Management of lower vaginal agenesis in a patient with unicornuate uterus

Abstract

Background: Absence of the lower vagina occurs from abnormal development of the sinovaginal bulbs and vaginal plate.

Case: We present a case of an adolescent girl, with a history of VACTERL and VATER status post flap vaginoplasty at 8 m of age and correction of vaginal-urethral fistula, with primary amenorrhea and cyclic pelvic pain. MRI showed a right hematocolpos. On exam the obstructed vagina was deviated to the right. Given prior urologic and rectal surgery, and laterality of unicornuate system, intra-operative ultrasonography (US) was performed. A needle was used to enter the inferior aspect of the obstructed vagina. A pull-through vaginoplasty was performed.

Summary and conclusion: Vaginal pull-through is the standard treatment for lower vaginal agenesis. In a unicornuate system, the use of intra-operative US is helpful to determine the appropriate angle of dissection.