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Monday 29 August 2011

NEURO PEDIATRICS


MALFORMATIONS OF THE NERVOUS SYSTEM

Cranial Defects:
  1. Craniosynostosis
           one or more of the sutures close too soon
          ICP increases; interfeeres with normal brain growth à MR

Assessment: 
          suture lines of the skull manually palpated
          Radiographs are made to confirm

  1. Microcephaly
          The brain fails to grow
          May be due to a chromosomal defect or from drugs, toxins or radiation
          MR
  1. Hydrocephalus
          Imbalance of CSF absorption or production
          caused by malformations, tumors, hemorrhage, infection, trauma

Types:
  1. Communicating – impaired absorption within arachnoid space
  2. Non-communicating – obstruction of CSF flow within the ventricular system

Assessment:
          Infant – increased HC
          Macewen’s sign – cracked-pot sound on percussion of bones of head
          Anterior fontanel tense, bulging
          Scalp veins dilated
          Frontal bossing, sunsetting eyes
          Child – behavior changes
          Headache, nausea and vomiting
          Ataxia, nystagmus

Surgical Implementation:  Hydrocephalus

Goal: to prevent further CSF accumulation by bypassing the blockage and
            draining the fluid from the ventricles to a location where it may be
            reabsorbed

  1. VP Shunt – CSF drains into the peritoneal cavity from the lateral ventricle
  2. AV shunt – CSF drains into the right atrium

PostOP Care:
  1. Keep child flat as prescribed – to avoid rapid reduction of intracranial fluid
  2. Observe increase ICP – if present, elevate HOB 15-30 deg
  3. Monitor for infection
  4. Measure HC
  5. Monitor I and O
  6. Provide comfort measures; administer medications (diuretics, antibiotics, or anticonvulsants)
  1. Toddler – headache and anorexia à earliest common signs of shunt malfunction

SPINA BIFIDA
            CNS defect that occurs as a result of neural tube failure to
            close during embryonic development
            defect closure usually done during infancy

Types:
  1. Spina bifida occulta
          Posterior vertebral arches fail to close in the lumbosacral area
          Spinal cord intact; not visible
          Meninges not exposed on the skin surfaces
  1. Meningocoele
          Protrusion involves meninges and a sac-like cyst
          Lumbosacral area
  1. Myelomeningocoele
          Protrusion of meninges, CSF, nerve roots, portion of spinal cord
          Sac covered by a thin membrane à may rupture or leak
          Neuro deficit evidence

Assessment:
          Depends on spinal cord involvement
          Visible spinal defect
          Flaccid paralysis of legs
          Altered bladder and bowel function

Implementation:
            Evaluate sac; measure lesion
            neuro check
            monitor for increase ICP
            measure HC; assess fontanelles
            Protect the sac
            1.  Cover with sterile, moist (normal saline) non-adherent
                        dressing
            2.  Change dressing every 2-4 hours
            Place prone position
            head is turned to one side for feeding
            diapering may be C/I  until defect repaired
            Aseptic technique
            Watch for early signs of infection
            Administer antibiotics
            Administer anticholinergics – improve urinary continence
            Administer laxatives , antispasmodics

MENINGITIS
            infectious process of the CNS caused by bacteria and viruses
            acquired as a primary or as a result of complications
            diagnosis – CSF analysis (increase pressure, cloudy  CSF, high protein, low glucose
            bacterial or viral

Assessment:
            signs and symptoms vary depending of age group
            fever, chills
            vomiting, diarrhea
            poor feeding  or anorexia
            altered LOC
            bulging anterior fontanel
            nuchal rigidity

Implementation:
            isolation; maintain for at least 24 hours after antibiotics are initiated
            administer antibiotics as prescribed
            monitor VS and neuro status
            Monitor I and O
            assess nutritional status
            determine close contacts of the child with meningitis

SEIZURE DISORDERS
            Sudden transient alterations in brain function resulting from excessive levels of electrical activity in the brain

Assessment:
            obtain information from parents about the time of onset,  precipitating events and behavior before and after the seizure
            seizure precautions:
  1. Raise side rails
  2. Pad side rails
  3. Place waterproof mattress on bed
  4. Instruct child to swim with companion
  5. Alert caregivers to the need for special precautions

Emergency Treatment for Seizures:
            Ensure patency of airways
            If the child is standing or sitting, ease the child down to the floor
            place pillow or folded blanket under the child’s head
            loosen restrictive clothing
            clear area of any hazards
            if vomiting occurs, turn child to one side
            do not restrain child; do not place anything in the child’smouth
            Remain with the child until fully recovers
            Prepare to administer medications



CEREBRAL PALSY
            disorder characterized by impaired movement and posture resulting from an abnormality in the extrapyramidal motor system
            spastic type- most common

Assessment:
            extreme irritability and crying
            feeding difficulties
            stiff and rigid arms and legs
            delayed gross development
            abnormal motor performance
            alterations of muscle tone
            abnormal posturing
            persistence of primitive reflexes

Implementattion:
            early recognition
            PT, OT, speech therapy, eduaction and recreation
            assess the child’s developmental level and intelligence
            early intervention
            encourage communication and interaction with the child on a functional level
            provide safe environment
            position upright after meals
            provide safe, appropriate toys for age and developmental level

NEURAL TUBE DISORDERS
·         “SPINA BIFIDA”
o    Latin for “divided spine”
·         “CLEFT SPINE”
·         All of the disorder occur because of lack of fusion of the posterior surface of the embryo in the early intrauterine life
·         ‘closure disorder’
·         INCIDENCE:
o    May occur as a polygenic inheritance pattern
o    poor nutrition – folic acid deficiency
·         TYPES OF DISORDERS
o    ANENCEPHALY
§  Absence of the cerebral hemispheres
§  Occurs when the upper end of the neural tube fails to close in early intrauterine life
§  Elevated AFP during pregnancy
§  Incompatible to life
o    MICROCEPHALY
§  d/o in which brain growth is so slow that it fails more than three standard deviations below normal on growth charts
§  associated with: rubella, cytomegalovirus, or toxoplasmosis
§  Cognitively challenged

o    SPINA BIFIDA OCCULTA
§  Occurs when the posterior laminae of the vertebrae fails to fuse
§  Occurs most commonly at the fifth lumbar or 1st sacral level
§  Characteristic:
·         Dimpling at the point of poor fusion
·         Abnormal tufts of hair or discolored skin may be present

o    MENINGOCOELE
§  Herniation of the meninges covering the spinal cord through unformed vertebrae
§  Presence of orange, protruding mass at the center of the back
§  Occurs in the lumbar region
o    MYELOMENINGOCOELE
§  The spinal cord and the meninges protrude through the vertebrae
§  The spinal cord ends at the point resulting to the absence of sensory and motor function at the point of the d/o
§  Flaccidity
§  Lack of sensation of the lower extremities
§  Loss of bowel and bladder control
§  Hydrocephalus accompaniment
o    ENCEPHALOCOELE
§  Cranial meningocoele
§  Occurs most often in the occipital area of the skull but may occur as a nasal or nasopharyngeal d/o
·         ASSESSMENT
o    Check the nature and pattern of voiding and defecation
o    “voids continually”
o    “defecates continually”
o    Pressure on the protruding mass can rupture the sac, leading to a quick decompression of the CSF
§  May lead to herniation of the brain stem into the spinal cord and interference with respiratory and cardiac centers
·         NURSING DIAGNOSIS
o    Risk for infection
o    Impaired physical mobility
o    Risk for impaired elimination
·         MANAGEMENT
o    Parents should be made aware about the defect
o    Avoid drying of the sac to prevent infection and CSF drainage
o    Prone or side-lying position
o    Use a rolled blanket or diaper placed behind the child’s upper back (above the d/o) and a separate one behind their lower back (below the d/o)
o    May cover the area with sterile wet dressing
o    Avoid rupturing the sac!
o    Always check any leakage for evidence of glucose to confirm if the fluid is CSF

ARNOLD-CHIARRI MALFORMATION
·         Overgrowth of the neural tube in weeks 16 to 20 weeks of fetal life
·         Projection of the cerebellum, medulla oblongata, and 4th ventricle into the cervical canal
o    This projection causes the upper cervical spinal cord to jackknife backward, obstructing CSF flow and causing hydrocephalus
·         Absent gagging and swallowing reflexes
o    Increasing risk for tracheal aspiration
REYE’S SYNDROME
·         Acute encephalitis with accompanying fatty infiltration of the liver, heart, lungs, pancreas, and skeletal muscle
·         Occurs in children 1 to 8 years of age regardless of gender
·         Cause: unknown
·         Possible cause: after a viral infection
·         FATAL!
·         ASSESSMENT
o    Lethargy
o    Agitation
o    Anorexia
o    Confusion
o    Combativeness
o    Mimic the sx of drug intoxication
o    Progression of cerebral symptoms
§  From confusion to stupor to deep coma
o    Seizures
o    Respiratory arrest due to pressure on the brainstem
o    Elevated ALT or SGPT and AST – confirmatory
o    Definitive diagnosis- fatty infiltration on liver biopsy
·         MANAGEMENT
o    Directed toward the symptoms
STURGE-WEBER’S SYNDROME
·         ENCEPHALOFACIAL ANGIOMATOSIS
·         Congenital port-wine birthmark on the skin of the upper part of the face that extends inward to the meninges and choroid plexus
·         Defect is unilateral
·         Blood flow is sluggish and anoxia may develop
·         ASSESSMENT
o    Hemiparesis on the side opposite the lesion
§  Due to destruction of motor neurons
o    Intractable seizures
o    Calcification of the cerebral cortex
·         MANAGEMENT
o    Surgery

NEUROFIBROMATOSIS
·         VON RECKLINGHAUSEN’S DISEASE
·         Unexplained development of subcutaneous tumors
·         Can occur as mutation or inherited as an autosomal dominant trait carried by the chromosome 17
·         With presence of irregular but excessive skin pigmentation
·         Characteristic: presence of more than 6 spots larger than 1 cm in diameter
·         With hearing loss
·         Vision impairment
·         FATAL!