V07-09: THE KIDNEY THAT WASN'T
A14-year-old girl presented to the Emergency Room with severe lower abdominal pain. She described her pain as intermittent left stabbing colic-like pain, occasionally associated with her menses. Upon investigation, she was found to have a non-functioning dilated large pelvic kidney with normal mullerian organs. She was offered a laparoscopic nephrectomy. Herein, we show a video depicting the surgical technique and the unexpected postoperative pathologic findings.
Using a 3 trocar laparoscopic approach, a transperitoneal nephrectomy was done. Upon entry in the abdomen the kidney was easily visualized. Using the LigaSure, the omentum draping the kidney was circumferentially taken down. We noted an unusual appearance to the kidney with abnormal appearing cystic structures and lobulations. As we dissected further medially, multiple small renal vessels arising from the iliac vessels were clipped and cauterized. On the lateral edge of the kidney, a tubular structure that appeared to be a bifid pelvis was transected. Finally, the kidney was freed from the pelvis. Since the kidney was too large to be placed within the EndoCatch bag, hence we attempted deflating the kidney by aspirating the fluid that looked like old blood. In order to remove the specimen, the kidney was then morcelated. It was removed after extending the incision of the lower quadrant trocar. The operative time was 192 minutes.
No operative or postoperative complications were reported.The patient was discharged on the first postoperative day. However, the pathology revealed uterine, tubal and ovarian tissues. There was no renal tissue. The patient had normal menstrual cycles postoperatively and the abdominal pain she experienced before the surgery resolved. A postoperative ultrasound showed normal uterus and right ovary.The left ovary was not visualised. The patient was diagnosed with OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome, a congenital malformation of the urogenital system. In our case, the apparent left pelvic kidney was in fact uterine duplication with an obstructed blind ending hemivagina and left renal agenesis.
The diagnosis of OHVIRA was missed and should be considered in cases of a non-functioning dilated pelvic kidney in females who report pain upon menarche and with menses.