By: Jennifer Ahn, MD, MS & Jason Van Batavia, MD, MSTR
Some children are born with or develop problems in the genitourinary tract, made up of the kidneys, bladder and reproductive organs. When this happens, there are various treatments and repairs to help them lead healthy lives. This includes genitourinary (GU) reconstruction, which means modifying or repairing the genitourinary tract with surgery.
Here are common genitourinary tract conditions that can be helped with genitourinary reconstruction.
Bladder exstrophy is a rare congenital birth defect that happens when the bladder and abdominal wall do not form properly. In children born with this condition, the bladder is open and exposed on the abdomen. In addition, the urethra and genitals (clitoris in females and head of the penis in males) are split in half.
Diagnosis & treatment
Bladder exstrophy is often diagnosed before birth with a
prenatal ultrasound. After a diagnosis, families should meet with a
pediatric urologist to discuss the condition, what to expect at birth, and surgical options. For babies diagnosed at delivery, a pediatric urologist and often pediatric orthopedic surgeon will be called to talk with the family and develop a reconstructive plan.
Bladder exstrophy requires reconstructive surgery to close the bladder and abdominal wall. It may also involve fixing abnormalities of the hip bones and genitals. Repair can require multiple surgeries, lengthy hospital stays, and close and regular follow up. Depending on the type of surgery, the child may need a cast or need to stay immobile for some time to heal.
Surgery for bladder exstrophy can be done days to several months after the baby is born, depending on the child's case. The goals of surgery are good bladder function, being able to hold urine without leakage (continence), and acceptable external genitalia appearance and function. However, the long-term goal is to maintain normal kidney function.
During fetal development, three openings form to create the genital, intestinal, and urinary tracts. When these do not fully separate in a female it is called a cloacal anomaly. The most severe form is called persistent cloaca. With this condition, the vagina, urethra, and rectum all drain into the same tract or cavity.
Diagnosis & treatment
Diagnosis in newborns is based on the absence of an anus and an abnormal-looking vaginal opening. Repair of cloacal anomalies involves creating a colostomy (bringing part of the intestines out to the skin to drain stool into a bag) soon after birth. In some cases, urine may then drain normally. However, additional surgery may be needed to place a tube into the bladder or vagina, or to create an opening between the bladder and the abdominal wall (called a vesicostomy).
Surgery to create an anus and separate the genital (vagina) and urinary (urethra/bladder) tracts can take place when the child is older. Specific timing of this surgery depends on how severe the condition is and factors such as the child's rate of growth. It usually involves a team that includes pediatric surgeons, pediatric urologists, and pediatric gynecologists. The goal is to create anatomy that resembles that of a typical female and to avoid incontinence. Additional surgery or procedures may be needed to keep the genital tract open and working properly.
Other GU reconstruction procedures children may need:
Bladders are meant to store urine at low pressures until the bladder is ready to be emptied. When bladders are too small or have too much pressure that can cause kidney damage, bladder augmentation (or expansion) may be needed.
In bladder augmentation surgery, part of the intestinal tract is taken and placed onto the bladder to make it larger. The bladder then needs to be catheterized using a flexible tube inserted through a narrow opening to empty it. Often, a channel is created at the same time.
Follow-up care is aimed at preventing complications such as bladder infections, stones and tears caused by poor drainage. The chances of other possible complications, such as electrolytes problems or too much acid in the blood, depend on which part of the intestinal tract was used for the surgery. If your child has abdominal pain or blood in the urine after bladder augmentation surgery, contact your doctor immediately.
After bladder augmentation, a child may be at higher risk for cancer near area of intestine used in the surgery, or in the bladder itself. Because of this, children who have this procedure need continued follow up with a pediatric urologist or surgeon.
This surgery creates a channel from the skin on the abdomen to the bladder so a catheter (drainage tube) can be put in to empty the bladder. The child's appendix is usually used to create the channel. The opening of the channel on the abdomen may be brought out through the belly button to make it less noticeable. The new channel should allow the child to hold urine. However, if incontinence does develop, this can be treated with medication or surgery.
Bladder neck procedures
Some conditions leave a child's bladder neck, an area that connects the bladder to the urethra, weak or open. This can contribute to urine leakage. There are a handful of surgical options to address this. These include injecting a filler material or placing an artificial urethral sphincter (the muscle that controls how the ureter opens and closes).
Other options more common in children include bladder neck reconstruction. This surgery makes the bladder neck narrower and tighter, preventing urine from leaking out as easily. Surgery can also be done to close the bladder neck, disconnecting it from the urethra. This requires an alternate way to drain the bladder, often an appendicovesicostomy (see above). In another procedure, a sling can be made from the patient's own tissues (usually from the abdominal wall), or synthetic material.
MACE (Malone Antegrade Continence Enema)
Children with bladder issues often will also have bowel problems, especially
constipation. Severe constipation may lead to symptoms like
abdominal pain, nausea, vomiting. It can also cause incomplete bladder emptying and urinary tract infections. Some need to use enemas to be able to empty their colons of stool. For these children, a Malone Antegrade Continence Enema (MACE) can help. The surgery uses the appendix or a piece of the intestines to create the channel from the skin on the abdomen to the colon, allowing a catheter to be placed that delivers the enema directly to the colon. This makes it easier for the parent and child to clean out the colon and prevent constipation.
If your pediatrician suggests that your child visit a pediatric urologist to discuss GU reconstruction, rest assured that they have most extensive and comprehensive training to evaluate your child's specific needs. They can also offer a wide range of treatment options to improve your child's quality of life.
About Dr. Ahn
Jennifer Ahn, MD, MS, is an Attending Pediatric Urologist at Seattle Children's Hospital and Assistant Professor in the Department of Urology at University of Washington.
About Dr. Van Batavia
Jason P. Van Batavia, MD, MSTR, is an attending pediatric urologist in the Division of Urology at Children's Hospital of Philadelphia. He is a basic and translational researcher and Assistant Professor of Urology (in Surgery) at the University of Pennsylvania School of Medicine. He is a candidate member of the American Academy of Pediatrics Section on Urology.