Neuromuscular dysfunction of the lower urinary tract (ch. 142) Flashcards
What is the prognosis and mortality for children with neural tube defects?
1/3 die by 5 years of age
Another 1/4 die by 40 years of age.
What is the most common cause of neurogenic bladder dysfunction in children?
Abnormal development of the spinal cord.
When does formation of the spinal cord take place?
days 18 to 35 of gestation
What is the incidence of neural tube defects?
0.3-4.5 per 1000 live births.
What race is most and least likely to have neural tube defects?
Most likely: hispanics
Least likely: African Americans
What helps prevent neural tube defects?
Folic acid 4 weeks before and during pregnancy.
What other malformation is almost always associated with myelomeningocele?
Arnold-Chiari.
What is the association between vertebral level and neurologic lesion in myelomeningocele?
The neurologic lesion does not correlate with the vertebral lesion as they may differ by up to 3 levels in either direction.
What is the two hit hypothesis for myelomeningocele?
- The initial failure of the neural tube to form properly.
2. Ongoing injury to the neural tube by the intrauterine environment.
What is the impact of prnatal closure of myelomeningocele on bladder function?
The literature indicates that prenatal closure of myelomeningocele does not improve bladder functional outcomes. It may improve neuromotor function but increases pregnancy complications.
What is the immediate post natal managment of myelomeningocele?
Evaluation should begin as soon as possible.
Renal US
PVR
UA
Urine culture
Cr
Urodynamics when healed from spinal surgery
What are the indications for VCUG in neonatal myelomeningocele patients?
Hydronephrosis
Thickened bladder wall
Detrusor overactivity
Poor compliance
Elevated LPP
DSD
What should be done if there is an abnormality on renal ultrasound or VUR on VCUG?
Obtain a DMS scan.
What proportion of newborns with myelomeningocele have a structural abnormality of the urinary tract?
10%
What are the rate bladder contractions, DO, acontractile bladder?
Bladder contractions 63%
50% DO
37% acontractile
What are the three categories of LUT dynamics seen in newborns with myelomeningocele?
Synergic 26%
Dyssynergic 37%
Complete denervation 36%
Which of the three categories of LUT dysfunction after MMC are associated with upper tract deterioration?
DSD 71%
Complete denervation 23%
Synergic 17%
What constitutes early intervention for MMC (myelodysplasia)?
CIC
Anti-muscarinic therapy.
What is the impact of CIC and early intervention on outcomes newborns with MMC (myelodysplasia)?
CIC significantly reduces the rate of UTI, Upper urinary tract deterioation, VUR, and Surgery.
For which myelodysplasia patients does early intervention not benefit?
Those that can empty their bladders spontaneously.
How can GFR be measured in children with myelodysplasia?
Gold standard: renal scan
Cystatin C
Cr does not accurately reflect GFR in children.
What is the impact of spina bifida on kidney size?
Spina bifida is associated with small kidneys. This is thought to be due to hyperhomocysteinemia related to placental vasculopathy.
What is the rate of renal dysfunction in children with spina bifida?
30-50%
What is the rate of ESRD in children with spina bifida?
18% before puberty
15-30% after puberty
What is the impact of early intervention with CIC and anti-muscarinics on renal dysfunction and ESRD?
Renal dysfunction decreases from 30-50% to 6%
ESRD decreases from 15-30% to 1.6%
What factors are associated with increased risk of renal deterioration?
DSD High detrusor pressure DO Hydronephrosis VUR Febrile UTI
What are the sexual differences seen in patients with spina bifida?
50% are dissatisfied with their sex life.
Females reach puberty on average 2 years earlier than normal (10.9-11.4years)
30% of women with spina bifida experience sexual abuse.
What is the fertility rate for males and females with spina bifida?
Males: 17-39%
Females: 70-80%
Is spina bifida hereditary?
Both males and females with spina bifida have an increased risk of having a child with spina bifida (3.7% if on parent)(15% if both parents)
What is the rate of erectile dysfunction in men with myelomeningocele?
75%
What treatments are available for men with ED and myelomeningocele?
PDE5i (80% effective)
Ileoinguinal to dorsal penile nerve graft (experimental)
What is the rate of fecal incontinence in patients with spina bifida?
2/3 over age 6.
1/3 age 16-25.
What is the treatment approach to constipation in patients with spina bifida?
- High fiber and polyethylene glycol
- Post prandial toilet regimen (10%)
- Digital stimulation (16%)
- Tap water enemas 500ml-1L (42%)
- Antegrade continence enemas (ACE) (32%)
What is the follow up for neurogenic bladder in newborns and toddlers?
US Q 6 months till 2yo
Urodynamics q12months
DMSA if VUR or febrile UTI
What are the follow up recommendations for toddlers to adolescents with neurogenic bladder?
US q1-2 years
UDS when neurologic change
DMSA when febrile UTIs
What are the follow up recommendations for Adolescents to adults with neurogenic bladder?
US q12 months
UDS when change in symptoms
VCUG with rUTI
What is the follow up for adults with neurogenic bladder secondary to spina bifida?
US q 3 years
UDS with change in symptoms
