MCN Exam 4 Study Guide – Pt.

5 Colin MacKenzie

Neuromuscular Disorders in Children

1. Neural Tube Defects :

a. Spina bifida occulta (B):

i. Most minor type
ii. “occult” – can’t see it (not an externally visible defect)
iii. Defect of the vertebrae, but no protrusion of the spinal cord or meninges
iv. Often identified by dimpling, patch of hair, slight discoloration of the skin
1. May not be identified at all
v. No treatment is needed initially
1. No associated symptoms resulting in neuro deficits
vi. Complications are rare, but as they get older (adolescence or adulthood)
may experience:
1. Back pain
2. Muscle weakness in their legs
3. Tethered cord syndrome
a. Spinal cord becomes attached to the spinal column (causes
restrictions in movement)
vii. Nursing Management & Treatment:
1. Focus is on education for the family
2. Occasionally surgery may be needed
b. Meningocele (C):
i. General Info:
1. Meninges have herniated through the vertebral defect
2. Spinal cord is usually normal
3. Only minor or no associated neuro deficits
ii. Assessment:
1. Visible external sac protruding from the spinal area
a. Most are covered with skin and pose no threat
b. Assess to ensure sac covering is intact
2. Immediately report leaking of CSF

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3. Monitor head circumference and for s/s of ICP

a. Hydrocephalus has been associated with meningocele
4. Observe for s/s of constipation or bladder dysfunction
a. May result due to ↑ size of lesion
iii. Nursing Management & Treatment:
1. Surgical procedure to have this closed (within the 1st day or so of
birth)
a. Done to prevent infection and protect exposed meninges
2. Management is supportive
a. Prevent rupture of sac
b. Prevent infection
c. Provide adequate nutrition and hydration
3.
c. Myelomeningocele (D):
i. General Info:
1. Most severe NTD
2. Both meninges AND spinal cord are protruding through the defect
a. Causes loss of motor and sensory function beyond area of
defect (think paraplegia)
b. Higher the defect on the spine, greater the neuro deficit
3. Child may also have brain issues (i.e. mild hydrocephalus; problem
with one, there is a problem with the other)
a. ↑ r/f meningitis, hypoxia, hemorrhage
4. Diagnosed in utero → c-section delivery → surgical procedure
within 1st 2 days of life → NICU care
a. Just recently, prenatal surgeries are being done to close
this defect
i. Not all mothers will qualify for this surgery (very
specific criteria)
ii. Child should have mobility and bowel/bladder
control after this new procedure is done
b. Prior surgery was done to prevent infection and protect
exposed meninges
i. Even with this surgery, the child will not walk and
will have permanent, lifelong bowel and bladder
control problems
5. Due to possibly needing multiple surgeries, ↑ r/f developing a
latex allergy
ii. Risk Factors:
1. Lack of prenatal care
2. Lack of preconception and/or prenatal folic acid supplementation
3. Family history

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MCN Exam 4 Study Guide – Pt. 5 Colin MacKenzie

4. Medications that antagonize folic acid (i.e. anticonvulsants)

iii. Assessment:
1. Sac covering should be intact
2. Assess movement of extremities and anal reflex
a. Commonly found neuro deficits include:
i. Flaccid paralysis
ii. Absence of DTR’s
iii. Lack of response to touch and pain stimuli
iv. Skeletal abnormalities (i.e. club foot)
v. Constant dribbling of urine (neurogenic bladder)
vi. Relaxed anal sphincter
iv. Nursing Management & Treatment:
1. Initial pre-op care and nursing management focus is on:
a. Prevent sac from drying out
i. Cover it with sterile soaked dressings
ii. Pay special attention when using a warmer/isolette
b. Prevent trauma
c. Prevent infection
d. Prevent hypothermia
i. Larger r/f heat loss since internal organs are
exposed to the environment
e. Position infant in prone position so supported on their side
i. Avoids putting pressure on the sac
ii. Position so that urine and feces flow away from the
sac
f. Promote adequate nutrition
g. Educate/support child and family
i. Focus is on what’s going to happen with the baby in
the next 24-48 hours (operative procedure and
immediate post-op care)
2. Post procedure:
a. Position infant prone or side-lying position so incision can
heal
b. Keep diaper open and under the baby (helps to ↓ r/f
infection)
c. Wound care:
i. Look out for s/s of local and/or systemic infection
d. Nutrition:
i. Possible NG feedings until the wound starts to heal
ii. Continue to encourage breast feeding (mom can
pump and milk can be stored)
3. Long-term care (multidisciplinary):
a. Promote urinary elimination

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MCN Exam 4 Study Guide – Pt. 5 Colin MacKenzie

i. Have a neurogenic bladder (does not empty on its

own)
1. Catheterized every few hours (↑ r/f
infection)
2. Eventually will have to learn how to do this
on their own
3. Educate the parents on how to change and
maintain the catheter
b. Promote bowel elimination
i. Need a bowel training program as the child ages
1. Dietary modifications, enema/suppositories
c. Promote mobility
i. Maximize whatever they may have
d. Latex-free environment
i. High risk of developing a latex allergy (wear a
medic alert bracelet)
e. Meticulous skin care
i. Be aware of skin breakdown and pressure sores
2. Cerebral Palsy:
a. General Info :
i. Nonprogressive brain dysfunction
ii. Once the damage is done, its done, it won’t get worse
iii. Associated with birthing trauma such as anoxia, usually perinatal
occurrence (i.e. infection)
iv. Most common movement disorder of childhood
v. Spastic type is the most common (although there are 5 types in total)
vi. Goals for management:
1. Optimal development and function
2. Multidisciplinary treatment

b. Patho:

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MCN Exam 4 Study Guide – Pt. 5 Colin MacKenzie

i. Caused by abnormal development of, or damage to, the motor areas of

the brain
ii. Results in a neurologic lesion
c. Health History (risk factors & birth history):
i. Way too many to list!
ii. Could be prenatal, perinatal, or postnatal
d. Signs & Symptoms:
i. Poor feeding, decreased growth patterns
ii. Seizures
iii. Vision and hearing deficits
iv. Delayed development
v. Persistent primitive reflexes
vi. Spasticity, hypertonicity
vii. Muscle weakness
viii. Ataxia (lack of coordination of muscle movement during voluntary
movements such as walking)
ix. Cognitive disabilities
x. Pathologic drooling
e. Labs & Diagnostics:
i. Primary methods used are EEG, MRI or ultrasound of the brain, and
genetic testing
f. Nursing Management & Treatment:
i. Overall focus is to assist the child gain optimal development and function
within the limits of the disease
ii. Multiple surgeries may be required (typically orthopedic or neurological)
iii. Manage medications
1. Don’t fix the problem, but may minimize negative outcomes
2. Anticonvulsants
a. Only if seizures are present
3. Benzodiazepines (diazepam, lorazepam)
a. Decrease spasticity
4. Baclofen
a. Used for severe spasticity that is unable to be managed by
other medications
b. Centrally acting skeletal muscle relaxant
i. Used to treat painful spasms and ↓ spastic pain
c. Baclofen pump education:
i. Check incisions daily for redness, drainage, or
swelling
ii. Notify provider if child has persistent pain or a
temp greater than 101.5
iii. Avoid tub baths for 2 weeks
iv. No stomach sleeping for 4 weeks

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MCN Exam 4 Study Guide – Pt. 5 Colin MacKenzie

v. Discourage twisting at the waist, reaching high

overhead, stretching, or bending forward or
backward for 4 weeks
vi. When incision has healed normal activity may
resume
vii. Wear loose clothing to prevent irritation at the
incision site
viii. Carry implanted device ID and emergency info
cards at all times
5. Anticholinergics
a. To help with abnormal movements
b. Prevents some spasms and drooling which may occur
6. Botulin toxin injections
a. Used to block neuromuscular conduction to relieve
spasticity in a specific area
iv. Promote mobility
1. Orthotics and braces
2. Wheelchair use
3. Skin care
v. Promote nutrition
1. Special diet, upright positioning
a. To prevent aspiration and reflux
2. G-tube for weight management
3. Increased caloric needs
4. Speech therapist
vi. Prevent contractures
vii. Provide support/education

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