PEDI GU REVIEW voiding dysfunction i

VOIDING DYSFUNCTION

Pediatric GU Review

UCSD Pediatric UrologyGeorge Chiang MD

Sara Marietti MD

Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007

(not for reproduction, distribution, or sale without consent)

Classification

• Functional– Detrusor contractility

• Normal • Overactive• Inactive

• External sphincteric activity– Normal (synergetic/coordinated)– Overactive (dyssynergic or uncoordinated)– Inactive

• Common causes of pediatric neurogenic bladder– Open neural tube defects– Sacral agenesis– Occult neural tube defects– Spinal cord lesions– Cerebral palsy

Open neural tube defects

• Myelomeningocele– Meninges and neural tissue are exposed

• Meningocele– Meninges are exposed, neural tissue lies

within spinal column

• Lipomyelomeningocele– Myelomeningocele with lipoma of the spinal

cord

Myelomeningocele

• 1 per 1,000 live births

• 1 per 100 if 2 affected siblings

• Incidence decreasing because of elective termination and folate supplementation

• Pathogenesis– Failure of caudal neural tube formation

• Lumbosacral 47%, Lumbar 26%, Sacral 20%

Myelomeningocele

• Neurologic dysfunction– Can’t be predicted by vertebral level– Global neurologic deficits are a result of:

•Dystrophic neural elements•Brainstem dysfunction from Arnold-Chiari•Exposure of the neural tissue to the outside environment including amniotic fluidFetal Closure has mixed urological results

Myelomeningocele

• Evaluation of newborn– Physical exam– Labs for renal function– Ultrasound before and after neurosurgical

intervention– Monitor PVR and maintain <5 cc– UDS

• ?established baseline function and identifies at-risk babies (poor compliance, DSD) and helps with counseling

Myelomeningocele

• UDS findings– Bladder: 62% contractile bladder, 37%

acontractile, 17% poor compliance– Sphincter: 40% intact sacral reflex

(sphincteric contraction), 24% partially denervated, 38% absent

– Bladder-sphincter coordination: 28% synergetic, 37% dyssynergetic, 36% complete denervation

Myelomeningocele

• UDS findings– Patient with DSD on initial UDS have highest

risk of upper tract deterioration– Patients with DSD or poor compliance should

be started on early CIC and anticholinergics– Continued follow-up is based on initial UDS– Greatest change in sphincter function occurs

over the first year of life

Myelomeningocele

• Continued Management– VUR– Incontinence– Bowel function– Secondary tethered cord– Adolescent and adult issues

Myelomeningocele

• VUR– Presence is closely associated with elevations

in storage and voiding pressure– Incidence of VUR is reduced with lowering

bladder pressure– Once bladder is optimized, resolution of VUR

can be seen in 30-55% of patients

Myelomeningocele

• VUR– Indications for surgery (recurrent UTI on prophylaxis,

persistent VUR in older children, reflux in children undergoing bladder outlet procedures and high grade VUR in patients undergoing augment)

– With CIC antireflux surgery success approaches 95%– Endoscopic management?

Myelomeningocele

• Incontinence– Failure to store: high storage pressure, poor compliance, or

detrusor overactivity• Medical management• Endoscopic management (BoTox)• Surgical Management

– Failure to store:sphincter (low outlet resistance)• Medical management (alpha agonists)• Endocopic management (Deflux)• Surgical management (BNR, Sling, AUS)

• Failure to empty– Whether secondary to poor detrusor function or increased outlet

resistance (CIC or cath channel

Myelomeningocele

• Bowel function– Regimen of diet, medications, enemas– ACE

• Secondary tethered cord– Should be suspected if changes in clinical or UDS

pattern develops– On MRI may be difficult to distinguish tethered cord

from post-op changes– May have to intervene on clinical suspicion– Improvement in hydro and UDS have been seen after

surgical intervention

Myelomeningocele

• Adolescent issues– 28-40% of teenagers had one or more sexual

encounter– 72% of males claim normal erections with 2/3

reporting ability to ejaculate– 70-80% of females were able to have uneventful

pregnancy

Occult Spinal Dysraphism/Tethered Cord

• Lipomeningocele• Intradural lipoma• Diastematomyelia• Tight filum terminate• Dermoid cyst/sinus• Aberrant nerve roots• Anterior sacral meningocele• Cauda equina tumor

Occult Spinal Dysraphism/Tethered Cord

• Incidence 1 in 4,000

• Often not diagnosed until failure to achieve continence

• UDS will often show lower tract dysfunction

• Following surgerym younger children have better rates of improvement on UDS

Sacral Agenesis

• Associated with maternal IDDM

• Neurologic deficit corresponds poorly with vertebral level

• Over 1/3 will have UMN lesions on UDS

SCI

• Period of spinal shock will last 4-6 weeks

• UDS at 6 weeks post injury

• Neurogenic detrusor overactivity with DSD in suprasacral injuries

• Annual surveillance for upper tract changes and stones

Cerebral Palsy

• Nonprogressive perinatal insult

• UDS will demonstrate detrusor overactivity 80%; 5% with DSD

• Routine UDS usually not needed

• Most achieve continence (related to neuromuscular disability)

Non-neurogenic voiding dysfunction

• Uroflow ?with EMG• Management

– Behavior modification– Biofeedback– TENS – Bowel management– Phamacotherapy

• Ditropan, Detrol, etc• BoTox

Non-neurogenic voiding dysfunction

• Nocturnal enuresis– Prevalence at age 7=9.5% with 15% regression per year– Etiology

• Derangement of circardian rhythm of ADH• Reduced functional bladder capacity (largest daytime void if <70%

EBC then DDAVP less effective)• DSD• Genetics• ADHD• Arousal failure

– Management: Voiding diary, bowel habits, Alarm, Acupuncture• Meds: DDAVP (works on distal tubules and collecting ducts 30%

cure); Ditropan, Imipramine (50% success with decreased detrusor activity, increased outlet resistance, enhances arousal and suppresses REM sleep)

Non-neurogenic voiding dysfunction

• Hinman syndrome– Occult neuropathic bladder– Acquired form of DSD with bladder decompensation,

incontinence, UTI and bowel dysfunction– Children with large trabeculated bladders and 2/3 with

hydro, ½ with VUR– 75% bladder instability– UDSobstruction during voiding secondary to

voluntary constriction of external sphincter– Biofeedback, CIC, Family dynamics

QUESTIONS

A 12 year old girl has recurrent UTIs. An ultrasound shows a large, thickened bladder wall and bilateral hydronephrosis. A VCUG shows no reflux and a large PVR

a) MAG-3 Renogram

b) Spinal MRI Scan

c) Urethral pressure profile

d) Sphincter EMG during voiding

e) Cystoscopy and urethral dilation

The UDS of a 6 year old child with diurnal wetting without infection will usually demonstrate

a) A small hypertonic bladder

b) Detrusor hypocontractility

c) Abnormal external sphincter activity on filling

d) Abnormal external sphincter activity during voiding

e) Normal external sphincter activity on filling and voiding

A 16 year-old girl with neuorogenic bladder remains incontinent despite an aggressive program of CIC and anticholinergic medications. UDS demonstrates a flaccid, large capacity bladder and low urethral resistance. The best long-term management is CIC and:

a) Periurethral collagen injection

b) Bladder neck tubularization

c) AUS

d) PV sling

e) Mitrofanoff

A 7 year old girl with myelo has bilateral hydro and urinary incontinence and CIC and Ditropan 5 tid. CMG reveals a pressure of 35 cm at 100 ml and a detreusor LPP of 54 cm at 120 cc. The next step is:

a) Intravesical ditropan

b) Detrusor myomectomy

c) Tolterodine

d) Vesicostomy

e) Enterocystoplasty

A 6 year old boy with a history of imperforate anus has persistent diurnal urinary incontinence. Evaluation includes an MRI scan showing the conus medullaris at the L2 level. A CMG shows no PVR, a flat filling curve with high volume detrusor instability, and normal voiding pressures. These findings suggest:

a) Tethering of the spinal cord

b) Vesico-colonic fistula

c) DSD

d) No significant uropathology

e) Intrinsic sphincter deficiency

Spontaneous bladder perforation after enterocystoplasty is most likely the result of:

a) Transmural cystitis

b) Mucous plug

c) Catheter trauma

d) Ischemic necrosis

e) Bladder calculi

In pediatric patients with VP shunt following augmentation cystoplasty, the usual time to presentation, and the most frequent type of bacterial infection is:

a) <30 days gram +

b) < 30 days, gram –

c) >30 days, mixed growth

d) >30 days. gram +

e) > 30 days, gram-

To perform an appendicovesicostomy, the blood supply to the appendix that must be preserved arises from a branch of which artery:

a) Ileal

b) Anterior cecal

c) Posterior cecal

d) Ascending colic

e) Inferior mesenteric

The best artery to use as a pedicle for a gastrocystoplasty when avoiding use of the antrum is the:

a) Right gastroepiploic

b) Left gastroepiploic

c) Gastroduodenal

d) Right gastric

e) Left gastric

An 11 year old boy with spina bifida is scheduled for bladder augment and implantation of an AUS. Infection or erosion are more common if sphincter implantation is performed:

a) Prior to bladder augment

b) Following bladder augment

c) Simultaneous with augment

d) With sigmoid enterocystoplasty

e) In an area of previous surgery

A 7 year old girl has urgency, frequency, and diurnal enuresis. She is wet every day, requiring clothes changes twice daily. Physical exam and urinalysis are normal. She is placed on ditropan and timed voiding. She returns 2 weeks later and reports no change in the diurnal enuresis. The next step is:

a) MRI scan of the LS spine

b) Add imipramine

c) UDS

d) Voiding diary

e) Urethral dilation

The consequence of DDAVP when used to treat nocturnal enuresis is:

a) Increased urine osmolality

b) Decreased urine sodium

c) Down-regulation of vasopressin receptors

d) Mild hyponatremia

e) Mild hypernatremia

A 1 year old 10 kg boy with spina bifida is maintained on 2 mg ditropan TID and CIC every 4 hours. U/S shows progressive bilateral hydro and diuretic scan shows no evidence of obstruction. A CMG shows a detrusor pressure of 40 cm H20 at a avolume of 35 cc and a detrusor LPP of 58 cm at a volume of 50 cm. The next step is:

a) Increase ditropan

b) Change to tolterodine

c) Decrease cath interval

d) Cutaneous vesicostomy

e) enterocystoplasty

A 7 year old boy with neurogenic bladder, renal failure, and moderate acidosis is scheduled for a renal transplant. He has diurnal and nocturnal incontinence despite treatment with ditropan 5 qid. UDS reveals a bladder capacity of 120 cc with an end-filling detrusor pressure of 70 cm H20 with grade 2/5 bilateral VUR and normal uroflow curve with a 60 cc PVR. The best option is:

a) Transplant into native bladder

b) Vesicostomy and transplant into native bladder

c) Bilateral nephrectomy and ureteral augmentation prior to transplant

d) Ileal augment prior to transplant

e) Gastrocystoplasty prior to transplant

A nine year old boy has microscopic hematuria after a bicycle accident. VCUG is normal except for spina bifida occulta. Renal ultrasound and voiding pattern are normal. The next step is:

a) Neurologic consultation

b) MRI of the lower spine

c) UDS

d) Reassurance

e) EMG testing

A 10 year old boy with neurogenic bladder managed with CIC has normal upper urinary tracts. He has had asymptomatic bacteriuria on multiple occasions. Starting prophylactic antibiotics will likely::

a) Result in resistant organisms

b) Have no effect on urinary bacteria

c) Significantly decrease the frequency of bacteriuria

d) Lead to fewer symptomatic infections

e) Decrease the risk of stone formation

Sacral agenesis in the neonate is most closely associated with which maternal factor:

a) Advanced age

b) Sacral agenesis

c) Insulin dependence

d) Folic acid deficiency

e) 1st trimester use of contraceptive pills

A 14 year old boy with bladder exstrophy underwent division of the bladder neck with Mitrofanoff appendiceal stoma without bladder augmentation. Six months later, he has episodic urinary incontinence per stoma, not necessarily related to bladder fullness. A CMG on anticholinergic therapy reveals a 400 cc compliant bladder without uninhibited contractions. He leaks when he sits up at the end of the study, with no evidence of detrusor contraction. The next step is:

a) Observation

b) Inject stoma with bulking agent

c) Revise appendicovesicostomy

d) Replace appendix with ileal Monti tube

e) enterocystoplasty

An eight year old girl underwent colocystoplaty one year ago for a neurogenic bladder. She now has a temp of 101F, vomitting, and abdominal pain. She is oliguric and U/A shows pyuria and bacteria. Physical exam reveals abdominal tenderness, pulse of 125, and BP 95/65. A cystogram is normal. After volume resuscitation, the next step is:

a) CT cystography

b) Suprapubic cystostomy

c) Laparotomy

d) Abdominal u/s

e) cystoscopy

A 9 year old boy has primary nocturnal enuresis. He has used an alarm for 4 months but continues to wet the bed. He has daytime urgency and voids seven to eight times a day, but remains dry during that time. Repeat urinalysis is normal. The next step is:

a) Voiding diary

b) UDS

c) VCUG

d) DDAVP

e) Oxybutynin

A 6 month old boy with spina bifida has moderate hydroureteronephrosis on an ultrasound exam. A cystogram shows trabeculation but no reflux. The most likely cause of the upper tract dilation is:

a) Associated constipation

b) Urine storage pressure >40 cm H20

c) Uninbited detrusor contractions

d) Unsuspected urinary infection

e) Detrusor-sphincter-dyssynergia

A 3 month-old boy had a lumbar myelomeningocele closed at birth. The finding associated with the potential for detrusor sphincter dyssynergia is:

a) Level of lesion

b) Intact bulbocavernosus reflex

c) Lower extremity movement

d) Spontaneous voiding

e) Decreased anal sphincter tone

A 6 year old girl with recurrent symptomatic UTIs has a normal renal ultrasound. A VCUG is shown. The next step is prophylactic antibiotics and:

a) Timed voiding

b) Urethral meatotomy

c) Internal urethrotomy

d) Urethral dilation

e) Alpha-blockers

6 months following implantation of an AUS, a 22 year old myelo male has bilateral ureteral dilation by ultrasound. The diagnostic study most likely to reveal the cause of the problem is:

a) CMG

b) Diuretic renogram

c) Whitaker test

d) Repeat u/s after catheter drainage

e) IVP

An 8 year old boy was treated for lipomyelomeningocele at birth. He is on CIC q 4hrs and oxybutynin 5 bid. He develops new incontinence. The next step is:

a) UDS

b) MRI scan of spine

c) Increase cath frequency

d) Increase anticholinergics

e) Start imipraime

A 7 year old girl has diurnal enuresis that is not associated with infection. She has marked constipation and encorpresis. Her physical exam reveals a normal appearing spine and normal neurologic exam. Her bladder is not percussible. The next step is timed voiding and:

a) Spinal MRI scan

b) VCUG

c) UDS

d) Oxybutynin

e) Laxatives/enemas

Paternity in males with spina bifida correlates positively with:

a) Serum testosterone level

b) Sacral neurologic level of lesion

c) Reflexogenic erections

d) Shunt-dependent hydrocephalus

e) Glans penis sensation

A 2 month old girl with a high myelomeningocele closed at birth is managed with diaper voiding. She recently had a febnle UTI. Video UDS shows bilateral Grade V reflux, a thick-walled bladder and leakage around a 5 Fr urethral catheter at 40 cc volume and 50 cm H20. The next step is:

a) Vesicostomy

b) Conduit diversion

c) Antibiotics and oxybutynin

d) Augmentation and bilateral reimplants

e) Bilateral reimplants and CIC

The most compelling reason to use large bowl rather than small bowel in an undiversion procedure is a decrease in:

a) Incidence of reflux

b) Intraluminal pressure

c) Incidence of malignancy

d) Mucous production

e) Incidence of bladder stones

A 12 year old boy who has previously undergone an ileal augmentation catheterizes every 4 hours but develops recurrent bladder calculi. The most effective way to dimish recurrence is:

a) Restrict dietary sodium

b) Potassium citrate

c) Daily bladder irrigation

d) Increase cath frequency

e) Catheterize in the supine position

Non-neurogenic bladder is characterized by:

a) High intravesical pressure with poor compliance

b) Detrusor areflexia with detrusor internal sphincter dyssynergia

c) Detrusor areflexia and low urethral closing pressure

d) Uninhibited contractions with detrusor external sphincter dyssynergia

e) Uninhibited contractions with detrusor internal sphincter dyssynergia

While performing videourodynamics study in a 3 year old child, the recommended rate of bladder filling is:

a) 10 cc/min

b) 20 cc/min

c) 30 cc/min

d) 40 cc/min

e) 50 cc/min