Leading The Way in Treating and Managing Posterior Urethral Valves (PUV)
Lurie Children's nationally ranked Division of Urology oversees one of the most experienced Multidisciplinary Valves Program (MVP) Clinic in the country. Their focus is treating children with a rare urological condition known as posterior urethral valves. Affecting only boys, PUV affects about 1 in 5,000 births and can lead to extreme damage to the kidney and bladder. Lurie Children's urologist Dr. Ed Gong and kidney specialist Dr. Kavita Hodgkins run the valves program and talk with us today about best practices in treating this rare and serious condition.
Topics Covered
- Drs. Gong and Hodgkins (urology and kidney specialists, respectively) developed the Multidisciplinary Valves Program to ensure that boys which this relatively rare condition (posterior urethral valves) have centralized and focused care.
- PUV has a wide range of symptoms. It can lead to extreme damage to the kidney and require the need for kidney transplant, but symptoms can also be relatively minor, such as problems with toilet training and wetting.
- The PUV team at Lurie Children’s participates in a consortium of pediatric urologists, sharing ideas and working to improve the management of PUV.
- Typically PUV can be diagnosed in utero, via an ultrasound. Drs. Hodgkins and Gong may “meet” their PUV patients before they are even born, to help parents develop a treatment plan.
- Surgical repair of the blockage involved in PUV does not completely cure the condition. Rather, in most cases children will need longitudinal care because it can have chronic impact on kidney health and function.
Transcription
Dr. Edward Gong: We made the decision to concentrate the care of these kids onto a few individuals in order to really focus the ability to care for these kids, the nuances between them.
Maggie McKay (Host): Ann & Robert H. Lurie Children's Hospital of Chicago's nationally ranked Division of Urology has one of the most experienced Multidisciplinary Valves Program or MVP clinic in the region. Their focus is treating children with a rare urological condition known as posterior urethral valves or PUV. Affecting only boys, PUV affects about one in 5,000 births and can lead to extreme damage to the kidney and bladder. Lurie Children's urologist, Dr. Ed Gong, and kidney specialist, Dr. Kavita Hodgkins, who run the Valves Program, talk with us today about best practices in treating this rare and serious condition.
This is Precision: Perspectives on Children's Surgery, the podcast from Ann & Robert H. Lurie Children's Hospital. I'm Maggie McKay. I'm so interested to learn more about your expertise in treating and managing posterior urethral valves, doctors. Thank you so much for making the time to be with us here today. It's such a pleasure to have you.
Dr. Kavita Hodgkins: Thank you for having us. It's a pleasure to be here.
Maggie McKay (Host): Dr. Hodgkins, will you please start with introducing yourself and telling us your title.
Dr. Kavita Hodgkins: My name is Kavita Hodgkins, and I'm a pediatric nephrologist here at Lurie Children's and a member of The Chicago Institute of Fetal Health.
Maggie McKay (Host): And Dr. Gong?
Dr. Edward Gong: My name is Edward Gong. I'm a pediatric urologist here at Lurie Children's. I lead our urologic team in The Chicago Institute of Fetal Health. We are also the co-leads for the Multidisciplinary Valve Clinic here at Lurie Children's.
Maggie McKay (Host): And what are your roles in treating children with this rare condition, Dr. Hodgkins?
Dr. Kavita Hodgkins: My role is to monitor and support the health of the kidneys. This condition can very seriously impact the development and ongoing health of the kidneys for our patients, and that requires really continued lifelong management.
Maggie McKay (Host): Dr. Gong?
Dr. Edward Gong: We realized about three or four years ago that, well, this is a fairly rare condition. It does happen fairly commonly, and we had a significant number of children with valves, for short, here at Lurie Children’s. The problem was the care of these kids was spread over so many different urologists that no one could really develop an expertise in their management. So we made the decision to concentrate the care of these kids onto a few individuals in order to really focus the ability to care for these kids, the nuances between them. At that time, we asked Kavita Hodgkins to join us because, while it's a primary urethral or bladder problem, one of the major problems occurs with it is with kidney function and we needed to have someone with expertise on the kidney side.
Maggie McKay (Host): And why are boys the only ones affected with P U V? What is P U V Dr. Hodgkins?
Dr. Kavita Hodgkins: PUV or posterior urethral valves is a condition that occurs only in boys because it affects an area of the urethra, which is the tube leading from the bladder outside, that only occurs in the male urethra. And it leads to essentially a blockage within that tube, so urine that's being constantly made by the kidneys and draining into the bladder is not able to exit the bladder and drain in a healthy way out of the body. That blockage of urine then has a backwards effect on the development of the bladder and the development of the kidneys.
Maggie McKay (Host): Are babies born with this condition or do they develop it later, Dr. Gong?
Dr. Edward Gong: Babies are born with this condition. It's congenital. And during fetal development, these tiny little flaps of tissue that are supposed to recede in the normal urethra end up persisting. And with that persistence, these little tiny flaps are enough to cause havoc in the bladder and the kidneys.
Maggie McKay (Host): And how serious is it if it goes untreated?
Dr. Kavita Hodgkins: The outcomes can be fairly variable, but this is one of the more common causes of serious kidney disease that we see in children. Children with PUV can lead to end-stage kidney disease or the need for dialysis or kidney transplant in about 15 to 20% of children.
Maggie McKay (Host): Anything to add, Dr. Gong?
Dr. Edward Gong: It's incredible how variable the symptoms can be for posterior urethral valves. Some kids just have a little bit of problem with toilet training, wetting and no kidney problems whatsoever. Other kids can run the full gamut, needing kidney transplant or dialysis. There are kids whose bladders never work. They can't empty their bladders at all, even after the valves have been treated because the bladder itself never developed properly. And some of these kids end up needing to undergo intermittent catheterization to empty their bladders for the rest of their lives. Some kids need major reconstruction of the urinary tract. On the other hand, we have plenty of boys with a diagnosis of valves who have lived normal lives. They've been perfectly fine with no problems with urination or kidney function after the valves were treated.
Maggie McKay (Host): Lurie Children's is one of the leaders in managing this condition. So what makes it different from other hospitals? I know you work in tandem together in the MVP Clinic or the Multidisciplinary Valves Program. So can you tell us more about that?
Dr. Kavita Hodgkins: That has been an incredibly exciting new program that we've been able to start now about three years ago, where Dr. Gong and I can work side by side taking care of patients with this fairly rare condition and really combine our approaches where we're thinking simultaneously about the medical management of our patient's kidney disease and the management of their bladder dysfunction, surgical interventions that they may need and taking kind of a holistic picture of where our patients are at and what we think they might need in the future. I think it's allowed us to be quite proactive where Dr. Gong may see a red flag or a warning sign, and can share that, and we can respond to that as a team or vice versa.
And we're also noticing some patterns in patient presentation and some emerging patterns that may have clues about outcomes. Dr. Gong was mentioning the variability that we see in terms of how significantly each patient is affected and we're looking for clues to be able to help families predict better what their child might come to face over time. And I think this joint clinic will really be an exciting way to expand that understanding.
Maggie McKay (Host): Dr. Gong?
Dr. Edward Gong: Beyond concentrating the experience and the care of this rare condition here at Lurie Children's, we also participate in a regional consortium of pediatric urologists where we share ideas. Within the context of this, we have been trying to standardize the management to create a pathway for the care of these kids so that they all get the same testing, the same necessary testing, and that allows us to change and to tweak management individually.
Maggie McKay (Host): And you touched earlier on the fact that every case is different. Some boys may just need to manage it and never have a problem later. Some people may need surgery. What's the most common scenario? Or is there one?
Dr. Edward Gong: The most common scenario for diagnosis here at Lurie Children's is, frankly, through The Chicago Institute of Fetal Health. Kids are typically identified prenatally to have severe dilation of the kidneys, urinary tract and bladder. And when these kids are identified early, Dr. Hodgkins and I meet with them prior to delivery, and we have conversations regarding the potential diagnoses and potential prenatal treatments if they're indicated.
Typically, prenatal treatment for these kids is not necessary, and the boys are born. They tend to be fairly healthy at birth. The reason for this is the kidney function in utero is supplied by the mother and not the baby. If these boys are left undiagnosed, they end up getting really sick, typically within a couple weeks of life, and they come into the hospital with what we call failure to thrive. They're just not growing. They're not feeding. They're sick. They come in with kidney failure. But if we catch them prenatally, then we can dictate their care from the very beginning. And when that happens, these babies, after they deliver wherever they are, get transferred into Lurie Children's. And after the transfer, we begin the management.
And the first step is fairly simple. You get ultrasound to confirm the diagnosis. We put in a catheter to bypass the valve and allow urine to drain from the bladder. At that point, we have some time to figure out, "Well, how good is the kidney function right now? Can we improve the dilation just by draining the bladder?" And if we can, the next step is to prove that there are valves there with an x-ray-based test called a voiding cystourethrogram.
And once we know for sure that these flaps of tissue are there, they go to the operating room fairly early in life, not for any sort of cutting outside the body, but using scopes and endoscopic approach. We look into their urethra, find that area where these little flaps of tissue are, and we cut them open.
In the best case scenario, doing this alone leads to complete decompression of the kidneys and the bladder, and a good number of these kids end up doing fine for a very long duration. Unfortunately, there are a handful of kids within this group, and it tends to be about 15% to 20% who end up progressing to renal failure. Similarly, within this group, there's about 15% to 20% that progress to bladder failure, where in order to protect their kidneys or to help them remain dry, to be continent, they end up needing intermittent catheterization.
Maggie McKay (Host): Is there anything else either of you would like to add about PUV?
Dr. Kavita Hodgkins: I think one misconception or misunderstanding that sometimes occurs is that the big problem in the development that leads to this condition is this blockage of the posterior urethra/ and that once that's surgically repaired, once that blockage is removed, then the problem has been solved. And that's unfortunately not the case because there's chronic impact to the kidney health and function, to the bladder health and function that has already occurred during development, that we can't completely reverse. And sometimes that impact to the kidneys, the impact to the bladder is not immediately apparent. Sometimes children are quite healthy and voiding normally and are able to toilet train and the kidney health is reassuring, but that can change later in life. Those things can be dynamic in this condition in posterior urethral valves.
And so having this multidisciplinary home for our patients, we hope will really improve followup and longitudinal care, so that we can keep monitoring our patients looking for any warning signs or red flags of changes in those two aspects, the health and function of the bladder and the health of the kidneys. And sometimes around puberty is a common age where we can see, unfortunately, declines in kidney function or declines in bladder function, that we can be really proactively prospectively monitoring for and managing as best as we can once we see those changes.
Maggie McKay (Host): Thank you so much, Dr. Hodgkins and Dr. Gong, for your time and your expertise, sharing your knowledge. We appreciate it and it is fascinating and helpful to a lot of parents going through it with their child.
Dr. Kavita Hodgkins: Thank you for having us. It was great to be here.
Dr. Edward Gong: Thank you very much for having us.
Maggie McKay (Host): For more information, please visit luriechildrens.org/urology or to make an appointment, call 1-800-KIDS-DOC. And if you found this podcast helpful, please share it on your social channels and check out the full library for topics of interest to you.
This is Precision: Perspectives on Children's Surgery, the podcast from Lurie Children's Hospital. I'm your host, Maggie McKay. Be well.
