Urethral obstruction after primary exstrophy closure: what is the fate of the genitourinary tract?

Abstract

We assessed the impact of posterior urethral obstruction after primary bladder exstrophy closure. A review of the records of patients with classic bladder exstrophy at our institution identified 29 boys and 12 girls with a mean age of 11.75 years who had had posterior urethral outlet obstruction after closure was done in the neonatal period. Of these 41 patients 75% underwent closure elsewhere. At closure osteotomies were done in 13 patients and 23 were younger than 72 hours. Paraexstrophy skin flaps were used at primary closure in 27 cases (66%). Obstruction presented as recurrent urinary tract infection, upper tract deterioration with or without renal failure, bladder stones, difficult catheterization, urethral stitch erosion, a full bladder on ultrasound, a prolonged dry interval, urinary retention, inability to catheterize, bladder rupture, rectal prolapse and epididymitis or prostatitis. Usually the initial obstructive episode developed within 60 days of closure and it was recurrent. Therapy included suprapubic catheter placement, vesicostomy, ureterostomy, nephrostomy and multiple urethral manipulations, such as dilation with or without steroid injection, internal urethrotomy, urethral stitch removal, clean intermittent catheterization or open urethroplasty. All 6 patients who underwent long-term diversion via vesicostomy, ureterostomy or a conduit for greater than 6 months required permanent bowel segments for reconstruction, while in 5 of the 6 who underwent short-term diversion via nephrostomy or suprapubic tube placement for less than 6 months reconstruction was bowel-free. Of the 36 children in whom functional reconstruction was performed 9 are undergoing staged reconstruction, reconstruction failed in 14, 4 are socially dry and 9 are continent. Posterior urethral obstruction after exstrophy closure markedly decreases the success of staged bladder reconstruction, presents a significant risk to the upper urinary tract and should be detected early.