Speaker 1In this class we're going to talk about urinary diversions in children both indications and construction and I'm going to start by telling you that urinary diversions in children are very uncommon. We are going to talk about the few indications and the surgical options but even among those of you who work in the pediatric population, I'm betting you would agree with me that urinary diversions in children are very rare. I've seen many more fecal diversions in the pediatric population than urinary. They're really required only for management of anomalies that cause reflux or obstruction and therefore that threaten renal function. The most common temporary diversion is a vasocostomy which of course is an opening between the bladder and the abdominal wall and we will talk a little bit more about that. Long-term diversions in the pediatric population very very very uncommon. Most commonly if they do have to be done they involve creation of a catheterizable channel between the bladder and the abdominal wall. So why would a child have a urinary diversion? So we're going to talk about several pathologic conditions that might require urinary diversion. The first is prune belly syndrome which is very accurate but also terrible terminology. It's also known as Eagle Barrett Syndrome. Fortunately this is a very rare congenital anomaly. The vast majority, as you can see, 97% of cases occur in boys. It's called a syndrome because it's a collection of anomalies, primarily for specific anomalies. So look at the illustration on top and you can see why they call it prune belly. You can see how wrinkled the abdominal surface is in this baby. So this is caused because of severely deficient abdominal wall musculature. Since you lack the normal abdominal muscles that typically guard the intra-abdominal contents and serve as a barrier between intra-abdominal pressure and the abdominal surface, without that muscular sheath, that muscular guard, any increase in intra-abdominal pressure can cause breast cancer. The abdominal pressure is exerted directly against the overlying skin and it causes breakdown of the connective tissue components and you get this extensive wrinkling. You get an abdominal surface that's very stretched out, very wrinkled, and looks like a prune. Unfortunately you also get major defects in the smooth muscle of the urinary tract. So think about the fact the bladder is smooth muscle, the ureters are smooth muscle, and the collecting components of the kidneys are all smooth muscle. So if there's a tremendous deficiency in the smooth muscle of the urinary tract, then again, all components of the urinary system are prone to, vulnerable to pressure. When pressure rises within the bladder, the bladder just stretches out, becomes very dilated. When pressure is exerted against the ureteral walls, the same thing happens. When pressure is exerted against the collecting ducts, same thing happens. Now let's come back to the bladder. If you have a significant deficiency in the musculature of the bladder wall, instead of the bladder contracting effectively to empty. It contracts weakly. It only partially empties. The bladder distends. There's no muscle, not enough muscle to prevent that. Bladder distends. Then you've got back pressure on the ureters. They distend. Now you've got back pressure on the kidneys and all the collecting components of the kidney dilate and distend. So you get this very stretched-out, abnormal urinary system, and you can get significant renal failure. They also have undescended testicles, and they may have associated defects that involve the cardiac system, the GI system, or the musculoskeletal system. But most commonly, it's that stretched-out, wrinkled abdominal surface. The dilated, distended urinary system and undescended testicles. Almost always, these babies are diagnosed before birth. It's very obvious on prenatal ultrasounds that the kidneys are distended, that the bladder's distended, that the abdominal wall is stretched out. So what's the prognosis, and what is management? Well, prognosis is variable. It depends on the severity of the disease. The severe forms where you have practically no abdominal musculature and where you have massive dilatation of the renal system, fortunately that's uncommon because it's almost always fatal. Those babies die either because of respiratory failure, they can't clear their own secretions because they have no abdominal muscles. So they die either of respiratory failure or renal failure. failure. But severe forms are the least common. Most of these babies have mild to moderate forms of prune belly and they do much better. Most of these kids do well as long as they're diagnosed and treated. So what does treatment look like? So you have to look at each component of the syndrome. So what can you do about the abdominal wall? If you have significant stretching and wrinkling, they can do a plastic procedure to taper and tighten the abdominal wall, an abdominoplasty, just like we do for people who lose large amounts of weight and have wrinkled, pendulous skin. We can do the same thing for these babies. We can also put these kids in binders so that they have external support for the abdominal cavity. Many of these kids will need a temporary urinary diversion in order to decompress the ureters in the kidneys, just get the whole system decompressed. And most commonly what they do is a vesicostomy, where they create the opening between the bladder and the abdominal wall. So now the bladder doesn't have to contract, urine just drains out. Sometimes they'll do a nephrostomy instead, so they'll actually bring the ureter out to the abdominal wall, so that urine drains from the kidney through the collecting duct into the ureter and out. Once the urinary system is decompressed to some extent, then they can do surgical remodeling of the urinary system. They identify the components with the strongest musculature. They resect redundant components, so they taper the whole system down so that it works more effectively. And then they do orchopexia for the undescended testicles. So babies with severe prune belly typically don't survive. Babies with mild to moderate disease usually do and usually do well if they are treated appropriately. A more common anomaly is spina bifida with myelomeningocele. Now this is known as a neuroendocrine neural tube defect. In the past, it was distressingly common. Now it is less common. We know that development of this anomaly is affected by folic acid levels in the mom at the time of conception. So across the board, women of childbearing age have been encouraged to supplement folic acid levels so that this is not a risk for them. As a result, we've seen a marked reduction in the incidence of spina bifida. But obviously, there are other issues at play because even though we pretty consistently supplement folic acid, spina bifida has not been eliminated. So research is ongoing. So let's talk about what spina bifida and myelomeningocele mean. Spina bifida translates into open spine. So what happens is at one level of the vertebral column, you get failure of closure. So you actually end up with a bony defect where the vertebral bodies fail to close, fail to fuse. When you have a bony defect, then it allows the spinal cord to herniate through that defect. and that's what you see on top. You can see the spinal cord with all the nerve pathways in yellow. You can see it herniating through that defect. Now when the spinal cord herniates through the defect, of course, all of these nerve pathways get compressed and damaged, and so it's almost like having a congenital spinal cord injury. Also very common for these kids to have hydrocephalus and so they require a shunt between the brain and typically the abdominal cavity. Now Spina Bifida with myelomeningocele, as we said, can occur at any point along the vertebral column. Because you get damage to all of the nerve pathways that come off the cord, you're going to get variable degrees of sensory and motor loss below the level of the lesion. The higher the lesion, the more profound the sensory motor loss. The lower the lesion, the better. So kids with thoracic level lesions are typically paraplegic, wheelchair bound, in addition to having neurogenic bowel, neurogenic bladder. Kids with lumbosacral lesions, which fortunately are the most common, many of these kids are ambulatory. They're braced and they might be on crutches, but they are ambulatory. I've had some kids in our clinic with lumbosacral lesions, they did not require crutches. They were ambulatory just with minor braces. But even the ambulatory kids are going to have bowel and bladder dysfunction because the nerve pathways that control bowel and bladder function exit the cord at the sacral level. So even low-level lesions cause fecal and urinary incontinence, which is a huge issue for these kids. And no matter the level of the lesion, most of these kids will have hydrocephalus and will require shunts. So we just said neurogenic bowel and bladder are typical of all lesions. And we're going to talk a little bit more about these things in a little bit more detail, but we're going to talk a little bit more about the lumbosacral lesions. Kids with thoracolumbar lesions, this is what happens. There's a reflex arc and the bladder contracts based on that reflex arc. There's actually sensory pathways over to the cord that signal bladder filling and that activate that reflex arc that causes bladder contraction. The problem is that reflex arc does not control the sphincter. So the problem is that the reflex arc does not control the sphincter. So the bladder contracts, but what does the sphincter do? Many times, the sphincter remains closed, and so the bladder is contracting against the closed sphincter. So even though it looks like bladder contraction via the reflex arc is a good thing, in actuality, many times it results in reflux and renal damage because the sphincter does not routinely open. So reflux, impaired reflex, and renal damage. So reflux, impaired emptying, renal failure. What about sacral lesions? Well, the nerve pathways that cause bladder contraction come off the cord at the sacral level. If you have damage to those nerve pathways, you end up with an acontractile bladder, a bladder that just sits and fills, and then you get overflow leakage. Because they're in constant severe retention, they're at risk for recurrent UTIs, and again, at high risk for renal failure. So whether this child has a thoracolumbar lesion or a sacral lesion, even though the underlying pathology is different, the end result is the same. All of these kits are at risk for renal failure due to impairment, as well as impaired bladder emptying. So in managing these kids, our focus is on protecting renal function. Finding a way to empty the bladder consistently that doesn't put back pressure on the kidneys, doesn't cause reflux, doesn't put the child at risk. Now, we also want to address continence, but continence is a secondary concern. Our primary concern is presently, we don't know what the cause is. We don't know how long it's going to take to get it back to normal. So we don't know how long it's going to take to get it back to normal, and we don't know how long it's going to take to get it back to normal. So we don't know how long it's going to take to get it back to normal. So we don't know how long it's going to take to get it back to normal, and we don't know how long it's going to take to get it back to normal, and we don't know how long it's going to take to get it back to normal. fortunately is rare is bladder atrophy and actually there is a group of defects known as bladder atrophy epispadius complex. No, you don't have to know that term. But this is a defect that involves the bladder, the urethra, the abdominal wall, and the pelvic bones. So you have to think about normal development. And normal development of the bladder and the urethra, they start out as a flat plate. The bladder does not start as a hollow organ and the urethra does not start as a hollow tube, as a channel. Instead, they start as a flat plate to which the ureters are attached. Urine flows from the kidneys through the ureters out this plate and mixes with amniotic fluid. So fetal urine is a big part of the amniotic fluid. Then with normal development, what happens is the bladder closes to form a hollow reservoir. The urethra closes, tubularizes to form a hollow channel. The pelvic bones fuse at the symphysis and the abdominal wall closes. So everything starts open, everything then closes. What happens with bladder atrophy? Something interferes with that sequence of events. So the bladder fails to close. It does not become a reservoir. It does not become a hollow organ. The urethra fails to close. It doesn't form that hollow channel. It's still open. The pelvic bones do not fuse at midline at the symphysis and the abdominal wall fails to close. So you end up with a defect in the abdominal wall. Widely separated pelvic bones and a flat bladder plate that protrudes through that abdominal wall defect and is visible at the abdominal surface. Because development of the genitalia is occurring at the same time as development of the lower urinary tract, it's very common to see some abnormalities involving the genitalia in the abdominal wall. I'm going to tell you in addition to bladder atrophy. This is one of those anomalies that looks absolutely overwhelming to the parents and to anyone who doesn't know exactly what it is they're seeing. Because here's this red thing sitting on the baby's belly and you're telling me that's their bladder? How are we ever going to get this fixed? But actually the goal is to take this infant to surgery as soon as possible. If we can take them within just a couple of days after surgery, the bones are much more pliable, it's much easier to do the repair. So what we want to do, we want to take that bladder plate, we want to close it so that it forms a hollow reservoir. We want to take the urethra, we want to pull that together so that it forms a hollow channel. We want to pull the pelvic bones together at the symphysis and then we want to close the defect in the abdominal wall. Until we can take that baby back to surgery and do this very complicated repair, we want to keep the bladder tissue moist and protected. We don't want it to dry out, we don't want it to be traumatized because then we're going to get an inflammatory response. It's going to result in high levels of collagen deposited in the bladder wall. Then we're going to have a stiff bladder that has a hard time stretching and storing. So very important for us to keep that bladder plate covered and protected. There's more than one thing we can do, but one of the simplest things to do is to just take a piece of plastic wrap like Glad Wrap Pressing. Press and seal or whatever, put it on top of the bladder so that there's protection from the diaper. It's fine for the urine to just flow down and around and into the diaper, but we don't want that bladder plate rubbing against the diaper. So goal number one, protect the bladder plate so you can get the baby back to surgery. Goal number two, take the baby to surgery as soon as possible. Close the bladder, close the urethra, and fuse the pelvic bones, close the abdominal wall. Then at age three to four, they're going to evaluate continence. And they may need to do a follow-up procedure to promote continence. They may need to reconstruct the bladder neck. They might need to re-anastomose the ureters to the bladder so that you get reflux protection and so that you have support for continence. But some of these kids have long-term issues with continence because the sphincters didn't form normally. And if they do have long-term issues with continence, then they may need diversion down the road. And if they do need diversion down the road, typically what they will do is they will enlarge the bladder if necessary because you want a bladder with good storage capacity. So if necessary, they will augment, enlarge the bladder. And then they'll do that appendico-vasocostomy. Now there are variations of bladder atrophy. Remember it was bladder atrophy, epispatias complex. Epispatias hypospatias is fairly common. So this occurs in male infants. And what you have is incomplete tubularization of the urethra. It can involve either the ventral urethra or the dorsal urethra. So here you can see the urethra is partially closed and then partially open. And what happens is when they void, urine sprays everywhere. But that's easily corrected with a staged procedure. Continence is not affected. So we're not going to spend time on that except just I want you to know what those terms mean. Cloacal atrophy is a devastating anomaly because it involves the GI, GU, and reproductive systems. You have bladder atrophy. So everything we've already talked about. That midline abdominal wall defect. Through the defect you've got protrusion of the open bladder plate. What about the bowel? Well the bowel typically ends in a blind pouch that is also herniating through that defect in the abdominal wall. So you've got the bladder plate exposed and you've got into the bowel exposed. And the end of the bowel instead of being tubularized is open as well. So you've got an extra feed blind pouch involving the bowel. And essentially this baby is born with a bowel obstruction. So they require early emergent ileostomy to restore fecal elimination and feeding tolerance. And then the third thing. So you've got to deal with the bladder. You've got to deal with the bowel. Bowel takes precedence, takes priority over bladder because you've got to eliminate that bowel obstruction. So the first thing that happens is they undergo ileostomy. Then they're going to be taken back to do the bladder repair. And then the other thing that these parents are dealing with is ambiguous genitalia. So many times it's not clear from looking at this baby whether they're male or female. It takes chromosomal studies and then either vaginal or penile reconstruction. You can imagine or probably we can't imagine how overwhelming this is for the parents. What's the first thing people ask? Is it a girl? Is it a boy? How awful would it be to have to say I don't know? And that's only one of the problems we're dealing with. So any issues involving the bowel, the bladder, the genitalia, particularly hard to deal with and these parents are dealing with all three. So they need a lot of support. Fortunately we rarely see cloacal atrophy. So I just want you to know what the term means. Now we said that vasocostomy is the most common temporary diversion in children. It's a very simple diversion. So you want to be aware of what a vasocostomy is and the basics of vasocostomy management. It's basically an opening between the bladder and the abdominal wall. I call it a buttonhole type stoma because essentially they make an incision. They line the layers up. And then they suture the bladder and the abdominal wall together so you end up with a little slit opening in the abdominal wall and urine drains through that slit opening. So, you look at a buttonhole and you're like, "Yes, I get what you're saying. That's what a vesicostomy is like." Vesicostomy is usually done only in children and only on a temporary basis. It's easy to do vesicostomy in a child because the bladder is an abdominal organ. So, you can just make a little incision, line the bladder up, sew the two together. It would be very hard to do vesicostomy in an adult because in an adult, the bladder is a pelvic organ. So, really not intended for diversion in adults, intended for temporary diversion in children. If my child has a vesicostomy, how do I manage? Do I have to pouch them? No. Babies, toddlers, it's normal for them to be incontinent. We can manage them with a doctor. We can manage them with a nurse. So, we can manage them with a nurse. We can manage them with a nurse.
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