At Boston Children's Hospital, we take a multidisciplinary approach to treating posterior urethra valves (PUV). From your first visit, you'll work with a team of specialists from various disciplines who are dedicated to helping your family find the best care for your child—when he is first diagnosed, throughout treatment and as he grows.
How is PUV treated?
In 95 percent of cases, PUV is treated with a surgical procedure called endoscopic incision of the valves
- endoscopic incision of the valves, also known as valve ablation or posterior urethral valve ablation, is a surgical procedure that trims down excessive tissue of the valves.
- This is a minor and minimally invasive procedure and only requires a small incision at the site of the ablation (removal) of the valves.
- While your child is under general anesthesia, doctors will insert a small telescope called a cystoscope into the urethra. With the cystoscope, doctors are able to view the interior lining of the bladder and the urethra on a monitor.
- Doctors will use the imaging to examine the obstruction and remove the valves.
Emergency surgery may be used if your child has:
- respiratory distress (difficulty breathing on his own). A newborn with respiratory distress may need:
- endotracheal intubation: a general term referring to the placement of a tube into a patient's body.
- hydronephrosis (dilation of the kidneys, at times severe). A newborn with hydronephrosis may need the following surgical treatments:
- urinary diversion, a procedure that diverts the urine before it reaches the urethra. This is done with a vesicostomy, an operation performed on infants who are too small to undergo endoscopic valve ablation. This procedure creates an opening from the bladder to the outside of the body so the urine can drain directly into a diaper.
- proximal diversion, a is a less common procedure in which the ureter is brought out to the skin, allowing the urine to be brought out (diverted) to the side of the body. This procedure is done for boys with more severe presentations of the disease. For example, when there are very thick bladder walls or if the bladder itself shows marked abnormality.
Although medication alone can't treat PUV, it may be used in addition to surgery to treat more severe problems.
For example, the wall of the bladder has two layers: a soft inner lining and an outer layer of muscle. Some boys have severe hyperactivity of that muscle, which makes the bladder muscle stiff. These boys may benefit from medications that help relax and soften the bladder muscle.
The most common classes of medication used to help relax the bladder muscle include:
- anticholinergics / antimuscarinics
How often do children with PUV need to be seen?
Ongoing medical management is very important for boys with PUV. Due to the varying degrees of severity, some boys will need to be followed more closely than others. For the most part, boys will need to be monitored for kidney function, bladder functioning and voiding ability (emptying of the bladder).
Generally, after the initial surgery is performed, a child will receive another voiding cystourethrogram (VCUG) to ensure that the appearance of the valves is gone or much less significant.
Six months after the valves have been ablated, doctors will continue to monitor the child's bladder function using a urodynamic study:
- a urodynamic study monitors bladder function and gives specific objective information about the bladder's capacity, storage pressures, bladder (detrusor) muscle, and emptying function of the bladder.
How might PUV affect my child's long-term health?
There could be some long-term problems, so it's important for your son's doctor to see him periodically.
Some of these problems could include:
Even after surgery, your son may continue to experience mild symptoms of urinary incontinence, frequency and urgency. A simple outpatient procedure can often cure these symptoms.
If your son's urinary tract is severely affected, extensive reconstructive surgery may be helpful in preventing damage to the kidneys.
It's essential that you keep in close contact with your son's physician so that she can monitor your son for any sign of developing problems.
Will my child be OK?
Most boys with PUV will have normal kidney function, normal bladder function and normal continence. In most cases, these boys will also be able to have normal sexual development and normal fertility.
Since boys have a 20-25% chance of kidney failure over the course of their lifetime, our specialists in Urology make maintaining optimal bladder function the primary goal of treatment. We help these boys throughout their lifetime with bladder function issues, incontinence and decreasing the risk of urinary tract infections (UTIs). This is done through urodynamic testing to objectively study the bladder function.
Coping and support
If you are feeling overwhelmed by your child's diagnosis of posterior urethral valves (PUV), remember that Children's is here to help. We're dedicated to helping families locate the information and resources they need to better understand their child's particular condition and take part in their care.
This list outlines some of our resources at Children's and in the wider community that may be helpful to you:
Resources for children and families:
- The Bladder Exstrophy Program focuses on children, teens and families who are affected by a urinary disorder such as PUV. The support group, which meets three or four times a year on Saturday, offers guest speakers and teen chats. We also puts parents in touch with other families whose children have similar issues.
Adolescence can be stressful time—even for physically healthy teens. Having a condition like PUV may present further difficulties for teenage boys.