Obstructed uteri with a cervix and vagina.

OBJECTIVE

To describe a rare anomaly of the female reproductive tract and review the embryology associated with the defect.

DESIGN

Case report and review of the literature.

SETTING

Major academic medical center.

PATIENT(S): A 14-year-old girl with two hemiuteri lacking any communication with a single normal midline cervix and vagina.

INTERVENTION(S): Diagnostic laparoscopy with chromopertubation to identify the anomaly and subsequent bilateral supracervical hemihysterectomies.

MAIN OUTCOME MEASURE(S): Incidence, pathogenesis, fertility implications, and treatment options for patients with congenital defects in the upper vagina, cervix, and uterus.

RESULT(S): Based on classic embryology, the lower vagina forms from the urogenital sinus while the upper vagina, cervix, and uterus form from the müllerian ducts. If a cervix is present, then the upper vagina and uterus are also usually present and should communicate.

CONCLUSION(S): This anomaly cannot be fully explained by traditional embryologic developmental theory. It is likely that an insult occurred between 9 weeks, when the uterovaginal canal is formed, and 12 weeks, when the müllerian ducts fuse.