Definition - What does Cervical Agenesis mean?
Cervical agenesis is the absence of the cervix, the tube that connects the uterus to the vagina, from birth. The absence of this tube breaks the channel between the uterus and vagina, leading to infertility. Cervical agenesis is an extremely rare condition with fewer than 100 cases reported in the literature.
FertilitySmarts explains Cervical Agenesis
Cervical agenesis results from the abnormal fusion of the Mullerian ducts (structures that form the uterus, cervix, fallopian tubes, and upper part of the vagina in a female embryo). What causes this Mullerian duct abnormality is not clear. In less than 50% of cases, cervical agenesis occurs along with the absence of the vagina (vaginal agenesis).
Because of the blockage at the level of the cervix, females with cervical agenesis never have menstrual periods. Usually, these females also lack a functioning uterus. However, in about 4.8% of cases, a functioning uterus may be present — in which case the blood resulting from the shedding of the inner uterine lining gets pooled up into the uterus. This, in turn, causes cyclic pelvic/lower abdominal pain due to the blockage at the level of the cervix and the failure of the menstrual blood to flow out of the uterus. The collected blood in the uterus then spreads to the fallopian tubes and ovaries. Thus, infertility is inevitable.
The diagnosis is usually confirmed by an ultrasound followed by an MRI, which reveals the absence of a cervix. The first line of treatment after diagnosis typically involves the use of contraceptive pills, a progesterone preparation like medroxyprogesterone or a gonadotropin-releasing hormone agonist to prevent the menstrual shedding of the endometrium, thereby relieving pain.
To correct the cervical deformity, a surgical connection can be established between the uterus and vagina via a procedure referred to as the uterovaginal anastomosis. This procedure has yielded encouraging results, with a greater number of women achieving pregnancy after the surgery. A pitfall with the procedure, however, is common as the surgically created cervix can become narrow (aka cervical stenosis), making hysterectomy (surgical removal of the uterus) unavoidable.