treated by coital dilatation in 20 patients. Primary vaginal dilation for vaginal agenesis strategies to anticipate challenges and optimize outcomes.