Committee Opinion

Abstract

Müllerian agenesis occurs in 1 out of every 4,000-10,000 females. The most common presentation of müllerian agenesis is congenital absence of the vagina, uterus, or both, which also is referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis. Satisfactory vaginal creation usually can be managed nonsurgically with successive vaginal dilation; however, there are a variety of surgical options for creation of a neovagina. Regardless of the treatment option selected, patients should be thoroughly counseled and prepared psychologically before the initiation of any treatment. Evaluation for associated congenital renal anomalies or other anomalies is also important. Although exact gynecologic screening recommendations are evolving, all women with a neovagina should undergo routine gynecologic care; however, vaginal cytologic screening is not indicated.

Transition to Adult Care in Persons With Disorders of Sexual Development

“…Pap screening is not recommended for patients born without a cervix, but monitoring the vagina with visual inspection and biopsy of suspicious lesions is appropriate, particularly in patients experiencing bleeding, pain or unusual discharge. 14,15 All patients, with or without a cervix, should be offered the HPV vaccination due to risk of dysplasia and sexually transmitted infections. 16 …”

“…14 Women do not necessarily require vaginal elongation for their well-being, thus a patient should guide the decision of whether or not she desires to have penetrative intercourse as well as when she is ready to proceed. Working with a local physical therapist who provides care for women with pelvic floor dysfunction or pain may be helpful; direct communication may improve the therapists’ understanding of the condition and goals of treatment.…”

Treatment management during the adolescent transition period of girls and young women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS): a systematic literature review

“…This is characterized by the congenital absence of the uterus and the upper 2/3 the vagina with a normal 46,XX karyotype and a normal, age-appropriate hormone profile, since functional ovaries are present [4]. Therefore, the development of secondary sexual characteristics occurs normally [4, 5] and thus the diagnosis MRKHS is generally made late, at the beginning of puberty due to the lack of onset of menstruation (primary amenorhhea) or, seldom, due to the impossibility of sexual intercourse [4]. On physical examination, patients have normal breast development, normal secondary sexual body proportions, body hair, and hymenal tissue [5], which is one of the reasons for misdiagnosis up to 40 % at the first contact to a physician [6].…”

“…Therefore, the development of secondary sexual characteristics occurs normally [4, 5] and thus the diagnosis MRKHS is generally made late, at the beginning of puberty due to the lack of onset of menstruation (primary amenorhhea) or, seldom, due to the impossibility of sexual intercourse [4]. On physical examination, patients have normal breast development, normal secondary sexual body proportions, body hair, and hymenal tissue [5], which is one of the reasons for misdiagnosis up to 40 % at the first contact to a physician [6]. On the other hand, this means, that these young women get their diagnosis of “being not a complete women” in the sensible phase of puberty.…”

“…The bicycle seat stool provides the perineal pressure. Use of dilators in the management of MRKHS is appropriate and successful for mature, highly motivated patients who wish to avoid surgery because the treatment endures several months [5] and is very painful and dilation of the uretra could occure, It is no option, if the vaginal dimple is to short.The surgical treatment should avoid big scars and transplanted tissue like skin, bowl or peritoneum. Therefore the laparoscopic-assisted creation of a neovagina is the treatment of choice.…”