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50 Cards in this Set

  • Front
  • Back
What is vesicoureteral reflux? Is VUR deleterious in inself?
-VUR represents the retrograde flow of urine from the bladder into the upper urinary tract.
-No, reflux in itself that is without bacterial contamination and low in pressure has not been documented to
be injurious. However, reflux in the presence of bacteria is a risk factor for upper urinary tract infection or
pyelonephritis, as it accelerates bacteriuria by delivering infected urine to the renal pelvis.
-Sterile urine is benign, which is the basis of current medical management to maintain urine sterility until
reflux resolves.
What triad of clinical findings constitutes reflux nephropathy?
-renal scarring
-hypertension
-VUR
In discussing the epidemiology of VUR, what demographic factors affect prevalence and in what way?
i. Age- prevalence of reflux correlates inversely with the age of the study population. With linear growth,
spontaneous resolution of reflux occurs in many patients.
ii. Race- VUR is more commonly a disease of fair-skinned individuals. Prevalence is significantly lower
among AA children and children of Mediterranean origin. Several studies have shown a relative 10-fold
lower frequency of reflux in female children of african descent, with resolution of VUR sooner in
this population as well. Hispanic and Caucasian children have an almost equal incidence.
iii. Sibling predisposition- siblings of patients with known reflux have approximately a 30% prevalence
of reflux, with younger siblings being at greatest risk.
iv. Gender- difficult to assess because of the epidemiology of UTIs in children, as boys and girls may
present with reflux at different ages. Because UTIs are more common in uncircumcised boys than girls
during the neonatal period, many boys are diagnosed with VUR as neonates. However, after the first year
of life, the incidence of UTIs is much higher among girls than boys. Therefore, most school-aged children
diagnosed with reflux are girls.
With what genitourinary anomalies has VUR been shown to occur?
i. Posterior urethral valves-congenital bladder outlet obstruction has been associated with reflux in up to
50% of patients.
ii. duplicated collecting system-reflux is commonly associated with the lower pole moiety of a duplicated
system (Weigert-Meyer)
iii. Prune-Belly syndrome
iv. UPJ obstruction
v. bladder (paraureteral diverticula)
vi. MCDK/Renal agenesis
vii. Megacystis-Megaureter association
What is the traditional classification system for defining reflux? How may the typical patient present?
-PRIMARY reflux: results from a congenital deficiency in the formation of the UVJ, in the absence of any
other predisposing pathology.
- these patients reflux despite an adequately low-pressure urine storage profile in the bladder. May have a
laterally ectopic ureteral orifice consistent with a deficient submucosal ureteral tunnel, or low ureteral
tunnel length-diameter ratio. Lack of submucosal or intramural ureteral length prevents the terminal ureter
from closing like a flap valve when the bladder fills.
- these patients are the vast majority represented in clinic, otherwise healthy children who present with
symptomatic UTIs.
-SECONDARY reflux: occurs as a result of other urinary tract dysfunction, which leads to a
decompensation of a normally formed UVJ. Successful treatment of these patients depends on
identification of the underlying etiologies, such as:
i. Neurogenic bladder:
-myelomeningocele/spina bifida: look for classic PE findings such as sacral dimple, hairy patch,
gluteal cleft abnormalities, diminished rectal tone
-spinal cord injury: ask about constipation
ii. Obstruction:
-voiding dysfunction: uninhibited bladder contractions, early attempts to suppress bladder
contractions during incomplete emptying
-PUV: note that reflux is present in 48-70% of patients with PUVs.
-ectopic ureteroceles, prolapsing ureteroceles into the bladder neck in females.
iii. Infection: cystitis may also predispose an otherwise marginally competent UVJ to
demonstrate reflux.
T/F: Ditropan cures VUR.
True, in some cases. In older girls with uninhibited bladder contractions, a study showed that ditropan can
eliminate reflux in up to 80% of refluxing ureters
Describe the grading system of VUR, based on radiographic contrast images generated by VCUG
Grade I: Into nondilated ureter
Grade II: Into pelvis and calyces without dilatation
Grade III: Mild to mod. dilation of ureter, renal pelvis, calyces with minimal blunting of fornices
Grade IV: Moderate ureteral tortuosity and idlation of pelvis and calyces
Grade V: Gross dilation of ureter, pelvis, calcyes with loss of papillary impressions and ureteral tortuosity
Which children should be evaluated for VUR?
Children < 5yo
All children with a febrile confirmed UTI
Any male with UTI regardless of age or fever unless sexually active
What are the two gold standards for detecting reflux in children and what are the advantages and
disadvantages of each?
VCUG and radionuclide cystogram (RNC). VCUG is better to show anatomic detail (such as PUV, bladder
trabeculation. RNC exposes the child to only 1% of the radiation from a VCUG. However, it is unreliable for
Grade I VUR as the overlay of the bladder obscures.
Due to a scheduling snafu, you are called by Doris in the Peds Urology clinic to come evaluate a child for
possible reflux, even though Dr. Sutherland is out of the country. He has a documented urinary tract infection, is
febrile, and mom is asking whether he should have a VCUG study today. You are not sure and
decide to call Dr. Sutherland, who takes your call while sunbathing in his speedos off the warm waters of Pompeii.
What is his answer to you?
-Never call me again, that's why I have a Peds Chief. No to the VCUG-though the study should occur at 1-2 weeks following the acute episode.
-the likelihood of detecting reflux is highest if the child is evaluated soon after the infection. It is believed
that the presence of recent infection in the bladder predisposes a marginally competent UVJ to reflux, which may be missed if the delay to VCUG is long.
What other studies may be helpful in the diagnosis and management of reflux? How?
i. Urodynamics- many children with reflux have voiding dysfunction. The diagnosis is often suspected by a
history of incontinence, frequency or urgency. Appropriate management of the voiding dysfunction often results in
resolution of the reflux.
ii. Renal ultrasound- useful as an adjunctive technique in the grading of hydronephrosis and as a baseline
for follow-up studies to monitor renal growth. Other important details on U/S include the degree of corticomedullary
differentiation or increase in echogenicity of the kidney.
iii. Nuclear medicine renal scan (DMSA) and SPECT study: useful in the detection of renal cortical scars,
sequelae of repeated infections. DMSA which is a radiotracer that is taken up by renal tubules and is indicate of
glomerular filtration. SPECT is a 3-D reconstruction which only slightly increases the sensitivity of the DMSA
(which is already 98%)- does not add much to your management strategy
What is the best initial therapy for a 4-year old girl with newly identified grade II left VUR?
Because low and moderate grade reflux (I-III) has a good chance for spontaneous resolution, antibiotic
prophlaxis and observation is the best initial therapy for this child. Approximately 80% of patients with low-grade
reflux will have spontaneous resolution, and more than 50% of patients with grade III reflux will have resolution.
Grade IV reflux has a 10% chance of resolution, and grade V reflux rarely resolves spontaneously.
List three strong indications for considering surgical correction in a child with low-grade VUR?
-breakthrough infections or persistently positive urine cultures indicate that antibiotic prophylaxis is not working
and surgical correction should be considered.
-non-compliance with medical therapy
-anatomic issues such as a periureteral diverticulum or grade V reflux, due to the low likelihood of spontaneous
resolution.
You are in Dr. Sutherland’s clinic seeing a new patient with recurrent febrile UTIs who had a VCUG today. It
shows Grade IV VUR but also, the delayed post void films show retained contrast in the kidney. What do you think
is going on and how will you go about fixing this?
The patient has reflux but also possible concomitant UPJ obstruction. After confirming the presence of UPJ
obstruction with lasix scan, you would want to fix the UPJ obstruction first and then later fix the UVJ so as to
avoid further obstruction which may ensue if resistance added to UVJ when reflux is corrected. VUR is present
in 9-18% of those with UPJ obstruction.
What are the three radiologic signs of concomitant UPJ obstruction and VUR?
Pelvis shows little or no filling but ureter is dilated suggesting kinking from reflux.
Contrast that does enter the pelvis is poorly visualized because of dilution in large pelvic volume
Large pelvis fails to exhibit proper drainage but retains contrast
How can ultrasound RI values be helpful to you in suspecting reflux or infection?
Increased RIs are often associated with high grades of VUR as well as in cases of upper tract UTI (versus lower
tract)
You have a patient you are following with Grade III VUR and recurrent UTIs who is on
prophylactic antibiotics. She returns today with a follow-up renal ultrasound. What three findings
on renal ultrasound will make you suspicious of renal impairment?
Decrease in corticomedullary differentiation
Hyperechogenicity of the kidney
Absence of interval renal growth
You are consulted in the NICU for a child with a renal ultrasound showing renal agenesis. What is the next
diagnostic test you will order and why?
VCUG to check for contralateral VUR because it is associated with this in 46% of cases
What indicators will assist you in informing parents of the likelihood of their child’s VUR resolution? What age
will you quote at time of resolution and why?
Lower grade VUR (Grades I and II) are more likely to resolve. Also, more likely to resolve at a younger age.
Most resolve by age 5 because this is the time it takes for the UVJ to fully mature. Grade III reflux will
resolve in approximately 50% of cases, higher grades (bilateral III, grade IV and V) less likely
You have a patient who you have placed on prophylactic antibiotics for VUR who returns to your clinic. The
mother tells you that his pediatrician had drawn a CBC showing leukopenia and she is concerned. What is your first
thought?
The patient could be on TMP/SMX which can cause a transient leukopenia. You should discontinue the drug
and it should resolve on its own.
What is the first-line treatment for VUR?
Prophylactic antibiotics nightly (amoxicillin is choice in <2mo and TMP/SMX is choice in >2mo). Macrobid is
another option
What is one way to interpret breakthrough infections based on sensitivities?
If sensitive to prophylactic antibiotic, then most likely dose is too low or patient is noncompliant. If resistant,
then dose is too high or bladder is too full for too long
Name the 6 principles common to surgical reflux correction
Exclusion of causes of secondary VUR
Adequate mobilization of distal ureter without tension or damage to blood supply
Creation of submucosal tunnel generous in caliber with 5:1 length:width ratio
Attention to entry point of ureter into bladder, direction of submucosal tunnel, and ureteromucosal anastomosis to
prevent stenosis, angulation or twisting of ureter
Attention to muscular backing of ureter to achieve effective antireflux mechanism
Gentle handling of bladder to reduce postop hematuria and bladder spasms
Which reimplantation procedure is the most commonly performed and why? What are its drawbacks?
Cohen cross-trigonal because it is considered the easiest to perform and is also best for small and trabeculated
bladders and also overcomes the problem of tunnel length. Drawbacks include problems in the future with
retrograde access for stone procedures and stent placement
After performing a Politano-Leadbetter reimplantation on a child 2 weeks ago, a pediatrician calls you with the
report of moderate hydronephrosis on a renal ultrasound that he ordered. Should you immediately call the patient
into clinic?
No- if the patient is asymptomatic, it is not uncommon to see mild hydro up to even 2-3 months after surgery
secondary to inflammation
How does the Politano-Leadbetter and Paquin techniques differ?
Politano-Leadbetter is exclusively intravesical while Paquin is a combined intra and extravesical approach
What is the main advantage of performing an extravesical approach reimplantation? What is a common
complication from performing this procedure and does it resolve?
Main advantage is minimal to no hematuria and decreased bladder spasms postoperatively. However, a
common complication is postoperative transient voiding inefficiency in about 20%. Boys < 2yo with highgrade
bilateral VUR are at highest risk. Most resolve within 2 weeks with foley catheter left in place
What is the "high reimplant" phenomenon?
Angulation of the ureter occurs at the point of entry into the new hiatus secondary to a hiatus that was
positioned too far laterally and anteriorly such that when the bladder fills the ureter is carried laterally and
anteriorly resulting in difficulty draining when the bladder is full.
What procedure may effectively manage VUR before surgical option is entertained? How does it work and is
follow-up imaging recommended?
Deflux, which has achieved high success rates in lower grade VUR. Deflux substance seems to recreate the
natural intravesical tunnel between the ureter and bladder. Upper tract imaging with an U/S 4-6 weeks after the procedure is prudent.
What are the four main categories of megaureter? If you see megaureter on ultrasound in a newborn infant,
which type is most likely? (see Figure 117-23, p. 3468)
Obstructive, Refluxing, Non-obstructive, non-refluxing (most likely in infant), Obstructive & refluxing. Each
category is then divided into primary and secondary etiology
How should megaureter be evaluated?
Start with renal ultrasound, then VCUG to see if reflux is present. Also, obtain a lasix renal scan to assess
function and obstruction
How should primary nonrefluxing megaureter be treated?
Watchful waiting if no symptoms or UTI. If hydroureteronephrosis is severe and recurrent UTIs with
documented decrease in renal function, consider surgery
If you have an infant with severe hydroureteronephrosis as well as worsening renal function, what should you
do?
After obtaining your studies (including renal ultrasound and VCUG) to confirm the absence of a correctable
cause such as PUV, take the baby to the OR for a cutaneous ureterostomy to temporize them until they are old
enough for a reimplantation procedure
What are two ways to manage the severe dilation of MGU when attempting a reimplantation procedure?
Plication or infolding (if moderately enlarged ureter) whereas excisional tapering is useful for thick and/or
severely dilated ureters. Be sure to leave a stent in place for 5-7 days.
Describe the three types of posterior urethral valves and which is the most common?
-Type I is the most common type (95% of cases) and involves a ridge at floor of urethra, continuous with
the veru montanum- takes an anterior course and divides into two fork-like processes in region of
bulbomembranous junction.
• -Type II is really not a valve but instead a fold and is non-obstructive, arises from veru (extending along
posterior urethral wall toward bladder neck) and is most likely from hypertrophy of trigone muscle.
• -Type III constitutes 5% of valves and involves a membrane which lies transversely across urethra with
small perforation near its center, distal to veru, sometimes elongated
What is the most common cause of mortality in valve patients?
What is the most common cause of mortality in valve patients?
If the bladder is adequately drained on day of life one, do valve kids have a good chance of having normal kidney
and bladder function?
No. The majority will not void normally, due to poor sensation, hypercontractility, low compliance and eventual
incontinence. Also, most will have kidney damage, due to a variety of factors including infection, persistent
obstruction, HTN, and hyperfiltration of damaged parenchyma. Tubular damage will result in failure to
concentrate/acidify the urine and patients. Most damage is done during early fetal development
What are the two types of kidney damage resulting from PUV? Which is worse?
One type of damage is secondary to persistent high pressures (obstructive uropathy) and the other is from renal
dysplasia (from increased pressure during development of the kidney or abnormal embryologic development. Renal
dysplasia is worse because it is irreversible and also limits renal growth and development.
Describe the “pop-off” mechanism and the various types of pop-off valves. Does this constitute a good prognosis?
The pop-off mechanism involves a unilaterally refluxing kidney (nearly 90% on the left) which allows the high
pressures from the valve to transmit the pressures to the refluxing kidney primarily, sparing the contralateral kidney.
Bladder diverticula, urinomas, and urinary ascites are also considered pop-off mechanisms.
What are some radiologic clues suggestive of PUV on renal ultrasound? What are some clinical findings found on
examination of PUV patients?
-ultrasound findings include marked hydroureteronephrosis (>90% of PUV cases), a distended/thickened bladder,
keyhole sign (dilated bladder and prostatic urethra) and hyperechogenicity of the kidney (not seen in MGU)
-patients are often cyanotic and require respiratory support at delivery, due to pulmonary hypoplasia. Pulmonary
hypoplasia is a direct result of oligohydramnios and accounts for most mortality associated with PUVs.
-renal insufficiency/azotemia and fulminant sepsis may be present, due to long-standing obstruction and renal
dysplasia
- patients may have signs of FTT, lethargy, poor feeding and classic features of Potter’s (crushed baby) syndrome
such as deformed limbs. Urinary ascites may also be obvious.
What radiologic studies are utilized in the diagnosis of PUV?
-ultrasound (note clues above)
-VCUG: gold standard study, as it defines the anatomy and gross function of the bladder, bladder neck and urethra.
VCUG also allows identification of the numerous anomalies commonly associated with valves, including VUR
which is present in almost 50% of PUV patients.
- MAG3 scan, providing functional data/differential renal function.
You are called to consult on a NICU baby born today with PUV suggested on VCUG. The intern tells you that the
infant’s renal function appears to be normal, suggested by today’s low creatinine. Do you agree with this
interpretation? Why or why not?
-No. Creatinine in the first 48 hours is indicative of the mother since it is mediated through the placenta. You should
tell the intern to get electrolytes twice daily for the first several days until the numbers plateau. Especially important
are Na, K and bicarbonate.
What is the mainstay of treatment of PUV?
-Drain the bladder with a 3.5 or 5-French catheter immediately. Then, attempt valve ablation with a pediatric
resectoscope, laser or Bugbee electrode. If this is unsuccessful or unable to be performed because of patient small
size, perform a temporary vesicostomy and incise the valves later when the patient is older and healthier. If this fails,
then upper tract procedure (such as ureterostomy).
What is valve bladder syndrome? How may it be managed?
-a chronic condition in patients with valves in which, despite successful valve ablation, intrinsic bladder dysfunction
leads to deterioration of the upper urinary tracts and incontinence.
- findings include combination of poor sensation, high bladder volumes, poor compliance, and incontinence. These
produce storage pressures high enough to prevent adequate drainage of the upper tracts.
-initial management consists of timed voiding but if this does not successfully lower pressures and empty the
bladder, alpha blockers or CIC can be used.
What are the three urodynamic findings in boys with valves? Describe their treatment and expected age
associations.
-Low compliance/capacity: Infants. Anticholinergics, augmentation cystoplasty.
• -Detrusor hyperreflexia: Older children. Anticholinergics is mainstay of treatment
• -Myogenic failure: Adolescents. Timed voids, double voiding, alpha blockers
• These three are considered to overlap and represent stages of development.
When performing prenatal ultrasound to diagnose possible PUV, what is one important consideration to ensure
that the diagnosis is not missed?
Be sure that the scan is done after 24 weeks (before that, the diagnosis can be missed).
What are the four prognostic factors indicative of renal function with PUV children?
Ultrasound appearance: Amount of dysplasia- hyperechogenic
• Chemistries: Nadir of <0.8mg/dL in first year of life is good prognosis
• Age at Diagnosis: Now believed that older presentation is ? worse prognosis
• Presence of Reflux: Bilateral VUR is poor prognostic indicator
What are anterior urethral valves?
They are believed to occur as a diverticulum of the urethra- usually as a defect in the corpus spongiosum which
allows the urethra at this point to bulge out causing obstruction.
Where are congenital urethral strictures usually found and what is the treatment of choice?
Usually found in the posterior urethra. Treatment is vesicostomy with later balloon dilation of the stricture.
What is often confused with urethral polyps and how can you tell the difference?
Often confused with prostatic rhabdomyosarcoma but urethral polyps are usually solitary whereas rhabdo is multiple
and extend beyond the bladder.