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

Congenital anomalities of the vagina are rare congenital anomalies. Women born with this anomaly present with collection of blood in the uterine cavity or hematometra and pelvic pain. Presented is a case of a 12-year old girl with hypogastric pain and primary amenorrhea complicated by vaginal agenesis. She was managed conservatively by creating a neovagina with the use of bipudental flap or Modified Singapore flap. Management can be non-surgical or surgical but the management of congenital vaginal agenesis remains controversial. The decision to do a conservative surgical procedure or a hysterectomy depends on the clinical profile of the patient, the expertise of the surgeons, the extent of the anomaly, and it's association to other congenital anomalies.

Human ; Female ; Child ; Hematometra ; Vagina, Absence Of ; Amenorrhea ; Vagina ; Hysterectomy ; Pelvic Pain ; Surgeons