The genetic male with absent penis and urethrorectal communication: experience with 5 patients.

Abstract

Congenital aphallia is a rare anomaly that occurs only once in 30 million births. Five cases are described. All 5 patients 4 to 20 years old had been reared as female. The bladder neck or short urethra entered the anterior rectum in each patient. At referral 2 patients had a vesicostomy and 1 had a colostomy. Hydronephrosis and vesicoureteral reflux were present in 2 and 3 cases, respectively. After separating the urinary tract from the rectum a urethra was created in each patient and a bowel vagina was constructed in 4 during the same operation. The initial patient did not undergo simultaneous creation of a vagina. One patient later required a bladder neck narrowing operation for stress incontinence. She became incontinent again after transpubic surgery to create a sigmoid vagina was performed elsewhere 8 years later. The other 4 patients are dry, and they have normal urinary and bowel control. A single extensive operation on the bladder, vagina and rectum simultaneously is the ideal approach in these cases using the prone posterior sagittal and supine abdominal perineal positions.