Vaginal Agenesis Associated with Cervical Hypoplasia and Bicornuate Uterus

Abstract

A 16-year-old woman presented with primary amenorrhea and progressively cyclic
lower abdominal pain. Physical examination revealed well-developed secondary sexual
characteristics. Pelvic examination revealed normal appearing external genitalia and an absent
vagina. Pelvic sonogram revealed hypoplastic cervix and right adnexal mass. Laparoscopy
visualized additional information of bicornuate uterus and severe endometriosis. The operative
findings disclosed vaginal agenesis, hypoplastic cervix, bicornuate uterus, right hematosalpinx,
right ovarian chocolate cyst, multiple endometriotic spots at cul-de-sac, and severe pelvic
adhesion. Hysterectomy and right salpingo-oophorectomy were performed. Left ovary was
preserved to maintain hormonal production. Pathological study had confirmed the diagnosis of
endometriosis and cervical hypoplasia. Vaginal reconstruction was discussed with the patient.
Chromosome study was performed with the result of 46,XX. Clinical features and management
of this rare Müllerian anomaly were also discussed.