Addendum: Since the publication of this post 1 year ago (December 2018), I was contacted by Dr. Kropp regarding one of the 3 patients described in the post. She had an erosion of the AUS and Dr. Kropp had to remove the sphincter. This happened after having had the AUS for a decade.
This is a guest post by Dr. Bradley Kropp.
A few years ago, I diagnosed a grade school girl with female epispadias who presented with persistent urinary incontinence. Dr. Kropp immediately recommended an artificial urinary sphincter (AUS) when I called him to discuss the case. The CHOP/Milwaukee/Boston exstrophy consortium recommended traditional reconstruction with osteotomies when I emailed them. Her family spoke to both centers and decided to have Dr. Kropp place the AUS. Five years later, this girl is a happy, dry teenager that has normal bladder function, normal renal ultrasound, and the biggest smile.
I asked Dr. Kropp to please share his experience with the AUS and he was kind enough to write this post. So without further ado, here is what Dr. Kropp has to say about female epispadias and the AUS…
The artificial urinary sphincter is the best treatment for female epispadias
By Dr. Bradley Kropp
Female epispadias is the rarest diagnosis amongst all of the Exstrophy/Epispadias complex. Without surgery, most girls have some urinary incontinence due to an abnormal bladder neck. This leakage ranges from simple stress incontinence to total incontinence.
The classic surgery for female epispadias has been a formal bladder neck repair with or without a bladder neck sling. This approach has variable success rates, with up to 75 – 80 % continence rates reported on the largest series.
Two manuscripts led me to consider the artificial urinary sphincter (AUS) by showing that the epispadias bladder may not be as normal as initially thought to be:
- Kaefer et al. (1999) demonstrated, that in some patients, classic bladder neck repair may result in detrimental bladder dynamics due to unknown mechanisms.
- Yerkes et al (2000) also reported that epispadias patients do not void with a normal typical pattern.
The aha moment
It was after these two articles were published that Dr. Richard Rink and I sat down one night to discuss the difficulty of surgically creating a bladder neck that would allow for normal voiding. We both decided that, for the female epispadias patient, the AUS was probably the best chance for normal voiding. Both Dr. Rink and I have extensive experience with the AUS and understood all of the risk and benefits. However, despite the knowledge that this “man-made” device would definitely require several replacements throughout the child’s lifetime, we both still believed it would provide the highest chance of normal voiding with the least risk for bladder decompensation.
My experience with the AUS and female epispadias
I have placed 3 AUS (AMS 800) in 3 female epispadias patients as the primary treatment (no prior repairs). All of the cuffs were placed at the level of the bladder neck through a Pfannenstiel incision. The pump is positioned into the patient labia and the pressure regulating balloon is placed in the perivesical space. Pre-operative urodynamics demonstrated small capacity bladders (all less than 50 % estimated capacity) for all patients. Two out of 3 of the bladders developed bladder contractions during the pre-op urodynamics. One of the bladders remained acontractile. Post-operatively, all patients are completely continent of urine. 2 out of 3 patients void spontaneously to completion when they activate the labial pump. The one patient that did not develop a voiding contraction on the pre-op urodynamic study, requires CIC secondary to increased residual urine.
To date, with the shortest follow up of greater than 5 years, no patient has experienced a mechanical device failure or required any further surgery. One patient did develop a hematoma around the labial pump when she suffered a traumatic blow to the groin region during a sporting activity. Because of the swelling in the labial region, the pump was deactivated for several days while the swelling subsided. Reactivation of the pump was started after labial swelling had resolved and no further problems have been encountered.
Renal ultrasounds have been stable without the development of hydronephrosis. Patients are extremely happy with their current bladder management and understand that the typical life expectancy of the AMS 800 is roughly 10 -15 years. I continue to follow them yearly with RUS and urodynamic studies.
Before my experience with the AUS, I performed a formal bladder neck repair in 2 female epispadias patients to try to achieve continence. Both of these patients never voided properly and continued to have incontinence. Ultimately, both required bladder augmentation and the creation of a catheterizable stoma to achieve dryness.
Despite the minimal scientific evidence presented in this short post, I believe, based on more than 20 years of experience, that a bladder neck AUS (AMS 800) is the best form of management for female epispadias. In my opinion, the use of this device has the highest chance of spontaneous voiding and the lowest risk of requiring future bladder augmentation. The greatest downside is the inherent risk of requiring replacement several times during the lifetime of the child.
Dr Kropp practices in Oklahoma City, OK.
He can be reached at OKC Kids Urology