Tuesday, October 20, 2009

Complications of Spina Bifida

  • Ranges from minor physical problems to severe physical & mental disabilities.
  • Intelligence level is usually normal.
  • Generally all nerves below the malformation are affected.

Severity is determined by
  • Size & location of malformation
  • Whether or not the skin covers it
  • Whether or not spinal nerves protrude from it
  • Which spinal nerves are involved

Complications
  • Loss of sensation (esp. In lower limbs)
  • Paralysis (esp. In lower limbs)
  • Loss of normal bowel & bladder control
  • Urinary tract infection
  • Orthopedic problems (scoliosis, kyphosis, & clubfoot)
  • Lipomeningocele
  • Spinal cord tethering
  • Chiari II malformation
  • Hydrocephalus
  • Meningitis
  • Learning disabilities
  • Latex allergies
  • Gastrointestinal conditions
  • Depression
  • Obesity

Bladder complications
  • Early catheterization to preserve kidney & bladder functions
  • Further complicated by
    • Bladder stones
    • Bladder ruptures
    • Bladder tumors

Lipomeningocele
  • Congenital lesion that is associated with spina biida
  • Associated with abnormal fat accumulation that starts below the level of the skin & extends through the bony opening to the spinal cord
  • Females are 50% more likely to develop lipomeningocele
  • Damage is due to
    • Tethering of spinal cord
    • Compression due to progressive deposition of fat
  • Signs & Symptoms
    • Obvious soft tissue swelling over the spine in the lumbosacral region in more than 90% cases
    • Lesion is covered by skin & painless
    • Weakness (symmetrical/assymetrical)
    • Bladder & bowel incontinence
    • Pain of variable quality may be significant in adolescence & adulthood (esp. Radiation)
    • Loss of back mobility
  • Diagnosed with MRI
  • Managed with surgery

Spinal cord tethering
  • Literally low-lying spinal cord
  • Spinal cord remains attached to the surrounding skin, causing abnormal growth
  • Spinal cord becomes stretched & damaged
  • Results in
    • Progressive neurological, urological, or orthopaedic problems
  • Managed with surgery
  • Preventive strategies disappointing

* Chiari I malformation
  • Occurs when the lowest part of the cerebellum (cerebellar tonsils) descends a short distance into the cervical spine
  • No involvement of the brain stem

Chiari II malformation (Arnold-Chiari malformation)
  • Usually a rare condition
  • Common in children with myelomeningocele
  • Extension of both brainstem & cerebellar tissues into the foramen magnum
  • Cerebellar vermis (tissues connecting both cerebellum hemispheres) may only be partially present
  • Leading cause of death in the spina bifida population
  • Results in
    • Spinal cord compression
  • Signs & Symptoms
    • Difficulties with feeding, swallowing & breathing
    • Choking
    • Arm stiffness
  • Complications
    • Blockage of cerebrospinal fluid – HYDROCEPHALUS
  • Diagnosed with MRI
  • Managed with surgery

* Chiari III malformation
  • Occurs when the cerebellum & brainstem herniate through the foramen magnum & into the spinal cord
  • Part of the brain's fourth ventricle may also be involved
  • Rare instances of associated occipital encephalocele

* Chiari IV malformation
  • Occurs when there is incomplete or underdeveloped cerebellum (cerebellar hypoplasia)
  • Cerebellar tonsils are located further down the spinal canal
  • Parts of cerebellum are missing
  • Portions of skull & spinal cord may be visible

Hydrocephalus
  • An abnormal buildup of cerebrospinal fluid in the brain
  • Excessive buildup places damaging pressure on the brain
  • Managed by ventriculoperitoneal shunt insertion to drain cerebrospinal fluid

Meningitis
  • Infection of the meninges
  • Potentially cause brain injury
  • Potentially fatal

Learning disabilities
  • Difficulty paying attention
  • Problems with language & reading comprehension
  • Trouble learning mathematics
  • Perceptual motor problems (poor eye-hand coordination & ineffective motor skills)
  • Hyperactivity/Impulsivity
  • Poor memory
  • Poor organization
  • Problems in sequencing tasks
  • Difficulty in decision making/problem solving

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