Cervical agenesis is a rare mullerian anomaly with less than 100 cases described in the literature. The etiology of cervical agenesis is not well understood. Presentation is typically lower abdominal pain that may be cyclic or sporadic around of the time of expected menarche, but no menstruation occurs due to the obstruction at the level of the cervix. Diagnosis is confirmed usually by MRI, which must differentiate cervical agenesis from a transverse vaginal septum as the treatments differ. Retrograde menstruation usually results in development of endometriosis, endometriomas, hematosalpinx, and adhesions sooner than with conditions such as imperforate hymen. Cervical agenesis is associated with vaginal agenesis in <50 % of cases. Treatment options traditionally been hysterectomy as earlier attempts to create a fistulous tract between the vagina and cervix resulted in high morbidity with subsequent reoperations and hysterectomy and even death. Recent treatment with uterovaginal anastomosis has revealed encouraging results, with subsequent pregnancies and deliveries reported. Optimal patients for this treatment method may include those with cervical dysgenesis or distal cervical agenesis, rather than complete cervical agenesis. Careful counseling regarding fertility sparing treatment should be undertaken before deciding on the optimal treatment modality for the individual.
KeywordsCervical agenesis Cervical dysgenesis Cervical aplasia Retrograde menstruation Uterovaginal anastomosis Transverse vaginal septum Primary amenorrhea
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