Neurologic Disorders: Cranial Defects and Malformations

Malformations of the Nervous System

Cranial Defects

• Craniosynostosis: one or more sutures close too soon, increasing ICP and interfering with normal brain growth, leading to MR
• Assessment:
  • Suture lines of the skull manually palpated
  • Radiographs taken to confirm diagnosis
• Microcephaly: brain fails to grow, may be due to chromosomal defect, drugs, toxins, or radiation, leading to MR
• Hydrocephalus:
  • Imbalance of CSF absorption or production
  • Caused by malformations, tumors, hemorrhage, infection, or trauma
  • Types: communicating (impaired absorption in arachnoid space) and non-communicating (obstruction of CSF flow in ventricular system)
  • Assessment:
    • Infant: increased HC, Macewen's sign, tense and bulging anterior fontanel, dilated scalp veins, frontal bossing, and sunsetting eyes
    • Child: behavior changes, headache, nausea, vomiting, ataxia, and nystagmus
• Surgical Implementation: VP shunt (CSF drains into peritoneal cavity) or AV shunt (CSF drains into right atrium)

Post-Operative Care

• Otitis media
• Observe for increased ICP and elevate HOB 15-30 degrees if present
• Monitor for infection
• Measure HC and I&O
• Provide comfort measures and administer medications (diuretics, antibiotics, or anticonvulsants)

Spina Bifida

• CNS defect due to neural tube failure to close during embryonic development
• Types:
  • Spina bifida occulta: posterior vertebral arches fail to close in lumbosacral area, spinal cord intact and not visible
  • Meningocoele: protrusion involves meninges and a sac-like structure
  • Myelomeningocoele: protrusion of meninges, CSF, nerve roots, and portion of spinal cord
• Assessment:
  • Depends on spinal cord involvement
  • Visible spinal defect
  • Flaccid paralysis of legs and altered bladder and bowel function
• Implementation:
  • Evaluate sac and measure lesion
  • Monitor for increased ICP and measure HC
  • Protect the sac with a sterile, moist dressing
  • Place in prone position and turn head to one side for feeding
  • Administer antibiotics, anticholinergics, and laxatives
  • Monitor for early signs of infection

Meningitis

• Infectious process of the CNS caused by bacteria and viruses
• Diagnosis: CSF analysis (increased pressure, cloudy CSF, high protein, and low glucose)
• Assessment:
  • Fever, chills, vomiting, diarrhea, poor feeding or anorexia, and altered LOC
  • Bulging anterior fontanel and nuchal rigidity
• Implementation:
  • Isolation for at least 24 hours after antibiotics are initiated
  • Administer antibiotics as prescribed
  • Monitor VS and neuro status
  • Monitor I and O and assess nutritional status
  • Determine close contacts of the child with meningitis

Seizure Disorders

• Sudden, transient alterations in brain function due to excessive 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:
  • Raise side rails and pad them
  • Place a waterproof mattress on the bed
  • Instruct the child to swim with a companion
  • Alert caregivers to the need for special precautions
• Emergency Treatment for Seizures:
  • Ensure patency of airways
  • Ease the child to the floor if standing or sitting
  • Place a pillow or folded blanket under the child's head
  • Loosen restrictive clothing
  • Clear the area of hazards
  • Turn the child to one side if vomiting occurs
  • Do not restrain the child or place anything in their mouth
  • Remain with the child until fully recovered
  • Prepare to administer medications

Cerebral Palsy

• Disorder characterized by impaired movement and posture due to an abnormality in the extrapyramidal motor system
• 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
• Implementation:
  • Early recognition and intervention
  • PT, OT, speech therapy, education, and recreation
  • Assess the child's developmental level and intelligence
  • Encourage communication and interaction with the child on a functional level
  • Provide a safe environment
  • Position upright after meals
  • Provide safe, appropriate toys for age and developmental level

EENT Disorders

• Diplopia (Double Vision):
  • Vision occurs due to light rays reflecting from an object through the cornea, aqueous humors, lenses, and vitreous humors to the retinas
  • Defects in these structures cause vision disturbance