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Urology

Center for Antenatal Urology

The Division of Pediatric Urology at NewYork-Presbyterian Morgan Stanley Children's Hospital is an integral part of one of the world's leading maternal/fetal medicine centers. A large number of abnormal findings seen on sonograms during pregnancy are related to urology. Our staff works closely with specialists from the fields of Obstetrics, Neonatology, Pediatric Anesthesiology, as well as all pediatric surgical sub-specialties. This collaboration is invaluable to the management of any finding during pregnancy and also allows for a seamless transition from pregnancy to delivery to care of the infant.

Our pediatric urologists are highly experienced in treating the following prenatal urologic conditions, including:

• Hydronephrosis
• Ureteropelvic junction (UPJ) obstruction
• Reflux
• Ureterovesical junction obstruction (UVJ)
• Ureterocele
• Ectopic ureter
• Posterior urethral valves
• Prune belly syndrome
• Multicystic dysplastic kidney

For more information on treatments for these prenatal urologic conditions, please click on the links above right.

Ureterovesical junction obstruction (UVJ)
If there is a blockage where the ureter enters the bladder, it is called a UVJ obstruction. The ureter can get quite large and is called a megaureter. However, not all dilated ureters are obstructed and the hydronephrosis from UVJ obstruction will often improve with time. The same tests used for diagnosing UPJ obstruction, namely MAG-3 lasix washout renal scan, IVP, and MRI, can be used to diagnose UVJ obstruction. If a UVJ obstruction exists, it is often due to a segment of poorly peristalsing ureter near the bladder. In addition to removing the obstructed segment, the ureter needs to be tapered or narrowed to fit properly into the bladder.

Ureterocele
A ureterocele is a cyst-like enlargement of the ureter in the bladder. It is typically found when there are two ureters draining a kidney. When a ureterocele is present, it is associated with the upper ureter. It causes obstruction of varying degrees in the upper portion of the kidney and has varying levels of function. The ureterocele rarely can block the bladder outlet and prevent urine from exiting the fetal bladder. The ureterocele is also associated with reflux in nearly half of all cases.

The choices for treatment of a ureterocele are numerous and need to be individualized to each child, depending on the presence of reflux, function of the kidney's upper pole, size of the ureterocele, and degree of hydroneprhosis. Treatment options include cystoscopy and puncture of the ureterocele; upper pole nephrectomy; resection of the ureterocele and ureteral reimplantation; connecting the upper pole ureter to the lower pole ureter; and observation. Some of these procedures are appropriate for the newborn and some are best done at an older age.

Ectopic ureter
An ectopic ureter is one that drains somewhere other than the bladder. Like ureteroceles, ectopic ureters are usually associated with kidneys that have two ureters and involves the upper pole ureter. These ureters can be quite dilated. In girls, ectopic ureters cause continuous incontinence. As with ureteroceles, the evaluation of ectopic ureters requires a combination of postnatal ultrasounds, VCUG, nuclear renal scan, and MRI. If no function is seen in the upper pole of the kidney, an upper pole nephrectomy is performed. If function is seen, the ectopic ureter (along with the lower pole ureter) is reimplanted into the bladder. Another option is connecting the upper pole ureter to the non-obstructed lower pole ureter.

Posterior urethral valves
Posterior urethral valves (PUV) can be the most severe urologic condition seen in the fetus. The valves are abnormal leaflets at the beginning of the urethra and can partially or completely block the flow of urine out of the bladder. PUV can cause from mild to moderate to severe hydronephrosis in one or both kidneys. The hydronephrosis can be so pronounced that there is the chance of significant renal damage. Other times, one kidney can bear the brunt of most of the hydronephrosis and the other kidney is spared and will function normally. Fortunately, in most cases both kidneys have reasonable function.

Of great significance with PUV is the amount of amniotic fluid around the fetus. This fluid is composed almost totally of urine that leaves the fetal bladder. Without this fluid, the fetal chest is compressed and the lungs do not develop properly. Uncommonly, a fetus is found to have PUV, severe hydronephrosis, and oligohydramnios (reduced amount of amniotic fluid). It is in this concerning, although unusual, situation that a fetal procedure is considered. The procedure most often performed is placement of a stent, or tube, that drains urine from the obstructed urinary bladder into the amniotic space. This has the dual benefit of relieving the pressure on the bladder and kidney and also restoring the amniotic fluid to a normal volume. The stent is introduced via needle and a wire similar to the way an angioplasty stent might be delivered to a heart artery.

After delivery, in all cases of PUV a sonogram of the kidneys and bladder and a VCUG is obtained. A urethral catheter is inserted to drain the bladder. The child is monitored closely and lab tests are drawn to measure the kidney function. Shortly after the baby is born cystoscopy is performed, whereby a telescope is used to look inside the urethra and burn away the urethral valves. It is especially important a child with PUV be delivered and cared for in a medical center that has all of the pediatric specialties (i.e. Anesthesia, Neonatology/ICU, Pediatric Urology, Pediatric Nephrology, etc.) necessary to take care of this condition. The child may need no future procedures whatsoever or possibly other procedures based on the presence of reflux and kidney function.

Prune belly syndrome
Prune belly syndrome (PBS) is rare and is classically described as a male child with three main findings: a wrinkled abdominal wall due to insufficient abdominal wall muscles; testes found in the abdomen; and abnormalities of the urinary tract. It is the last finding that can be seen on the obstetrical ultrasound and alert the physician to the diagnosis of PBS. The obstetrical ultrasound may show evidence of an enlarged prostatic urethra, an enlarged bladder and hydronephrosis. These urinary tract findings can look like posterior urethral valves. At times the obstetrical ultrasound may demonstrate bilateral undescended testes. A small group of PBS patients may have an obstruction in the middle of the urethra (megalourethra). Oligohydramnios (deficiency in amniotic fluid) can be present and can lead to poor lung development.

Since many other anomalies can be associated with PBS, it is again critically important for the child to be in a major pediatric center with specialists from Anesthesia, Neonatology/ICU, Pediatric Urology, Pediatric Nephrology, Cardiology and Pulmonology available. Even prior to the urologist's evaluation, the child must be examined for possible abnormalities in the heart, lungs and bones.

The baby is then evaluated with an ultrasound. The ureters are often more severely dilated than the kidneys. Nevertheless, the kidneys can be so hydronephrotic and function so poorly as to ultimately require dialysis. But the majority of PBS patients do not require dialysis. A VCUG will assess for reflux which is quite prevalent in PBS.

Treatment options are debated as to how aggressively to tailor the enlarged bladder, but there is general agreement that the baby will need surgical procedures to bring the testes into the scrotum; to correct the ureteral reflux; and to reconstruct the abdominal wall. None of these procedures are necessary immediately after birth.

Multicystic dysplastic kidney (MCDK)
Multicystic dysplastic kidney (MCDK) describes a kidney that has been replaced by cysts. This should not be confused with polycystic kidney disease (PCKD) in which both kidneys are ultimately replaced by cysts and kidney function progressively deteriorates. In MCDK, the other kidney usually functions well and the child is able to lead a perfectly normal life (with perhaps some minor limits on high-speed activities, i.e. skiing etc).

During pregnancy, the ultrasound is quite reliable in distinguishing the MCDK from hydronephrosis. After birth a sonogram will confirm the MCDK. In cases where the diagnosis is in doubt, a nuclear renal scan is obtained. A VCUG is performed to rule out the possibility of reflux.

There is debate as to whether a MCDK requires removal and how often sonograms need to be repeated, but studies have shown that many MCDKs will shrink with time. Even if the MCDK needs to be removed, it is rarely required in the newborn period.