A 16-year-old male-looking patient presented at the emergency room for severe abdominal pain. Physical examination revealed acute abdomen, ambiguous genitalia, empty rectal vault with watery discharge and right lower quadrant palpable mass. Ultrasound showed a uterus and right adnexal mass. General surgery evaluated urethral patency and noted presence of recto-urethral fistula. Surgical exploration, right salpingo-oophorectomy and suprapubic cystostomy were done. Immediate referral to a reproductive endocrinologist was done postoperatively. Retrograde urethrogram and cystogram revealed neurogenic bladder with fistula formation. On follow up, whole abdomen MRI revealed thickened endometrium with fluid levels, tortuous left fallopian tube, multiloculated left adnexal mass and left renal agenesis. Serum levels of 17-hydroxyprogesterone and cortisol were noted to be elevated and karyotyping revealed 46 XX. Patient then underwent psychiatric evaluation and assessment. Patient was readmitted for urology and pediatric surgery diagnostic work up. However, regardless of the findings, patient decided not to undergo further surgeries and opted to be female.
Hyperplasia ; Vagina, absence of

The surgical management in a case of vaginal agenesis is technically challenging and requires a strong demand for expertise. The objective of this report was to record the hospital's first experience in vaginal reconstruction using Bilateral Pudendal Thigh Fasciocutaneous Flap. Presented here is a case of 23-year old, female who consulted for primary amenorrhea who plans of getting married soon. Physical examination revealed normal secondary sexual characteristics with absent vagina. Imaging revealed an absent uterus with normal ovaries and distal vagina that ends in a blind pouch. Patient underwent vaginal reconstruction with no post-operative complications. Follow-up revealed incision site infection but eventually resolved with oral and topical antibiotics. Four weeks post-operatively, good wound healing was noted, vagina was 6 centimeters in length, admits two fingers on internal examination. Overall, the authors' first neovaginoplasty using Bilateral Pudendal Thigh Fasciocutaneous Flap was a technically safe procedure with good cosmetic outcome.

Surgical Flaps ; Vagina