- 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
* Chiari IV malformation
Hydrocephalus
- 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
