Pediatric Voiding Dysfunction Flashcards
What tests would you order on a 6 yo with spina bifida (s/p back closure as baby, L5 myelomeningocele) who presents with worsening urinary incontinence, urgency, and recurrent UTI?
RBUS
VCUG
UDS
MRI spine
What would be your main dx for 6 yo with spina bifida (s/p back closure as baby, L5 myelomeningocele) who presents with worsening urinary incontinence, urgency, and recurrent UTI?
UDS shows small capacity bladder with intact sensation, poor compliance, uninhibited contractions, high DLPP, VUR
Tethered cord (MRI)
What is normal bladder capacity for a child?
Normal bladder capacity = (age +2) x 30 mL
What is DLPP and what is the upper threshold?
Detrusor leak point pressure – leakage in absence of valsalva or contraction
The detrusor leak point pressure (DLPP) is a measure of the pressure at which the detrusor muscle in the bladder begins to leak urine during the filling phase of the bladder.
The DLPP is measured during urodynamic testing, a diagnostic test used to evaluate bladder function. During the test, a catheter is inserted into the bladder and the bladder is filled with fluid. The pressure in the bladder is measured at various points, including when the detrusor muscle begins to leak urine. The DLPP is the pressure at which this leakage occurs.
sign of poor compliance
>40 cm H20 causes renal damage
Initial treatment for child with detrusor hyperreflexia, poor compliance, VUR, DLPP > 40 cm H20? Without tethered cord.
CIC + AC drug
CaP for VUR
Bowel regimen
Regular f/up UA, UCx, Renal US, Cr
What would be next steps for progression of bladder hostility, pyelonephritis, worsening hydro, or renal function loss?
Augmentation (+ reimplant if VUR)
Mitrofanoff, considered in boys
MACE
In a boy with spina bifida and UI (L4 myelomeningocele), what is initial workup of worsening or uncontrolled leakage?
RBUS
VCUG
UDS
+/- MRI
In a boy with spina bifida and UI (L4 myelomeningocele), RBUS demonstrates L hydro with loss of parenchyma, VCUG demonstrates Grade IV/V L VUR, bladder neck open, what testing would be indicated to assess kidneys?
DMSA
In a boy with spina bifida and UI (L4 myelomeningocele), RBUS demonstrates L hydro with loss of parenchyma, VCUG demonstrates Grade IV/V L VUR, bladder neck open, DMSA demonstrates 65%/35%, L/R.
UDS shows:
small capacity, overactive bladder (DO 30 cm H20, SUI, elevated PVR)
First tx options? If failed first options?
First options: CIC + AC, CaP for VUR
Failed non-invasive:
- Autoaugmentation with ureteral reimplant and bladder neck sling
- Augmentation enterocystoplasty (stomach, ileum, colon) with transvesical ureteral reimplant and sling (autologous or cadaveric vs. AUS)
- Continent vesicostomy (Mitrofanoff with bladder neck closure) + MACE
Complications and management of bladder augmentation:
- Autoaugmentation (detrusor myotomy):
- Damage to urothelium → repair 4-0 chromic
- Persistent bladder leak → continued urethral catheter
- Augmentation enterocystoplasty
- Contraction with incontinence → AC
- Mucus → flush
- Hematuria/dysuria syndrome → stomach only, H2 blocker, HCO3
- Stones → irrigation, extraction
- Spontaneous perforation → peritonitis and death (5-10%), first cath drainage, operate based on clinical suspicion (cystogram can be FN)
- Metabolic acidosis → stomach/colon, can lead to osteoporosis → tx oral NaHCO3 or Na/K Citrate
- Metabolic alkalosis → stomach, H2 blocker
- Malignancy → adenocarcinoma of bowel, or UC bladder, if new blood in urine or recurrent UTI do cysto, B12 deficiency (terminal ileum), pernicious anemia/neuropathy
In patient with lethargy, fever, and VP shunt, what should be done?
Consult NSG to r/o shunt failure
Describe open cystolithotomy on augmented bladder:
- prep and drape
- place foley
- lower midline incision
- place ring retractor
- incise detrusor
- avoid bowel portion of bladder
- remove stones
- close incision in layers
- leave SPT, Foley, drain
Increase interval of CIC and consider irrigation regimen
What are treatment options for 21 yo M with hx of L1 myelomeningocele with UDS demonstrating normal capacity and compliance, SUI but not UUI?
- Periurethral bulking (success low)
- Male sling (may need to CIC after)
- Leadbetter trigonal tubularization
- Tanagho bladder neck tubularization
- Kropp anterior bladder tube implantation
- AUS (revision 5-7 y)
Describe AUS:
- Dorsal lithotomy
- Inguinal incision for pressure reservoir (61-70 H2O used for bulbar urethra)
- Perineal incision for cuff placement → measure urethral circumference (4.5 cm common in bulbar urethra)
- Connect tubing
- Cycle the sphincter
- Lock the cuff in open position
- Leave foley overnight
- Activate at 6 weeks
If a patient has an AUS and develops hydronephrosis, worsening renal function, what is first step? What diagnostic testing is necesary?
First step it to ensure sphincter is working and deactivate it with cuff in open state, catheter to check PVR
US (done as mentioned in stem) VCUG ( to assess for VUR)
Need UDS
What is ddx of 15 yo M with enuresis and neurologic findings (decreased DTR, decreased rectal tone), not previously dx with any congenital issues? What tests should be ordered?
tethered cord
spinal dysraphism
Order: RBUS, KUB, VCUG, UDS, MRI spine
What are treatment options for 15 yo M with tethered cord and small capacity, overactive bladder with high DLPP and no SUI?
- Anticholinergics
- Timed voiding
- Bowel regimen
- CIC
- NSG for cord de-tethering
What surveillance is utilized for spina bifida patient with or without surgical intervention?
Yearly RBUS, Cr (cystatin C)
changes in sxs: UDS
UTIs/blood: cysto
If medication and cord de-tethering do not work for a poorly compliant, small bladder patient with incontinence, what are next steps?
Bladder autoaugmentation or enterocystoplasty
may need CIC
consider continent catheterizable channel
First goal of pediatric NGB management in babies with spinal dysraphism? What tests are done?
protect the kidneys from high bladder pressures and incomplete emptying
ID newborns at risk of renal damage
GET RBUS in first few days of life
* can consider contrast enhanced US
Look for:
hydronephrosis, parenchymal anomalies, bladder appearance
Consider VCUG (once patients can lie on back, once defect closed)
What workup should a baby with spinal dysraphism have in the early months after discharge from the hospital?
Urodynamics and a repeat renal bladder ultrasound are typically obtained around 2-4 months of age.
It is important to highlight that there are no reference values for bladder dynamics this early in life.
Several urodynamic findings have been associated with risk of UTI and kidney scarring including bladder trabeculations, VUR, high storage pressures evidenced by end fill pressure or detrusor leak point pressure > 40 cm H2O, and detrusor sphincter dyssynergia.
Initial CIC determination in infants is utilized until first UDS based on which factors?
consistently high bladder pressures or high residuals
trabeculated bladder
high grade VUR
high grade hydronephrosis
A 2-4 month old with urodynamics that is LOW risk for upper tract damage will have these features
A patient who is very low risk for upper tract damage on urodynamics will have
- a smooth walled bladder without VUR
- low bladder residuals
- low leak point pressures
For a baby’s first urodynamics, urodynamic findings have been associated with risk of UTI and kidney scarring include:
Bladder trabeculations
VUR
High storage pressures evidenced by end fill pressure or detrusor leak point pressure > 40 cm H2O
Detrusor sphincter dyssynergia
How do you decide if you should initiate CIC in a 2-4 month old?
If on urodynamics at 3 months, there is high end fill or detrusor leak point pressures (> 40 cm H2O), evidence of detrusor sphincter dyssynergia, or grade 5 VUR
in addition to q4h CIC, protocol can start patients on Oxybutynin 0.2 mg/kg TID
Spina bifida follow up in the first year of life
clinic visit with assessment for any interim UTIs and renal bladder ultrasound every 3-4 months,
with urodynamics often repeated around 1 year of age or sooner if clinically needed
After the 1st year of life, follow up visits are typically spaced out to every 6-12 months. The need for routine urodynamics after the 1st year of life is unclear. Some centers recommend a baseline DMSA scan to establish the presence of renal scars as these scars correlate well with development of hypertension. However, in patients with normal ultrasounds who do not have a history of UTIs, VUR, or trabeculated bladder the utility of routine DMSA scan is of questionable utility as these patients are unlikely to have any DMSA abnormalities.
What do you do for a “high risk” bladder in infancy and childhood?
Approximately 30% of children with spina bifida will have a high risk bladder.
A patient with a trabeculated bladder with VUR and high storage pressures has a very high risk of developing UTIs and renal damage.
Starting CIC and anticholinergics in these patients may prevent UTIs and renal damage.
The anticholinergic most commonly used is oxybutynin, 5mg/5mL elixir 0.2 mg/kg BID-QID.
All anticholinergics are off-label in neonates and children.
Early initiation of medical treatment has shown lower rates of renal function deterioration and need for bladder augmentation.
Side effects may limit dose adjustments. This can be reduced with intravesical use of anticholinergics. Intravesical oxybutynin can be dosed as 0.1 – 0.8 mg/kg TID. More recent literature supports the use of β3 agonists and has shown improvement in urodynamic parameters.
Overnight Foley catheter drainage can also be started to avoid high storage pressures by keeping the bladder empty all night as well as to help prevent UTIs.
When do you consider vesicostomy?
In infants or young children with neurogenic bladder who have persistent UTIs or concern for upper tract deterioration, despite regular CIC, anticholinergics, and overnight catheter drainage, early and strong consideration for vesicostomy should be given.
A vesicostomy is very successful in protecting the kidneys from the bladder and CIC and anticholinergics can generally be stopped. Risks of vesicostomy include bladder prolapse and stomal stenosis. Eventually, the vesicostomy is typically taken down and a bladder augmentation with other reconstructive surgeries can be done at the same time as vesicostomy take down.
We typically recommend waiting until at least age 5 before considering taking down the vesicostomy and obtaining a urodynamic study prior to this to evaluate for persistent of VUR, bladder size, and bladder compliance.
Management of VUR with ureteral reimplantation has not shown any benefit and should rarely be considered. Because these patients typically have high outlet resistance, a bladder outlet procedure is generally not needed at time of vesicostomy take down and augmentation cystoplasty with resulting high continence rates.
What are major risk factors for spinal dysraphism (spina bifida)
extreme maternal age
family history
maternal diagestes
obesity
use of perinatal valproic acid or carbamazepine
folic acid deficiency
What are the goals of pediatric neurogenic bladder management
- Protect the kidneys and prevent their deterioration resulting from high bladder pressures.
- Achieve continence.
- Achieve independence.
- Avoid reconstructive surgery if possible.
- Avoid or reduce frequency of symptomatic urinary tract infections.
What does anemia from renal insufficiency look like? Why does it happen?
microcytic
MCV value low/normal
reticulocyte low
low erythropoietin
4 categories of reconstructive surgery in patients with spina bifida
- Protect the kidneys and prevent their deterioration resulting from high bladder pressures.
- Achieve continence.
- Achieve independence.
- Avoid reconstructive surgery if possible.
- Avoid or reduce frequency of symptomatic urinary tract infections.
What is an appropriate dosing of botox for patient with spina bifida and NGB?
200-300 IU
not medical prob incontinence
- pri mono-sx noct enuresis
- behavioral d/o or developmental delay
nl time for toilet trainingn time
3-3.5 yrs
def primary incontenence
never continent or continent > 6 mo
def secondary incontenence
previously achieved continence - nuropathic or severe dysfunciton ultil proven otherwise
r/o traumatic events, abuse, dm, renal d/o
dysfunctional voiding def
active habitual contraction of urethral sphincter during voiding
observed by uroflow
hinnman syndrome
non-neurogenic neurogenic bladder
severe dysfxn voididng- bladd changes like neurogenic bladder
silent upper tract changes
assd w emotional trauma
post void leakage cause
vaginal reflux
urine trapped behind labia
small vol leaks 0-5 mins after standing to void
only aware they feel wet
can be radiographically impressive
tx - reposition on toilet, and blot vag
SUI differential in kids
covered exstrophy
female epispadias (missed)
sacral agenisis
enuresis risoria
giggle incontinence.
total loss of urine w/ intense emotion/ cataplexy.
Imbalance between cholinergic and monaminergic system
tx - methylphenidate (ritaline).
also have diurnal inctontenence - w/ and w/o laughter. Tx w/ std urge tx +/- anticholinergics
why does leakage in ureteral ectopia decrease overnight in girls
urine pools in vagina
ddx for u retention in peds
- constipation
- UTI
- bladder base tumor - RMS
- neurologic disease
myelomeningocele
5% void normally.
incomplete lesion –> cant predict voiding.
dynamics of bladder and sphincter can change over time
caudal regression
can have either UMN or LMN disease.
bladder neck incompetence.
external sphincter fixed (upper tract problems).
renal anomalies
conservative vs proactive medical mgmt of ngb
conservative - UT us –> intervene w CIC/meds if UT changes.
proactive - CMG risk dictates medical mgmt + renal us
concerning CMG findings for ut damage
> 40 cm h20 dlpp.
poor compliance.
neurogenic DO w/ DSD.
surgical mgmt options for ngb
- cutaneous vesicostomy (poor uds w max medical tx in infant or noncompliant parent).
- definitive surgical (UT deterioration refractory to medical mgmt, desire for continence).
urinary reconstruction for NGB
- enterocystoplasty 2. continent catheterizable channel 3. bladder outlet procedure, 4. +/- antireflux procedure (not done as often). +/- MACE
long term complications of enterocystoplasty
- mucous, 2. acidosis, 3. B12 deficiency obvious others
metabolic problem with urinary stasis
myperchloremic metabolic acidosis is worsened. Aggravated by renal insuff, possible bone demineralization
% risk of malig w augment
5%. No diff btw cic and enterocystoplasty, and cic alone.
highest risk of malignancy with augment
Highest risk in transplant, on immunosupression, and cic
dx of rethethering of SC
clinical dx. Not seen on imaging. 1. _ functional status (gait, strength). 2. _ continence/voiding (tough to notice if already wet). 3. new back/leg pain. 4. increased/febrile UTI 5. _ routine upper tract imaging
spinal dysraphism and imaging
all appear radiographically tethered. Looking for interval increase of syringomyelia or mass. And see if shunt has malfunctioned
suspect retethering for urologist
- verify CIC/ voididng/ meds 2. tx + urine culture, 3. bowel tuneup 4. cmg?
suspect rethethering - uds signs
- any _ from prior, 2. _ contractility, inc voiding pressure, 3. _ compliance, 4. _ DLPP (outlet _)
rethethering prognosis
some improvement, but return to baseline unlikely. Goal - 1. halt deterioration, manageable bladder. Amnt of improvement depends on duratino of change
circumcision and peds UTI
uncircumcised 10x more likely
host factors and peds UTI
- age/ gender, 2. periurethral colonization, 3. prepuce, 4. genetics/ bact adherence factors, 5. immune status, 6. anatomic anomaly, 7. pathologic flora
2011 aap uti guidelines - 1st febrile uti
2 mo- 2 yr - 1. cath specimen, 2. us @ acute phase, 3. vcug not required
VUR general stuff
VUR is only improtant in the setting of UTI - “bad” kidneys have more at stake. Hi grade VUR is exception
AUA VCUG 2010 guidelines
- general eval - ht, wt, bp, ua for protein/bacteria, 2. verify renal function - DMSA optional but rec for high grade, 3. followup us/cystogram @ 12-24 mo
AUA VCUG 2010 guidelines - prophylaxis
- < 1yoa, 2. prenatlly detected gr 3-5 3. bladder and bowel dysfunction.
AUA VCUG 2010 guidelines - intervention
breakthrough febrile uti
FIG. 6.1 Bristol stool chart with visuals and descriptions of different stool types. This scale provides a helpful, objective reference for documenting stool consistency when talking to patients about bowel function. Source: (Modified from Lewis SJ, Heaton KW. Stool form scale as a useful guide to intestinal transit time. Scand J Gastroenterol 1997;32(9):920-924.)
Table 6.1
Rome IV Diagnostic Criteria for Functional Constipation
FIG. 6.2 Management algorithm for childhood defecation disorders seen in a pediatric urology practice. Lack of improvement or intractable constipation should be diagnosed based on worsening or absence of suboptimal response to adequate medical treatment for at least 3 months. MACE, Malone antegrade continence enema.
FIG. 6.3 Urachal anomalies. (A) Patent urachus. (B) Urachal cyst. (C) Umbilical-urachus sinus. (D) Vesicourachal diverticulum.
FIG. 6.4 (A) Young’s original figures from his 1919 article describing three types of posterior urethral valves. (B) William P. Didusch illustrates the pathognomonic findings associated with posterior urethral valves: the thickened bladder with elevated bladder neck, dilated prostatic urethra, and the valve leaflets commonly ascribed to type 1 valves. The ureters are shown to be dilated. Source: (From Young HH, Frontz WA, Baldwin JC. Congenital obstruction of the posterior urethra. J Urol 1919;3:289.)
FIG. 6.5 Note small, irregular bladder, unilateral high grade vesicoureteral reflux and posterior urethral filling defect consistent with posterior urethral valves.
Image
FIG. 6.6 (A) Newborn female with classic bladder exstrophy: note the bifid clitoral halves, divergent labia and mons, as well as the anteriorly displaced anus and diastasis of pubic symphysis. (B) Newborn male: note the short, exposed urethral plate with dorsal curvature.
Image
FIG. 6.7 Pelvic bone abnormalities noted in classic bladder exstrophy. The posterior bone segment is externally rotated (12 degrees mean on each side), but the length is unchanged. The anterior segment is externally rotated (18 degrees mean on each side) and shortened by 30%. The distance between the triradiate cartilage is increased by 31%.
Image
FIG. 6.8 Prenatal ultrasound scan demonstrating bladder exstrophy. (A) Longitudinal view showing the low-set umbilicus ( cyan arrow, lack of intraabdominal bladder, and lower abdominal mass (red arrow). (B) Transverse view through the plane (X) in A shows presence of the umbilicus (cyan arrow) and the upper edge of the bladder plate that appears hyperechoic (red arrow).
Image
Image
FIG. 6.9 Robot-assisted laparoscopic appendicovesicostomy in a child with valve bladder syndrome and a history of posterior urethral valves. (A) The detrusor muscle is incised in the posterior bladder wall to allow tunneled anastomosis of the appendix. (B) Anastomosis of the appendix to the bladder with a feeding tube traversing the appendix into a cystotomy along the posterior wall of the bladder. (C) Postoperative appearance with appendicovesicostomy stoma visible just inside the umbilicus.
Image
FIG. 6.10 Appearance of a newborn with prune belly syndrome: wrinkled, redundant skin with bulging at the flanks due to deficient of abdominal wall musculature and massively distended bladder.
Table 6.2
Spectrum of Prune Belly Syndrome
Image
FIG. 6.12 Surgical correction of megalourethra in a patient with prune belly syndrome. (A) Penile deglovement. (B) Exposure of the scaphoid megalourethra. (C and D) Tailoring of the dilated urethral segment. (E) Completed urethroplasty. (F) Presentation of excessive preputial skin. (G) Completed procedure after urethroplasty and circumcision (note the empty scrotum).
Image
FIG. 6.13 Surgical technique for Monfort abdominoplasty and concomitant reconstruction of prune belly uropathy. (A) Delineation of redundancy by tenting up abdominal wall. (B) Skin incisions are outlined with a separate circumscribing incision to isolate the umbilicus. (C) Skin (epidermis and dermis only) is excised with electrocautery. (D) Abdominal wall central plate is incised at the lateral border of the rectus muscle on either side, from the superior epigastric to the inferior epigastric vessels, creating a central musculofascial plate. (E) Adequate exposure is provided for concomitant transperitoneal genitourinary procedures. B, Bladder; T, testis; U, ureter. (F) Only the more normal proximal ureter is preserved for vesicoureteral reimplantation, and the urachal diverticulum is excised. (G) Transtrigonal ureteral reimplantation is performed with or without ureteral tapering as needed. The bladder is closed in two layers, and ureteral stents (not shown) and a cystostomy tube are used. (H) Completion of abdominoplasty by scoring of the parietal peritoneum overlying the lateral abdominal wall musculature with electrocautery. (I) The edges of the central plate are sutured to the lateral abdominal wall musculature along the scored line. (J) Lateral flaps are brought together in the midline, with closed suction drains placed between the lateral flaps and the central plate. Skin is brought together in the midline, enveloping the previously isolated umbilicus. Source: (From Woodard JR, Perez LM. Prune-belly syndrome. In: Marshall FF, ed. Operative urology. Philadelphia, PA: Saunders, 1996.)
Table 6.3
Spinal Level of Myelomeningocele
Image
FIG. 6.14 Oxybutynin is a potent anticholinergic agent that dramatically delays detrusor contractions and lowers contraction pressure, as demonstrated on these two graphs. U.C., Uninhibited contraction.
