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Illnesses We Treat
Pediatric Urologic Problems
Pediatric urology provides management of problems of the urinary tract and genitalia in
children and adolescents. This field encompasses many diseases from the very common such
as hydroceles (collection of fluid around the testicles) and hernias to the very rare such
as ambiguous genitalia and exstrophy (an abnormality of formation of the bladder and bony
pelvis). An understanding of embryology, anatomy and physiology are crucial to dealing
with these congenital disorders.
Pediatric Kidney
Diseases
Pediatric Bladder Diseases
Pediatric Genital Abnormalities
Contact Information
Pediatric Kidney
Diseases
As maternal obstetrical sonographies have become more prevalent, obstetricians,
neonatologists and pediatric urologists have become exposed to a wide variety of
abnormalities detected during pregnancy. Over half of the prenatally detected
abnormalities are related to the genitourinary system. By far the most common prenatally
detected abnormality is hydronephrosis.
Hydronephrosis
- What is Hydronephrosis?
Hydronephrosis refers to extra urinary fluid in the kidney. Hydronephrosis is not a
separate disease, but a physical phenomenon that occurs with many diseases. Symptoms,
treatment, and prognosis are those of the associated diseases.
- What causes Hydronephrosis?
Hydronephrosis can be caused by obstruction of the ureters or bladder outlet.
Hydronephrosis can also result from reflux (retrograde passage of urine from the bladder
up the ureters to the renal pelvis.)
- What are the treatment options for Hydronephrosis?
While the indications for prenatal intervention are extremely unusual, The Mount Sinai
Medical Center's Department of Obstetrics has a good deal of experience in prenatal
intervention, specifically placement of vesico-amniotic shunts to treat bladder outlet
obstructions. However the majority of fetuses can be followed with frequent serial
ultrasounds until they are delivered at which time post-natal evaluation and management
are appropriate. Children with prenatally detected hydronephrosis should undergo repeat
ultrasound (usually after the first 48 hours of life) and often have a VCUG (voiding
cystourethrogram) to determine the cause of this hydronephrosis. A VCUG study tests for
the presence of reflux and bladder obstruction (see section on bladder).
It is becoming increasingly apparent that mild to moderate hydronephrosis in the
neonate represents a transient non-obstructive dilatation that will frequently resolve
with observation. Moderate to severe hydronephrosis may represent a significant urinary
tract disorder, the most common being ureteropelvic junction (UPJ) obstruction. Other
diagnoses (from common to uncommon) include vesicoureteral reflux, posterior urethral
valves, megaureter and prune belly syndrome. To determine if a UPJ obstruction exists a
functional study such as an IVP or a nuclear renal scan are necessary to evaluate the
clearance from that kidney. As stated previously the majority of cases of mild
hydronephrosis can be managed non-operatively. It should be stressed to parents that if in
fact surgery is necessary, the long-term results of repair of even the most severe
uropathies can be very encouraging.
Tumors of the Kidney
- What are some tumors of the kidney which afflict children?
The most common renal tumor in childhood is Wilms' tumor. Mesoblastic nephroma is also
a pediatric kidney tumor. Another tumor of childhood is the
neuroblastoma, which is a tumor actually near the kidney, found commonly in the adrenal gland (but it can be found
anywhere along the distribution of the sympathetic nervous system). These tumors require
surgical excision as well as combined management with pediatric oncology.
- What are signs/symptoms of tumors of the kidney?
Symptoms of tumors of the kidney include abdominal pain, an abdominal mass, blood in the urine, vomiting, and failure to thrive (amongst others).
- What is the prognosis for pediatric kidney tumors?
The prognosis of these tumors has improved greatly with the combination of surgery,
chemotherapy and radiation.
Kidney Stones
- What types of kidney stones do children get?
Kidney stones in children are much less common than in adults. The essence of treatment
in childhood stone disease is to find out what type of stone is present. These include calcium oxalate,
uric acid, and cystine stones, and struvite stones. Once the type of stone has been elucidated
the etiology of the stone formation needs to be identified.
- What are some causes of kidney stones in children?
Kidney stones are frequently caused by inborn errors of metabolism, refulx, and obstruction.
- What is the treatment for kidney stones in children?
A detailed evaluation in conjunction with pediatric nephrology is necessary.
Concurrently, treatment of the stone can be done with shock wave lithotripsy or endoscopy. This
procedure disintegrates the stone without the need for surgical incisions. Less commonly,
the stones need to be treated with open surgery.
Pediatric Bladder
Diseases
Urinary Tract
Infections(UTI)
Often urinary tract infection (UTI) is the first harbinger of a
congenital bladder or kidney anomaly. These UTIs may go undiagnosed if there is not a proper examination of the urine. If a true
UTI exists then renal and bladder ultrasound as well as a VCUG should be performed to rule
out any renal or bladder anomalies. Thirty to forty percent of children with a febrile UTI will
have some type of urologic congenital anomaly.
- What are the signs and symptoms of UTI’s?
The signs of a UTI include:
- Dysuria (burning on urination)
- Urinary frequency
- Urinary urgency
- Foul smelling, cloudy urine
- Incontinence (bed wetting episodes)
- Fever
Vesicoureteral
Reflux
- What is Vesicoureteral Reflux?
Vesicoureteral reflux is backwards or reflux passage of urine of the bladder up towards the kidney. There are 5 grades, with 1 being the mildest and 5 being the most severe. Most children develop grade
1-2 reflux and fortunately this will resolve over time in the great majority of cases.
Grade 3 will resolve approximately 50 percent of the time and grade 4 and 5 will resolve only in
the minority of cases.
- What are the treatment options for vesicoureteral reflux?
The goals of treatment in reflux are to prevent infected urine from reaching the kidney
which can cause pyelonephritis (kidney infection), scarring, hypertension, proteinuria and even end stage renal disease.
Therefore many of these children are placed on low-dose daily antibiotics. If reflux is of
high grade or associated with a concomitant condition that will make its resolution
unlikely then surgical repair of reflux is an extremely effective and appropriate option.
The surgery to correct reflux essentially lengthens the path of the ureter as it travels into the bladder. The surgery is successful approximately 98 percent of the time, and children go home in 2-3 days.
In an attempt to correct reflux without the need for traditional surgery, the Mount Sinai Division of Pediatric Urology is investigating a new cystoscopy procedure. In this method a material is injected through a cystoscope to alter the ureter's opening in the bladder.
Prune Belly
Syndrome
- What is Prune Belly Syndrome?
Prune belly syndrome is an extremely unusual condition. This occurs in males who have a
triad of findings: a wrinkled abdominal wall as a result of absence of abdominal
musculature; bilaterally undescended testes; and urinary tract anomalies. The urinary
tract anomalies include an extremely large bladder and severe reflux with dilated ureters
and varying degrees of dysplasia of the kidneys.
- What are the signs and symptoms of Prune Belly Syndrome?
here are no symptoms. Prune Belly syndrome is diagnosed at birth when a child has the above findings.
- What are the treatment options for Prune Belly Syndrome?
The testes are usually able to be brought into the scrotum and the
abdominal wall defect can be surgically repaired so that it has a less wrinkled appearance. Reflux is corrected if it does not resolve. While the bladder is large, it generally does not need surgery to reduce its size.
Exstrophy
- What is Exstrophy?
Exstrophy is an uncommon congenital bladder anomaly. Bladder exstrophy results when the
tissue making up the abdominal wall is deficient and the bladder is present as a flat
sheet on the abdominal wall instead of being a sphere inside the pelvis. Urine can be seen
leaking out of the abdominal wall from the exposed ureteral orifices. This is associated
with pubic separation and epispadias, which is a severe congenital curvature and foreshortening of the penis.
- What are the treatment options for Exstrophy?
Traditional treatment is to reconfigure the bladder as a sphere in the first 48 hours
of life and to bring the widened pubic bones together. At a later date construction of the
bladder neck as well as the correction of reflux should be performed. In the third stage
the penile abnormality is repaired. This is technically demanding surgery and requires
dedicated commitment to follow-up by both the treating physician and the family. At times,
several of these procedures can be combined under the same anesthesia, and in fact in many instances the exstrophy and epispadias are repaired together in one surgery in the first 2 days of life. The ultimate goals
are creation of a normally functioning bladder (including adequate volume, lack of reflux,
and dryness) and a normally appearing and functioning penis. Some of the patients require
augmentation (enlargement) of the bladder with a portion of the intestines.
Posterior urethral
valves
- What are posterior urethral valves?
Posterior urethral valves are wisps of tissue in the prostatic urethra preventing the
egress of urine out of the urinary bladder. This can range from a mild obstruction to a
very severe one resulting in renal failure, bladder dysfunction and even death. These
problems are diagnosed increasingly by prenatal ultrasound. If diagnosed prenatally, the
goal of treatment is to obliterate these valves via a cystoscope within the first week of
life. Attention should be directed to the possible electrolyte abnormalities as well as
possible pulmonary hypoplasia (underdevelopment of the lungs) that may have developed
secondary to the oligohydramnios (decreased amniotic fluid).
- What are the treatments for posterior urethral valves?
In rare cases, a combined obstetrical/urologic team needs to intervene prior to
delivery to protect the development of the kidneys and lungs. Posterior urethral valves can be treated in the first few days after birth in almost all infants. A cystoscope is introduced in the urethra, and through the scope, a small wire is used to burn away the valves. Long-term follow-up with the urologist is necessary in many cases.
Neural Tube Defects
(Myelodysplasia or Spina Bifida)
- What are neural tube defects (Myelodysplasia)?
Neural tube defects occur when the bones of the spine do not correctly form, leaving the spinal cord and its nerves and coverings to protrude out through the skin (or lie just beneath the skin).
The use of folate vitamins during pregnancy has decreased the incidence of neural tube
defects. However many children still present with myelodysplasia (which includes
myelomeningocele, occult spina bifida, etc). Since the bladder and the urinary sphincters
function under neurologic control from the spinal cord, damage to the spinal cord can
result in lower urinary tract dysfunction which, if untreated, can lead to renal damage.
- What are the signs and symptoms of neural tube defects (Myelodysplasia)?
Frequent infections, incontinence, and urinary frequency are common symptoms of neural
tube defects.
- What are the treatment options for neural tube defects (Myelodysplasia)?
The goals of treatment for a child with myelodysplasia are a normal capacity bladder
that stores urine at a low pressure and does so in a continent fashion. In addition we
hope to preserve the function of the kidneys and decrease the amount of infections that
the children have. A great advance in this cause has been the common usage of clean
intermittent catheterization as well as pharmacologic agents and urodynamic studies which
help elucidate the function of the bladder. If these conservative measures do not assist
in controlling bladder function other surgical procedures can restore the bladder towards
normal function.
Pediatric Genital
Abnormalities
Hypospadias
- What is hypospadias?
The most common genital abnormality seen by pediatric urologists are hypospadias
abnormalities. In this condition the urethral opening is not at the tip of the penis but
further down on the ventral surface (i.e., between the head of the penis and the scrotum). Often this abnormality is associated with chordee, a
curvature of the penis down towards the scrotum. Surgical repair is recommended for those
children whose hypospadias will affect future fertility, sexual intercourse, cosmesis and the ability
to void in the standing position.
- How is hypospadias caused?
The cause of hypospadias is not exactly known.
- How can hypospadias be treated?
The majority of procedures to correct hypospadias are done on an ambulatory basis under
general anesthesia and are extremely successful. The foreskin, which is present only on
the dorsal side, should not be removed prior to the reconstructive surgery as often this
skin will be used in the repair. However due to advances and newer techniques, certain
types hypospadias can be repaired even in the absence of the foreskin. This type of
surgery, as with all of these congenital anomalies requires a good deal of experience and
should be done only by someone who deals with these problems on a regular basis.
Episadias
- What is Episadias?
Epispadias includes a severe dorsal curvature of the penis up towards the abdominal
wall as well as the urethral opening near the surface of the abdomen. It is usually
associated with bladder exstrophy.
- What are the treatment options for Episadias?
Episadias can be corrected by creating a new urethra and rotating the corporal
bodies to give the penis its more normal orientation.
Chordee
- What is chordee?
A chordee is a curvature of the penis. Some children are born with a chordee without
associated hypospadias or epispadias.
- How is a chordee treated?
Correction of this abnormality can usually be done on an ambulatory basis by surgically resecting
the offending chordee tissue and/or placing plicating sutures in the penis.
Male/Female
Genitalia
- What causes children to develop ambiguous appearing genitalia?
During development, both the male and the female external genitalia arise from the same
common primitive structures. Their development into clearly recognizable male and female
genitalia can be disrupted by any abnormality of chromosomes, gonadal problems or
enzymatic disorders. Therefore children with XY genes can appear to have female genitalia
and conversely children with XX genes can appear to have male genitalia.
- How can this be treated?
Many of these conditions are amenable to surgical reconstruction and ultimately
fulfillment of sexual and fertility potential. These disorders require close and
immediate consultation between the pediatric urologist, pediatric endocrinologist,
geneticist and neonatal intensive care physician to determine what possible abnormalities are seen in the genes, hormones, and anatomy.
Undescended
testicles
- What are undescended testicles?
One of the most common conditions that the pediatric urologist deals with is the
undescended testicle. The testicle originally forms in the retroperitoneum (the back part of the abdomen) and then
descends to the inguinal canal and then into the scrotum. Nearly 3 percent of all children are
born with an undescended testis however a good portion of these children will have the
testis descend into the scrotum within the first year of life.
- What are the treatment options for undescended testes?
If the testis has not descended by the first 6-12 months of life it is unlikely to do
so. In addition, the testis, which functions and grows better in its
scrotal position, begins to undergo damage as early as one year of life if left outside of
the scrotum. We therefore recommend surgical correction of undescended testes at 6 to 12 months of age.
A non-palpable testis represents one of three scenarios:
- the testis is actually in the inguinal canal and for one reason or another cannot be
palpated
- the testis is inside the abdomen
- the testis does not exist on that side
Our preference to differentiate between these three possibilities is to perform
laporoscopy followed by the appropriate procedure (i.e. orchidopexy vs removal of
testicular remnant) in the same sitting. Many of the intra-abdominal testes can be brought
down into the scrotum via laporoscopy alone. If cryptorchidism is corrected, fertility potential should approach that of the general
population. Frequent testicular examinations are required to check for possible testis tumors.
Inginual hernia
- What is an inguinal hernia?
Inguinal hernias occur quite commonly in the pediatric age group. These hernias differ
from adult hernias in that the peritoneum (the sack that encompasses the intestines) fails
to seal. Therefore communication exists between the abdomen and the scrotum.
- What are the signs and symptoms of a hernia?
There is a bulge in groin or scrotum that often changes in size.
- How is a hernia treated?
Surgery: Inguinal hernias are treated by closing the patency with a surgical procedure. The
recurrence rate is minimal. This surgery is done under general anesthesia on an
ambulatory basis.
Hydrocele
- What is a hydrocele?
A hydrocele is a collection of fluid along the membrane covering the front and sides of
the testicle.
- What is the difference between a hernia and a hydrocele?
If the fluid from the peritoneum travels through this opening then the child has a
hydrocele. If intestinal contents travel into the scrotum then the child has a hernia.
They are essentially the same pathologic condition. They differ from adult hernias which
are usually a disorder that arises from muscle weakness.
- How is a hydrocele treated?
Surgery: Like a hernia, hydroceles are very easily treated by simply closing the
patency and the recurrence rate is minimal. This surgery also can be done under general
anesthesia on an ambulatory basis.
For more information or to make an
appointment, please contact
Daniel B. Herz, M.D.
Department of Urology
5 E. 98th Street, 6th Floor
New York, NY 10029
Tel: (212) 241-7439
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