HYDRONEPHROSIS

INTRODUCTION
Hydronephrosis develops when the kidneys are stretched from being filled with too much
urine. It mostly occurs in babies born with abnormalities that lead to the condition, although the
cause of these anomalies has not been found. It can also occur in childhood and affects boys five
times more often than girls. The severity of hydronephrosis ranges from mild to life threatening.
DEFINITION
Hydronephrosis is defined as distention of the renal calyces and pelvis with urine as a
result of obstruction of the outflow of urine distal to the renal pelvis. Analogously, hydroureter is
defined as a dilation of the ureter.
The presence of hydronephrosis can be physiologic or pathologic. It can be secondary to
obstruction of the urinary tract, but it can also be present even without obstruction. Obstructive
uropathy refers to the functional or anatomic obstruction of urinary flow at any level of the
urinary tract. Obstructive nephropathy is present when the obstruction causes functional or
anatomic renal damage.
INCIDENCE
Hydronephrosis is a relatively common congenital condition, with some estimates
showing it affects about 1 in 500 babies. Its often spotted on routine prenatal ultrasounds. Less
often, it goes undetected until an older child begins to experience symptoms.
CAUSES
There are two main types of problems that cause hydronephrosis. One isobstruction,
where urine is physically prevented from draining out of the kidney. The obstruction, or
blockage, can be located at any point in the urinary system, from the kidney down to the urethra.
The second is reflux, in which urine flows back up into the kidney.
Obstructive uropathy refers to the functional or anatomic obstruction of urinary flow at
any level of the urinary tract. Obstructive nephropathy is present when the obstruction causes
functional or anatomic renal damage. obstruction in the urinary tract may be acute or chronic,
unilateral or bilateral.
Types of obstruction
Ureteropelvic junction obstruction (UPJ): A blockage at the point where the kidney joins the
ureter (the thin tube that carries urine to the bladder). Usually, this is caused by a narrowing at
the top of the ureter.
Ureterovesical junction obstruction (UVJ): A blockage at the point where the ureter joins the
bladder.
Posterior urethral valves (PUV): A congenital condition, found only in boys, in which there
are abnormal flaps of tissue in the urethra (the tube that carries urine out of the body) that
obstruct the bladder. Also associated with vesicoureteral reflux, below.
Ureterocele: A bulge in the ureter that can obstruct part of the kidney and sometimes the
bladder.
Types of reflux
Vesicoureteral reflux (VUR): A backwash of urine that happens when the muscles at the
junction of the ureter and bladder arent working properly, and allow urine to flow back up
toward the kidney.
Vesicoureteral reflux (VUR): A backwash of urine that happens when the muscles at the
junction of the ureter and bladder arent working properly, and allow urine to flow back up
toward the kidney.
It is usually detected in the fetus in the first trimester of pregnancy, though it can also be
discovered later in the pregnancy or after birth. It is most commonly caused by narrowing of the
ureter close to the kidney; blockage in the kidney or bladder; or vesicouretal reflux, in which a
faulty valve between the bladder and the ureter causes the urine to back up into the kidney when
the bladder fills or empties. In more rare cases, antenatal hydronephrosis can result from a
duplication anomaly (two ureters leading from the kidney, one of which may be obstructed) or a
multicystic dysplastic (nonfunctional) kidney.
There are no known risk factors. Boys are about four to five times more likely to be born
with hydronephrosis than girls.

PATHOPHYSIOLOGY
Hydronephrosis can result from anatomic or functional processes interrupting the flow of
urine. This interruption can occur anywhere along the urinary tract from the kidneys to the
urethral meatus. The rise in ureteral pressure leads to marked changes in glomerular filtration,
tubular function, and renal blood flow. The glomerular filtration rate (GFR) declines
significantly within hours following acute obstruction. This significant decline of GFR can
persist for weeks after relief of obstruction. In addition, renal tubular ability to transport sodium,
potassium, and protons and concentrate and to dilute the urine is severely impaired. The extent
and persistence of these functional insults is directly related to the duration and extent of the
obstruction. Brief disruptions are limited to reversible functional disturbance with little
associated anatomic changes. More chronic disruptions lead to profound tubular atrophy and
permanent nephron loss.
Increased ureteral pressure also results in pyelovenous and pyelolymphatic backflow.
Gross changes within the urinary tract similarly depend on the duration, degree, and level of
obstruction. Within the intrarenal collecting system, the degree of dilation is limited by
surrounding renal parenchyma. However, the extrarenal components can dilate to the point of
tortuosity.
To distinguish acute and chronic hydronephrosis, one may consider acute as
hydronephrosis that, when corrected, allows full recovery of renal function. Conversely, chronic
hydronephrosis is a situation in which the loss of function is irreversible even with correction of
the obstruction. Early experiments with dogs showed that if acute unilateral obstruction is
corrected within 2 weeks, full recovery of renal function is possible. However, after 6 weeks of
obstruction, function is irreversibly lost.
Grossly, an acutely hydronephrotic system can be associated with little anatomic
disturbance to renal parenchyma. On the other hand, a chronically dilated system may be
associated with compression of the papillae, thinning of the parenchyma around the calyces, and
coalescence of the septa between calyces. Eventually, cortical atrophy progresses to the point at
which only a thin rim of parenchyma is present. Microscopic changes consist of dilation of the
tubular lumen and flattening of the tubular epithelium. Fibrotic changes and increased collagen
deposition are observed in the peritubular interstitium.


CLINICAL MANIFESTATIONS
Pediatric hydronephrosis and hydroureter
Fetal hydronephrosis is a readily diagnosed finding on antenatal ultrasound examination
and can be detected as early as the 12th to 14th week of gestation.
Although renal pelvic dilatation is a transient, physiologic state in most cases, urinary
tract obstruction and vesicoureteral reflux (VUR) are also causal. Most cases of antenatal
hydronephrosis are not clinically significant and can lead to unnecessary testing of the newborn
baby and anxiety for patients and healthcare providers.
Physical
Pain in the abdomen
Pain in the side (also called flank pain)
Blood in the urine (hematuria)
Urinary tract infection (UTI)
With severe hydronephrosis, the kidney may be palpable.
With bilateral hydronephrosis, lower extremity edema may occur. Costovertebral angle
tenderness on the affected side is common.
A palpably distended bladder adds evidence of lower urinary tract obstruction.
A digital rectal examination should be performed to assess sphincter tone and to look for
hypertrophy, nodules, or induration of the prostate.
In children, the physical examination, especially in a newborn, can help detect
abnormalities that suggest genitourinary abnormalities associated with antenatal hydronephrosis.
These include the following:
The presence of an abdominal mass could represent an enlarged kidney due to
obstructive uropathy or multicystic dysplastic kidney (MCDK).
A palpable bladder in a male infant, especially after voiding, may suggest posterior
urethral valves.
A male infant with prune belly syndrome will have deficient abdominal wall
musculature and undescended testes. The presence of associated anomalies should be
noted.
The presence of outer ear abnormalities is associated with an increased risk of
congenital anomalies of the kidney and urinary tract (CAKUT).
A single umbilical artery is associated with an increased risk of CAKUT, particularly
VUR.

DIAGNOSIS
Laboratory Studies
- Urinalysis is used to assess for signs of infection. Pyuria suggests the presence of
infection. Microscopic hematuria may indicate the presence of a stone or tumor.
- Complete blood cell count may reveal leukocytosis, which may indicate acute infection.
- Serum chemistry studies can reveal an elevation of BUN and creatinine levels, which
may be the result of bilateral hydronephrosis and hydroureter. In
addition,hyperkalemia can be a life-threatening condition.
Imaging Studies
Prenatal
Detection of antenatal hydronephrosis by ultrasound usually occurs in the second
trimester with a renal pelvic dilation (RPD) cutoff of greater than or equal to 4 mm. Mild
hydronephrosis (RPD of 4-10 mm or Society for Fetal Urology [SFU] grade 1 or 2) can be
associated with Down syndrome or other chromosome anomalies. More severe dilatation
increases the risk of renal and/or urinary tract disorders.
During the ultrasonography, the appearance of the fetal renal system can vary in both
healthy fetuses without hydronephrosis and those with hydronephrosis. Therefore, this diagnosis
should not be based on a single measurement.

An increase of maternal hydration can also
increase the RPD in both healthy fetuses and those with hydronephrosis.
If fetal hydronephrosis is detected, the following parameters need to be evaluated using
ultrasonography, as they guide further need for evaluation and are helpful in determining the
cause of hydronephrosis:
Severity of hydronephrosis: The likelihood of a congenital kidney or urinary tract
anomaly increases with the severity of RPD.
Unilateral versus bilateral involvement: Bilateral involvement increases the risk of a
significant renal abnormality and the risk of impaired postnatal renal function.
Ureter: Dilatation of the ureter can be consistent with vesicoureteral reflux (VUR) or
obstructive uropathy distal to the ureteropelvic junction.
Postnatal radiologic studies
Postnatal radiologic evaluation of a newborn with antenatal hydronephrosis begins with
an ultrasonography examination. The timing of ultrasonography and the need for other studies
depend on the severity of postnatal hydronephrosis and whether there is bilateral involvement or
an affected solitary kidney.
Ultrasonography of kidneys and bladder should be performed in the postnatal period on
affected infants. The timing of the study depends on the severity of the antenatal hydronephrosis.
In general, examination should be avoided in the first 2 days after birth because hydronephrosis
may not be detected because of extracellular fluid shifts that underestimate the degree of
hydronephrosis. However, infants with bilateral hydronephrosis and those with a severe
hydronephrotic solitary kidney require urgent evaluation on the first postnatal day because of the
increased likelihood of significant disease and a possible need for early intervention. For
unilateral hydronephrosis without antenatal bladder pathology, performing postnatal sonography
1-4 weeks after birth is recommended.
A voiding cystourethrography (VCUG) is performed to detect VUR and, in boys, to
evaluate the posterior urethra. For this procedure, a urinary catheter is inserted into the bladder
and contrast material is instilled. Fluoroscopic monitoring is performed while the bladder is
filling and during voiding. Infants usually tolerate this procedure well. Although the duration of
fluoroscopy is minimized, the gonads, especially the ovaries, are exposed to radiation.
[

Diuretic renography is used to diagnose urinary tract obstruction in infants with
persistent hydronephrosis and is usually ordered after a VCUG has demonstrated no
vesicoureteral reflux.

It measures the drainage time from the renal pelvis and assesses total and
individual kidney renal function. The test requires insertion of a bladder catheter to relieve any
pressure that can be transmitted to the ureters and kidneys. Intravenous access is needed for
hydration and the administration of the radioisotope and diuretic. The preferred radioisotope is
technetium Tc 99m-mercaptoacetyltriglycine (Tc99mMAG3), which is taken up by the renal
cortex, filtered across the glomerular basement membrane to the renal tubules, and excreted into
the renal pelvis and urinary tract.
Diuretic renography includes the following 2 phases:
First, radioisotope is injected intravenously and renal parenchymal (cortical) uptake is
measured during the first 2-3 minutes. The relative contribution of each kidney to
overall renal function (called the split renal function) is assessed quantitatively and is
useful as a baseline study. Subsequent studies can be compared to assess whether
kidney function remains stable or has deteriorated, suggesting true obstruction.
Second, at peak renal uptake, intravenous furosemide is administered and the
excretion of isotope from the kidney is measured, referred to as the washout curve.
This phase indicates the extent of obstruction, if present. In a healthy kidney,
furosemide administration results in a prompt washout. In a dilated system, if
washout occurs rapidly after diuretic administration (< 15 min), the system is not
obstructed. If washout is delayed beyond 20 minutes, the pattern is consistent with
obstructive uropathy. However, a delayed washout must be interpreted with caution.
In a series of 39 infants with antenatal unilateral hydronephrosis followed without
surgery, diuretic renography indicated obstruction in 24 patients whose renal function
never decreased and thus did not have obstruction. These results may be due, in part,
to the normally low neonatal GFR that can be refractory to diuretic therapy. If
washout is from 15-20 minutes, the study is indeterminate.
Split renal function results are the most useful criteria to evaluate a decrease in renal
function. In patients with unilateral hydronephrosis (which is the most common clinical
scenario), if the normal nonhydronephrotic kidney and hydronephrotic kidney both have equal
function, conservative management without surgery is a safe option. In a cohort of 831 cases of
antenatal hydronephrosis, renal scanning performed in 229 newborns demonstrated 16% of
patients had a significant decrease in renal function of 1 kidney, defined as a less than or equal to
35% differential renal function. A decrease in differential renal function was associated with
severe antenatal hydronephrosis (ie, renal pelvic diameter >10 mm at 20-24 wk gestation and
>16 mm at 33 wk gestation).
Magnetic resonance urography (MRU) in children is becoming more commonly used
in the diagnosis and management of congenital uropathies such as UPJ obstruction. MRU is
especially useful in the management of obstructed kidneys that have rotation or ascent
anomalies, or are solitary. MRU can more clearly define the anatomy and delineate the proper
surgical approach (ie, retroperitoneal vs transperitoneal). The disadvantage of MRU is that they
study often requires general anesthesia or heavy conscious sedation in children. Furthermore, the
contrast agent gadolinium can only be used if renal function is normal (requiring a preprocedure
serum creatinine test) because of reports of irreversible renal fibrosis in patients with renal
insufficiency. Newer MRU technology may even define obstruction, eliminating the need for
diuretic renal scanning.
Procedures
Antegrade or retrograde pyelography is usually used to relieve, rather than diagnose, urinary
tract obstruction. These tests, however, can also be performed for diagnosis when the history is
highly suggestive (unexplained acute renal failure in a patient with known pelvic malignancy),
even though hydronephrosis may be absent (due to possible ureteral encasement) on
ultrasonography and CT scanning.

MANAGEMENT
The management approach of infants with antenatal hydronephrosis is based on the
confirmation of persistent postnatal hydronephrosis and the following 2 predicative factors.
Bilateral involvement
Severe hydronephrosis: Fetuses with a renal pelvic diameter greater than 15 mm during
the third trimester are at the greatest risk for significant renal disease.
Bilateral hydronephrosis
Infants with severe bilateral antenatal hydronephrosis and/or bladder distension are at
increased likelihood of having significant disease. These infants and those with a severe
hydronephrotic solitary kidney should be evaluated initially by ultrasonography on the first
postnatal day. Bilateral hydronephrosis suggests an obstructive process at the level or distal to
the bladder, such as ureterocele or posterior urethral valves (PUV) in a male infant, which can be
associated with impaired renal function and ongoing renal injury.If postnatal ultrasonography
demonstrates persistent hydronephrosis, voiding cystourethrography (VCUG) should be
performed. In male infants, the posterior urethra should be fully evaluated to detect possible
PUVs.
Infants with mild or moderate hydronephrosis can be evaluated after 7 days of life.

Severe unilateral hydronephrosis
In newborns with severe antenatal unilateral hydronephrosis (renal pelvic diameter >15
mm in the third trimester), ultrasonography should be performed after the infant returns to birth
weight (after age 48 h and within the first 2 wk of life).
Moderate and mild unilateral hydronephrosis
In newborns with less severe antenatal unilateral hydronephrosis (renal pelvic diameter <
15 mm during third trimester), ultrasonography can be performed after they reach age 7 days see
if the hydronephrosis is persistent postnatally.
Most patients with moderate hydronephrosis have resolution by age 18 months.
Antibiotic prophylaxis
Higher rates of urinary tract infections have been reported in children with prenatally
diagnosed hydronephrosis compared with the general pediatric population. The risk of infection
rises if there is an underlying urologic abnormality, such as VUR or obstructive uropathy, and is
greater in girls compared with boys.
As a result, in infants with severe hydronephrosis who are at greater risk for an
underlying urologic abnormality, antibiotic prophylaxis is started after delivery until the
diagnosis of VUR or obstructive uropathy is excluded.
Surgical Care
The specific treatment of a patient with hydronephrosis and hydroureter depends, of
course, on the etiology of the process. Several factors help determine the urgency with which
treatment should be initiated. In general, any signs of infection within the obstructed system
warrant urgent intervention because infection with hydronephrosis may progress rapidly to
sepsis. A mildly elevated white blood cell count is often observed in patients with stones but
does not necessarily mandate immediate action in the absence of other signs or symptoms of
systemic infection. However, even a low-grade fever in a diabetic or immunosuppressed patient
(ie, on steroid therapy) requires immediate treatment.
The potential for loss of renal function also adds to the urgency (eg, hydronephrosis or
hydroureter bilaterally or in a solitary kidney). Finally, patient symptoms help determine the
urgency with which treatment is initiated. For example, refractory pain in a patient with an
obstructing ureteral calculus necessitates intervention, as does intractable nausea and vomiting.
Urethral catheterization is important to help rule out a lower tract cause for
hydronephrosis and hydroureter. Difficulty in placing a Foley catheter may suggest urethral
stricture or bladder neck contracture.
Urologists commonly use ureteral stent placement in cases of intrinsic and extrinsic
causes of hydronephrosis. The procedure is usually performed in conjunction with cystoscopy
and retrograde pyelography. Stents can bypass an obstruction and dilate the ureter for subsequent
endoscopic treatment.
Urologists or interventional radiologists can place a percutaneous nephrostomy tube if
ureteral stenting is not possible. Usually, ultrasonography is used first to locate the dilated
collecting system. Using the Seldinger technique, a tube ranging from 8-12F can be placed.
Nephrostomies are typically placed when a retrograde stent cannot be passed because of
anatomic changes in the bladder or high-grade obstruction in the ureter. Because this procedure
can be performed under local anaesthesia, patients who are too hemodynamically unstable
for general anaesthesia may undergo percutaneous nephrostomy tube placement. In addition,
nephrostomy tube placement may be performed with minimal use of radiation and may be useful
in pregnant patients.
Advances in endoscopic and percutaneous instrumentation have decreased the role of
open or laparoscopic surgery for hydronephrosis. Certain causes of hydronephrosis, mostly
extrinsic, still require treatment with open surgery. Examples include retroperitoneal fibrosis,
retroperitoneal tumors, and aortic aneurysms. Some stones that cannot be treated endoscopically
or with extracorporeal shockwave lithotripsy require open removal. Although endoscopic
management does play a role in low-grade low-stage ureteral tumors, these lesions also usually
require open or laparoscopic surgical management.
Urine should be collected from the kidney when obstruction is relieved to allow
identification and targeted treatment of any infection that may be present.