Neuro disorders Flashcards
frequently associated anomaly in SB Cystica
hydrocephalus
any malformation of spinal canal and cord
Myelodysplasia
neurogenic/neuropathic bladder dysfunction results to
UTI, ureterohydronephrosis, vesiculourethral
reflux)
When infants are detected as having meningocele or myelomenigocele, they may be born by caesarean section to what reason
to avoid pressure and injury to the spinal cord.
Parts of shunt
ventricular catheter, pumping chamber, unidirectional pressure valve, distal catheter
spina bifida =
myelomeningocele
→ Lateral ventricle/lumbar subarachnoid space to ureter→bladder (CSF is not reabsorbed = additional intake of water and sodium) → additional workload on the kidney (a kidney transplant might be considered as a last resort)
→ Older children
→ Necessitates the removal of the kidney
→ Last resort if other types have failed
→ Necessitates prompt treatment of UTI to prevent ascending infection
Ventriculoureteral shunt
GLASGOW COMA SCALE IN INFANTS & CHILDREN: Eye opening in adults
4 Spontaneous
3 To verbal stimuli
2 To pain
1 No response
medical care for mongolism:
* For children participating in sports that may
involve stress on back and neck
* Monitor for neck pain, weakness and
torticollis
atlantoaxial instability
Sac must be kept free from
contamination by urine and feces = *****, must cover dressing loosely
use steridrape
reduce chances of skin breakdown
- sheepskin/lamb’s wool blanket under the head
- s/e of immobility
s/s for spinal cord compression
persistent neck pain, loss of established
motor skills and bladder and bowel control,
changes in sensation
GLASGOW COMA SCALE IN INFANTS & CHILDREN: verbal response in adults
5 Oriented to time, place and person, uses appropriate words & phrase
4 Confused
3 Inappropriate words/verbal response
2 Incomprehensible words
1 No response
surgical procedure for neuropathic bladder dysfunction: → continent neobladder constructed from
bowel/stomach + CIC
→ use when severe bladder dysfunction
compromising renal function not relieved by other means
Urinary diversion
MANAGEMENT of SB
- Early surgery 24-72 hours after birth to replace the contents and to close the skin (skin graft)
- Multidisciplinary = pediatrics, neurosurgery, orthopedic, physical medicine, urology
a. Mobility
b. Bladder elimination
- Defect in closure of posterior vertebral arch with protrusion through bony tissue
- Visible with an external saclike protrusion
SPINA BIFIDA CYSTICA
s/s of shunt malfunction in infants
o early sign: tense bulging fontanel
o late: high pitched, shrill cry, seizures
goals of bladder elimination in older children
preserve renal function and achieve
optimum urinary continence
Associated anomalies in mongolism
a. Congenital heart defects
b. GU structural defects
c. Altered immune function
o Prone to URI due to hypotonicity of chest and
abdominal muscles and dysfunction of the
immune system predispose to the development of
respiratory infection
d. Visual defects
e. Increased incidence of leukemia
f. Thyroid dysfunction (congenital hypothyroidism)
❖ A congenital defect in which the spinal column is imperfectly closed so that part of the meninges or spinal
cord protrudes, often resulting in hydrocephalus and other neurological disorders.
❖ Failure of the posterior vertebral arches to fuse during the embryologic development.
spina bifida
head is short but wide; sagittal sutures
may separate
Brachycephaly
Mgt of infection in shunt placement
a. Antibiotics
b. The Shunt removal with external drainage and replaced when infection is resolved
how to enhance elimination in mongolism
▪ ↑ fiber and ↑fluid
position of postop in SB
prone or side lying
position of hydrocephalus in preoperative
carefully avoid stretching/straining neck muscles since they must support large head
diagnosis for hydrocephalus: Detects what type of hydrocephalus
Lumbar/ventricular tap
shunt:
→ Used in older children and in children with abdominal problems
→ CSF drains from the ventricles to the RA bypassing the internal jugular vein→drains into circulating blood
AV shunt (Atrioventricular Shunt)
causative conditions of communicating hydrocephalus:
- Tumor, infection, hemorrhage or pre-existing developmental defects
- Extraventricular: there’s a passage of fluid between ventricles and spinal cord.
foods rich in folic acid
(fortified breads and cereals, fruits, dark green leafy vegetables and egg yolk
PROGNOSIS of SB
dependent on the type of defect, the level of the lesion and the presence of other complicating factors
s/s of shunt malfunction in toddlers
o early sign=headache, loss of appetite
Early surgery for SB Prevent further neurological deterioration/impairment (menigeal sac expands during the first few hours after birth-stretching of other nerve roots; trauma to the sac can lead to rupture; potential for infection )
discharge teaching for hydrocephalus
▪ s/s of shunt malfunction
▪ s/s of infection
▪ replacement of shunt as child grows
▪ avoid contact sports
▪ hydrocephalus is a lifelong problem that needs evaluation regularly
frontal enlargement, brow bulges
bossing
Regular medical care for mongolism
a. Eyesight
b. Hearing
c. Thyroid function
d. Atlantoaxial instability
visual defects associated with mongolism
o Strabismus
o myopia (nearsightedness)
o nystagmus (involuntrary rapid eye movement)
o cataract
Manifestations of hydrocephalus in childhood
➢ No head enlargement
➢ Headache on awakening: improvement following emesis/upright posture,
➢ Decline in LOC
➢ Ataxia/spasticity
➢ s/s of ↑ICP
➢ visual defects secondary to pressure on optic nerve
when is the Initial placement of shunt
3 – 4 months of age then replaced as child
grows
physical CLINICAL MANIFESTATIONS of mongolism in head and face
a. Brachycephaly-head is short but wide; sagittal sutures
may separate
b. Flat facial profile- small, broad and flat nose
(underdeveloped nasal bone; saddle nose)
c. Oral cavity is smaller than normal- protruding tongue
d. Upward and outward slanting palpebral fissure
e. Small Low set ears
f. Short webbed neck with excess loose skin (puppy’s
neck)
PRENATAL diagnosis for SB
- Elevated MSAFP (maternal serum AFP (alpha feto protein))
- Ultrasound for scanning of uterus
- Amniocentesis
- Chorionic villi sampling (CVS)
DIAGNOSTIC EVALUATION of SB
- Clinical manifestations and examination of meningeal sac
- MRI
- UTZ
- CAT
- Myelography
- Lab exams
position to maintain hydration and nutrition in preop of SB
head turned to side
1) Isolate the segment of ileum chosen for augmentation on an adequate mesentery and re-establish intestinal continuity.
2) Close the ends of the segment with suture and open the antimesenteric surface.
3) Fold the segment of ileum for augmentation cystoplasty and sew it upon itself. This detubularizes the segment, reduces enteric contractions, and maximizes the volume that the segment contributes to urinary storage.
4) Anastomose the augmenting segment to the prepared bladder. Perform a wide-mouthed anastomosis to ensure that the augméntation is spherical.
Augmentation enterocystoplasty
Management of neuropathic bladder dysfuction:
a. Regular urologic care and prompt and vigorous treatment of infection
b. Regular emptying of the bladder
c. Meds to improve bladder storage and continence
d. Surgical procedure
CAUSE of mongolism
unknown; multiple causality
a. Advanced maternal and paternal age
* 35 y/o= 1: 1,500
* 40 y/o= 1:100
b. Hereditary = translocation
taking of folic acid supplements
of ***** each day for the month before and the first three months of pregnancy can reduce
the risk of neural tube defects
0.5 mg.
PRENATAL diagnosis for SB:
* Elevated AFP =(+) NTD =anencephaly, myelomenigocele; omphalocele; SCI Decreased =chromosomal disorder
* Best done at 16-18 weeks because after this time, AFP concentrations normally diminish + can do therapeutic
abortion just in case
Amniocentesis
what to cure in hearing in mongolism that is required to prevent hearing loss (hearing
loss influences cognitive function)
otitis media
measures to minimize ↑ICP
- minimize stimulation=quiet environment
- elevate HOB 30 degrees unless c/i
- administer osmotic diuretic, corticosteroid as needed=monitor I & O, no IVF at scalp
- Failure of the posterior arches to fuse
- Mildest form
- Occult-hidden, not visible; may be identified by a dimple or the pressure of tuft of hair at the point of defect
- Child asymptomatic/with slight deficit-no treatment necessary
SPINA BIFIDA OCCULTA
Anterior fontanel closes at
12-18mos
Function of CSF:
- to take food to the brain and spinal cord
- excretes metabolites
- act as a shock absorber
to rapid reduction of ventricular size may cause the cerebral cortex to pull away from the dura and tear the small interlacing veins ==
subdural hematoma
GLASGOW COMA SCALE IN INFANTS & CHILDREN: Eye opening in infants
4 Spontaneous opening
3 To loud noise
2 To pain
1 No response
diagnosis of mongolism:
▪ Done at 14-18th week
Amniocentecis
s/s of ↑ICP
↑RR, BT and BP (↑SBP); ↓PR
preop position to Prevent trauma to the sac in SB
prone with hips slightly flexed
and legs abducted with a pad between
knees (ex. Rolled towel
Management for hydrocephalus
- Identify cause – infection/tumor
- Treat cause – surgery, antibiotics
- Managing hydrocephalus: placement if shunt
acquired causes of hydrocephalus
- Neoplasm
- Hemorrhage
- Infection Clinical
diagnosis of mongolism:
▪ Most commonly done at 8-10th week
▪ Retrieval and analysis of chorionic villi for
chromosome analysis
Chorionic Villi Sampling CVS
3 reasons of Excess CSF in the Newborn
- Overproduction of fluid by choroid plexus in the lateral ventricles
- Obstruction of the passage of fluid somewhere between the point of origin and the point of absorption
- Interference with the absorption of fluid from subarachnoid
Nursing care for hydrocephalus
- Provide pre-operative care
- Provide post-op care
- Emotional support to parents=fear of retardation or brain damage
- Discharge teachings
→ Most serious complication of shunt placement
→ Most prevalent in the first 2 months after placement
→ Post op ileus and peritonitis in VP shunt
infection
severe pain comfort measure in hydrocephalus
opioids
Splitting of the already closed neural tube as a result of an abnormal CSF pressure in the first trimester
→ May involve entire length/restricted to a small area
→ The most common defect of the CNS
→ The higher the defect, the greater the neurologic dysfunction
spina bifida
NURSING DIAGNOSIS:
➢ High risk for infection r/t presence of shunt
➢ Impaired physical mobility r/t increased weight of head
➢ High risk for injury r/t shunt blockage
How shunts are applied
- A flexible tube is inserted into the lateral ventricle of brain
- A catheter is threaded under the skin (from the ventricles to the peritoneum)→fluid is cleansed and absorbed across the peritoneal membrane then to the circulation
- A subcutaneous pump (inserted in patients with hydrocephalus) may be attached to ensure patency
- Fluid is absorbed in peritoneum or enters special circulation via the right atrium
is clear and colorless. It bathes the surface of the brain
in the subarachnoid space between pia &
arachnoid matter.
CSF
management for mongolism
→ No cure
1. Surgeries to correct serious congenital anomalies
2. Regular medical care
Manifestations of hydrocephalus in infancy, late
➢ Bossing-frontal enlargement, brow bulges
➢ Depressed eyes
➢ Setting sun sign
➢ Pupils sluggish, unequal response to light
septal defect associated with mongolism
AVC
diagnosis for hydrocephalus:
o Injection of air
o Determine what part of the brain has
blockage of CSF
Ventriculography & pneumocephalography
diagnosis for mongolism
Karyotyping
Karyotype
1. Chorionic Villi Sampling CVS
2. Amniocentecis
3. Percutaneous Umbilical Blood Sampling (PUBS)
physical CLINICAL MANIFESTATIONS of mongolism in musculoskeletal
a. Short and thick hands with little finger curved inward (clindactyl), with single transverse palmar crease instead of 3 (Simon line)
b. Short, broad feet with wide separation between the 1st and 2nd toe
c. Poor muscle tone/marked hypotonia=ragdoll appearance; floppy
d. Hyperflexibility/hyperextension of joints
Provide optimal development in mongolism
Verbal stimulation and providing opportunities to master their skill
Prevent trauma to the sac in SB
➢ Cover the sac with a sterile saline
dressing to protect its integrity
(dressing changed every 2-4 hours)
➢ Position=prone with hips slightly flexed
and legs abducted with a pad between
knees (ex. Rolled towel) to minimize
tension on the sac
➢ Doughnut ring cushion on site to
prevent pressure
➢ No diaper until site has been healed
diagnosis for hydrocephalus:
- History and clinical manifestation
* Time of onset
* Presence of pre-existing lesions - Head circumference measurement daily =
occipitofrontal - CT scan and MRI
- Echoencephalography
- Skull X-ray
- Serial transillumination
- Ventriculography & pneumocephalography
- Lumbar/ventricular tap
PROGNOSIS of mongolism
80% survive to 30 years and beyond
PROGNOSIS of hydrocephalus
Dependent on cause (malignant tumor have high mortality rate), rate hydrocephalus develops, duration of raised ICP and frequency of complications
atlantoaxial instability is at risk for
spinal cord compression
Normal ICP
bulging/tense fontanel under circumstances (ex. Crying)
how to provide nutrition for mongolism
▪ Small frequent feeding
▪ Allow opportunities to rest at mealtime
▪ Use small but long, straight handled spoon to push the food toward the back and side of the mouth
▪ Monitor nutritional intake based on height and weight→obesity may result if too much food is given
surgical procedure for neuropathic bladder dysfunction: stoma created on the abdominal wall for urinary drainage
vesicostomy
cracked pot sound on percussion (sutures/bones of the brain are separate)
Macewen’s sign
causes of shunt failure
blockage, kinking, breakdown, migration
diagnosis for hydrocephalus: (neonates-infants
whose fontanels are open) to confirm
diagnosis and to compare the ration of lateral ventricle to cortex
echoencephalography
Assess dressing regularly hydrocephalus. leakage CSF if ***
+ glucose
how to Regular emptying of the bladder
clean intermittent catheterization (CIC)
Other name of communicating hydrocephalus
Extraventricular, Unobstructive
s/s of shunt malfunction
↑ICP; neurologic checks (Glasgow coma scale)
Short and thick hands with little finger curved inward
(clindactyl)
▪ Child’s diaper should be below the peritoneal dressing site/suture line for prevention of infection in hydrocephalus
Characterized by increased amount of CSF withinventricles and subarachnoid space resulting to
enlargement of the head.
HYDROCEPHALUS
Short webbed neck with excess loose skin
(puppy’s neck)
s/s of shunt malfunction in older children
o alterations in LOC; how they interact with the environment and compare with previous behavior or habits
PRENATAL diagnosis for SB: not conclusive/specific for NTD (neural tube defect)
Elevated MSAFP
TYPES OF SPINA BIFIDA
SPINA BIFIDA OCCULTA
SPINA BIFIDA CYSTICA
goals of bladder elimination in infant
to preserve renal function
type of hydrocephalus: Reduced absorption of CSF within the subarachnoid space
Communicating
pre-operative care hydrocephalus
a. Measure head circumference daily
b. Monitor for s/s of ↑ICP
c. positioning
d. reduce chances of skin breakdown
e. measures to minimize ↑ICP
f. nutrition
* frequent small feedings and frequent burping =prone to vomiting
Posterior fontanel closes at
2-3mos.
Failure of the posterior vertebral arches to fuse (or neural tubes to close) during embryologic development, which is usually between *** of fetal life. Normally, spinal cord and cauda equina are enclosed in protective sheath of bone and meninges
2-4 weeks
mild pain comfort measure in hydrocephalus
acetaminophen
Meds to improve bladder storage and continence
Anticholinergic pro-banthine
Clinical manifestation of hydrocephalus in older infants and children for diagnosis
Neurologic signs through pressure on adjacent
structures before causing CSF obstruction →↑ICP; manifestation r/t focal lesion
Clinical manifestation of hydrocephalus in infant for diagnosis. Also the predominant sign
Head enlargement
Manifestations of hydrocephalus in infancy (general)
➢ Irritability/lethargy
➢ Persistent infantile reflex acts-abnormal responses
➢ Decline in LOC (level of consciousness
➢ Late signs:
o Shrill, high pitched cry
o Difficulty swallowing/feeding
o Cardiopulmonary depression (severe cases)
o Seizures
diagnosis of mongolism:
▪ Done at 18-22th week
▪ Greatest risk of miscarriage
▪ Most accurate to confirm CVS and
amniocentecis
Percutaneous Umbilical Blood Sampling (PUBS)
cause of SB
unknown (multifactorial inheritance)
* Genes
* environment- poor maternal nutrition
* Folic acid deficiency
Cause of hydrocephalus in older children
acquired
Nursing mgt for SB
- Provide pre-operative care
- Provide post op care
- Promote mobility
- Provide emotional support =
- Discharge planning and patient and family
home care
single transverse palmar crease instead of 3
(Simon line)
Manifestations of hydrocephalus in infancy, early
➢ Rapid head growth (cranial sutures may
separate) – increased head
circumference
➢ Tense or bulging fontanels; sometimes
without head enlargement
➢ Prominent scalp veins
➢ Translucent skin, Thinning of skull bones
➢ Macewen’s sign
preop, what to give to Prevent trauma to the sac in SB to prevent pressure
Doughnut ring cushion
Intellectual and Emotional Development manifestations of mongolism
- Some degree of cognitive impairment – most of them are capable of being trained to some degree
- Generally loving, happy, friendly honest and frank individuals
physical CLINICAL MANIFESTATIONS of mongolism in sexual development
a. Males
o Small, underdeveloped genitalia
→produce less testosterone
→ delayed/incomplete secondary sex characteristic
→ infertility
b. Females
o Breast tissue may develop moderately; menstruation
can occur normally
GLASGOW COMA SCALE IN INFANTS & CHILDREN: motor response in infants
6 Spontaneous movement
5 Withdraws to touch
4 Withdraws to pain
3 Abnormal flexion (Decorticate)
2 Abnormal extension (Decerebrate)
1 No response
diagnosis of mongolism:
→ Sample of peripheral venous blood/a sample of
cells from the buccal membrane is taken; cells
are allowed to grow (2 weeks) then observed
though a microscope
→ Shows the 3rd chromosome 21 that is attached to
another in translocation
→ Visual presentation of a chromosome pattern of an
individual
karyotyping
CSF is secreted by the
Choroid plexus.
A chromosomal abnormality involving an extra chromosome (21) characterized by a typical appearance and mental retardation
DOWN’S SYNDROME/TRISOMY 21/MONGOLISM
to determine causative organism for the major complications of myelomeningocele (meningitis)
Lab exams
mgt of SB so child will not lag significantly behind agemates
mobility
how to prevent infection in mongolism
▪ Teach parents how to use bulb type syringe to clear nose
▪ Rinse mouth after feeding
▪ Cool-mist vaporizer to keep mucous membrane moist and to liquefy secretions
▪ Changing position frequently
▪ Performing postural drainage and percussion if necessary
▪ Good handwashing
▪ Proper disposal of soiled articles
▪ Antibiotics as prescribed
type of hydrocephalus: Obstruction to the flow of CSF within the ventricles or to subarachnoid space
Non-communicating
Two types of Spina Bifida Cystica
MENINGOCELE
MYELOMENINGOCELE
Shunt:
→ Most commonly used type
→ CSF drains from the ventricles to peritoneal cavity – fluid is
absorbed across the peritoneal membrane
Ventriculoperitoneal shunt (VP shunt)
Position of hydrocephalus in post operative
▪ Unoperative side to prevent pressure on pressure areas
▪ Flat as prescribed
▪ Gradually elevated not greater than 30 degrees if ↑ICP to prevent rapid CSF drainage
Amniocentesis is best done at *** because after this time, AFP concentrations normally diminish + can do therapeutic
abortion just in case
16-18 weeks
PRENATAL diagnosis for SB: considered for patients with family history of SB
Chorionic villi sampling (CVS)
discharge home care for SB
a. Position, feeding, exercise
b. Catheterize child
c. s/s of ↑ICP, hydrocephalus, shunt infection
and malfunction, UTI
d. safety issues
Causes of hydrocephalus
- Congenital
- Acquired (complication of illness/trauma)
o Encases meninges, spinal fluid but with no neural elements. Sac is covered with a skin
o Not associated with neurologic deficit
o Sac covering = translucent or membranous
o Spinal cord not affected
o Bony spine
o Sack containing spinal fluid
MENINGOCELE
o Treatment for hydrocephalus is most effective when disorder is recognized early because once ↑ ICP becomes so acute, the brain tissue is damaged and motor/mental deterioration results; the best shunting procedure can’t replace the damage to the brain cells
Point of greatest measurement; just above the brow, above the pinna of the ears and around the occipital prominence
occipitofrontal
types of hydrocephalus
Non-communicating
Communicating
causative conditions of noncommunicating hydrocephalus:
- Usually a result of development malformations (1-2 y/o)
o Arnold Chiari malformation
o Aqueduct stenosis, aqueduct gliosis, atresia of luschka and
magendie (Dandy Walker Syndrome), often associated with
myelomenigocele - History of prenatal infection
- Perinatal hemorrhage
- Neonatal meningoencephalitis
diagnosis for hydrocephalus: needs sedation to absolutely lie still→for an accurate picture to be produced
CT scan and MRI
GLASGOW COMA SCALE IN INFANTS & CHILDREN: motor response in adults
6 Obeys command
5 Localizes pain
4 Withdraws to pain
3 Flexion to pain (Decorticate)
2 Extension to pain (Decerebrate)
1 No response
PEDIATRIC DIFFERENCES
- Head heavy (large in proportion to body); neck muscles not well developed
- Cranial bones thin and not well developed; unfused sutures
- Brain highly vascular; Subarachnoid space small; dura firmly attached but can strip away from pericranium
- Excessive spinal mobility; muscles, joint capsule and ligaments of cervical spine immature
- Wedge-shaped, cartilaginous vertebral bodies; ossification of vertebral bodies incomplete
Postop hydration and nutrition for hydrocephalus
▪ NPO for first 24 hours
▪ Monitor IVF for fluid overload
most important indicator of manifestation in hydrocephalus; can lead to coma
decline in LOC (level of consciousness)
another name for spina bifida
(RACHISCHISIS/ SCHISTORRHACHISIS)
Poor muscle tone/marked hypotonia
ragdoll appearance
PRENATAL diagnosis for SB: shows fetal spinal defect and sac, AFP is increased if spinal cord defect is present
Ultrasound for scanning of uterus
Complications of Shunt Placement
- Shunt failure
- Infection
surgical procedure for neuropathic bladder dysfunction: → increases bladder capacity and reduces high bladder pressure
Augmentation enterocystoplasty
hallmark sign of hydrocephalus in infancy, late
setting sun sign
Cause of hydrocephalus in infants
congenital
s/s of shunt failure
recurrent hydrocephalus; confirmed by CT scan/MRI
postop care for hydrocephalus
a. Maintain patency of the shunt
b. Assess dressing regularly
c. Comfort measures
d. Hydration and nutrition
e. Prevention of infection
congenital causes of hydrocephalus
- Maldevelopment
- Intrauterine infection
GLASGOW COMA SCALE IN INFANTS & CHILDREN: verbal response in infants
5 Smiles, coos, cries to appropriate stimuli
4 Irritable; cries
3 Inappropriate crying
2 Grunts, moans
1 No response
surgical procedure for neuropathic bladder dysfunction
- Vesicostomy
- Augmentation enterocystoplasty
- Urinary diversion
DOWN’S SYNDROME/TRISOMY 21/MONGOLISM
most common chromosomal
abnormality
condition caused by an imbalance in the production and absorption of CSF (cerebrospinal fluid) in the ventricular system
HYDROCEPHALUS
spina bifida literally means
cleft spine, divided spine
NURSING CARE for mongolism
- Parent adjustment
- Provide physical care
- Prevention of infection
- Provide optimal development
naturally occurring folic acid
folate
sclera visible above iris, with upper lid retraction
setting sun sign
SB is one of the most common causes of
****→frequent
cause of UTI, ureterohydronephrosis, vesiculourethral
reflux)
neurogenic/neuropathic bladder dysfunction
3 types of Shunt in hydrocephalus
- Ventriculoperitoneal shunt (VP shunt)
- AV shunt (Atrioventricular Shunt)
- Ventriculoureteral shunt
o Contains meninges, spinal fluid and nerves; fluid leakage may occur
o Parallel to dysfunction=loss of muscle function to lower extremities, bowel and
o Bladder
o Lesion poorly covered with imperfect tissue
o Bony spine
o Spinal cord and spinal fluid contained in lump
MYELOMENINGOCELE
Other name of Non-communicating hydrocephalus
Intraventricular, Obstructive