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Campbell's > Neuromuscular dysfunction of the lower urinary tract (ch. 142) > Flashcards

Neuromuscular dysfunction of the lower urinary tract (ch. 142) Flashcards

1
Q

What is the prognosis and mortality for children with neural tube defects?

A

1/3 die by 5 years of age

Another 1/4 die by 40 years of age.

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2
Q

What is the most common cause of neurogenic bladder dysfunction in children?

A

Abnormal development of the spinal cord.

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3
Q

When does formation of the spinal cord take place?

A

days 18 to 35 of gestation

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4
Q

What is the incidence of neural tube defects?

A

0.3-4.5 per 1000 live births.

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5
Q

What race is most and least likely to have neural tube defects?

A

Most likely: hispanics

Least likely: African Americans

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6
Q

What helps prevent neural tube defects?

A

Folic acid 4 weeks before and during pregnancy.

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7
Q

What other malformation is almost always associated with myelomeningocele?

A

Arnold-Chiari.

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8
Q

What is the association between vertebral level and neurologic lesion in myelomeningocele?

A

The neurologic lesion does not correlate with the vertebral lesion as they may differ by up to 3 levels in either direction.

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9
Q

What is the two hit hypothesis for myelomeningocele?

A
  1. The initial failure of the neural tube to form properly.

2. Ongoing injury to the neural tube by the intrauterine environment.

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10
Q

What is the impact of prnatal closure of myelomeningocele on bladder function?

A

The literature indicates that prenatal closure of myelomeningocele does not improve bladder functional outcomes. It may improve neuromotor function but increases pregnancy complications.

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11
Q

What is the immediate post natal managment of myelomeningocele?

A

Evaluation should begin as soon as possible.
Renal US
PVR
UA
Urine culture
Cr
Urodynamics when healed from spinal surgery

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12
Q

What are the indications for VCUG in neonatal myelomeningocele patients?

A

Hydronephrosis
Thickened bladder wall

Detrusor overactivity
Poor compliance
Elevated LPP
DSD

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13
Q

What should be done if there is an abnormality on renal ultrasound or VUR on VCUG?

A

Obtain a DMS scan.

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14
Q

What proportion of newborns with myelomeningocele have a structural abnormality of the urinary tract?

A

10%

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15
Q

What are the rate bladder contractions, DO, acontractile bladder?

A

Bladder contractions 63%
50% DO
37% acontractile

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16
Q

What are the three categories of LUT dynamics seen in newborns with myelomeningocele?

A

Synergic 26%
Dyssynergic 37%
Complete denervation 36%

17
Q

Which of the three categories of LUT dysfunction after MMC are associated with upper tract deterioration?

A

DSD 71%
Complete denervation 23%
Synergic 17%

18
Q

What constitutes early intervention for MMC (myelodysplasia)?

A

CIC

Anti-muscarinic therapy.

19
Q

What is the impact of CIC and early intervention on outcomes newborns with MMC (myelodysplasia)?

A

CIC significantly reduces the rate of UTI, Upper urinary tract deterioation, VUR, and Surgery.

20
Q

For which myelodysplasia patients does early intervention not benefit?

A

Those that can empty their bladders spontaneously.

21
Q

How can GFR be measured in children with myelodysplasia?

A

Gold standard: renal scan
Cystatin C
Cr does not accurately reflect GFR in children.

22
Q

What is the impact of spina bifida on kidney size?

A

Spina bifida is associated with small kidneys. This is thought to be due to hyperhomocysteinemia related to placental vasculopathy.

23
Q

What is the rate of renal dysfunction in children with spina bifida?

A

30-50%

24
Q

What is the rate of ESRD in children with spina bifida?

A

18% before puberty

15-30% after puberty

25
Q

What is the impact of early intervention with CIC and anti-muscarinics on renal dysfunction and ESRD?

A

Renal dysfunction decreases from 30-50% to 6%

ESRD decreases from 15-30% to 1.6%

26
Q

What factors are associated with increased risk of renal deterioration?

A 
DSD
High detrusor pressure
DO
Hydronephrosis
VUR
Febrile UTI
27
Q

What are the sexual differences seen in patients with spina bifida?

A

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.

28
Q

What is the fertility rate for males and females with spina bifida?

A

Males: 17-39%
Females: 70-80%

29
Q

Is spina bifida hereditary?

A

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)

30
Q

What is the rate of erectile dysfunction in men with myelomeningocele?

A

75%

31
Q

What treatments are available for men with ED and myelomeningocele?

A

PDE5i (80% effective)

Ileoinguinal to dorsal penile nerve graft (experimental)

32
Q

What is the rate of fecal incontinence in patients with spina bifida?

A

2/3 over age 6.

1/3 age 16-25.

33
Q

What is the treatment approach to constipation in patients with spina bifida?

A
  1. High fiber and polyethylene glycol
  2. Post prandial toilet regimen (10%)
  3. Digital stimulation (16%)
  4. Tap water enemas 500ml-1L (42%)
  5. Antegrade continence enemas (ACE) (32%)
34
Q

What is the follow up for neurogenic bladder in newborns and toddlers?

A

US Q 6 months till 2yo
Urodynamics q12months
DMSA if VUR or febrile UTI

35
Q

What are the follow up recommendations for toddlers to adolescents with neurogenic bladder?

A

US q1-2 years
UDS when neurologic change
DMSA when febrile UTIs

36
Q

What are the follow up recommendations for Adolescents to adults with neurogenic bladder?

A

US q12 months
UDS when change in symptoms
VCUG with rUTI

37
Q

What is the follow up for adults with neurogenic bladder secondary to spina bifida?

A

US q 3 years

UDS with change in symptoms

Campbell's (2 decks)

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