Perineal Endometriosis Post Vaginal Stenosis Repair

A 17 year-old 46,XX female patient with a history of urogenital reconstruction was found to have perineal endometriosis at the site of previously repaired vaginal stenosis. The patient presented in early childhood with a 2-cm congenital urogenital sinus. At age 11, she underwent urethroplasty and native vaginal tissue vaginoplasty. Following this procedure, she developed a urethrovaginal fistula and stenosis of the distal centimeter of the vagina, allowing for placement of only a 7-French cystoscope. She underwent menarche at the age of 13 with regular nonpainful menses. At age 17, she underwent fistula repair and vaginoplasty revision, when she was noted to have a nodular blood-filled tissue at the site of the stenosis. The tissue was completely excised and confirmed as endometriosis by histopathology. She subsequently underwent pelvic floor physiotherapy, injection of triamcinolone, and dilation until desired caliber. Perineal endometriosis should be considered in patients with postoperative vaginal or introital stenosis even without typical symptoms of endometriosis such as dysmenorrhea or pelvic pain. If suspected on examination, excision should be considered at time of revision vaginoplasty to avoid recurrent stenosis or pain.