Ultrasound-assisted repair of a unique case of distal vaginal agenesis

OBJECTIVE: To describe a unique vaginal outlet obstruction and its ultrasound-assisted surgical correction. DESIGN: Case report. SETTING: An academic medical center. PATIENT(S): A 12-year-old girl was seen with cyclic abdominal pain, nausea, and an abdominal mass. External genital examination revealed no vaginal opening or dimple. Transabdominal ultrasound revealed a large hematometrocolpos 5 cm proximal to the perineum, with an otherwise normal-appearing uterus and ovaries. INTERVENTION(S): Surgical treatment included perineal incision, creation of a 5-cm passage through connective tissue, drainage of the hematometrocolpos, and mobilization of the proximal vagina, allowing for pull-through vaginoplasty. Ultrasound guidance was used throughout the surgery and allowed for a safe transperineal approach. A vaginal form was placed in the neovagina. MAIN OUTCOME MEASURE(S): Follow-up clinical evaluation and pelvic ultrasonography. RESULT(S): A well-healed perineum and patent 5-cm-long vagina were observed at 4-month follow-up. The patient reported three regular menses. Postoperative pelvic ultrasound scan was normal. CONCLUSION(S): We describe a unique case of isolated distal vaginal agenesis of significant length that was successfully treated via a perineal approach with the intra-operative assistance of ultrasound guidance.