Week 284: Case 1

1. Presented by Peter Illei, M.D. and prepared by Danielle Wehle, M.D.

The patient is a 15 year-old male hermaphrodite who was raised in the female sex. She has congenital nephropathy secondary to Denys-Drash syndrome and had a renal transplant at age 2. Currently, she presented with persistently elevated testosterone levels despite prior laparoscopic gonadectomy (testicular tissue was removed from the left side). She was brought to the operating room for laparoscopic exploration of the pelvis to identify remnant gonadal tissue. During surgery, a Mullerian remnant was found attached to the vagina and adhering to the midline interior abdominal wall. Attached to this and to the dome of the bladder on the right side was a nodular structure that was considered part of the remnant and was removed. Histology of this lesion is shown on the images.