congenital absence of the vagina or vaginal agenesis, where the vagina fails to develop properly скачать в хорошем качестве

congenital absence of the vagina or vaginal agenesis, where the vagina fails to develop properly

Congenital absence of the vagina, also known as vaginal agenesis, is a rare developmental condition where the vagina fails to form properly during embryogenesis. It is most commonly associated with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, though it can occur in other contexts. 🧬 Embryological Basis The Müllerian (paramesonephric) ducts normally give rise to the uterus, fallopian tubes, cervix, and upper vagina. In vaginal agenesis: The lower vagina (from the urogenital sinus) may be present. The upper vagina and uterus are absent or rudimentary. Ovaries develop normally because they arise from a different embryologic origin (genital ridge). 🔍 Clinical Features Primary amenorrhea (no menstruation by age 15–16) Normal external genitalia Normal secondary sexual characteristics (breast development, pubic hair) Short or absent vaginal canal Often discovered during adolescence when menstruation fails to begin 🧪 Diagnosis Pelvic examination: May reveal a vaginal dimple or short canal Imaging: Pelvic ultrasound or MRI to assess internal reproductive structures Karyotype: Typically 46,XX Hormonal profile: Normal estrogen and gonadotropin levels 🛠️ Management Options Treatment focuses on creating a functional vaginal canal for sexual activity and psychological well-being: 1. Non-Surgical Dilation (Frank Method or Ingram Modification) Uses graduated dilators to gently expand the vaginal dimple over time First-line therapy in motivated patients 2. Surgical Vaginoplasty Techniques include: McIndoe procedure (skin graft) Vecchietti procedure (laparoscopic traction) Intestinal vaginoplasty (using bowel segment) Reserved for cases where dilation fails or is not feasible 🧠 Psychological and Reproductive Considerations Fertility is affected due to absent uterus, but ovarian function is intact Assisted reproductive options include: Surrogacy using own eggs #Congenital