Congenital Spinal Deformities Flashcards
1. Describe the different types of spina bifida. 2. Describe the sequelae associated with spina bifida. 3. Describe the management of spina bifida occulta. 4. Describe syringomyelia and possible etiologies. 5. Describe the symptoms and general treatment of syringomyelia.
Neural Tube Defects
Spina Bifida
Syringomyelia
Spina Bifida
split in 2
Definition: congential defect in the walls of the spinal canal caused by lack of union (non-fusion) of vertebral dorsal arches (lamina)
Spina Bifida
1.Occurs during 1st trimester of pregnancy
>primary form in lumbrosacral region>thoracic region
2. Unknown etiology (viruses, radiation, environmental factors)
3. Incidence 1:1000 Us births 95% or 12ooo/year) Repeat incidence 1:40(genetics)
Figure 1 Development of the Spinal Cord
- early development of embryo’s head and brain-Neural fold begins to form 20 days
- Neural Tube is closing 21 days
- 28 days defect can begin to shoW
Who is at higher risk?
Women who :
1. have a child with spina bifida
>repeat incidence 1:40 range of 1-5:100
2. Have spina Bifida themselves 1-5%
3. If both parents have spina Bifida >15%
4. Have already had a pregnanvy affected by any type of neural tube defect> incomplete development of brain, spinal cord, or meninges
ex. ancephaly, Encephalocele, Spina Bifida
Spina Bifida Subclassifications
Spina Bifida Occulta
Spina Bifida Cystica
Spina Bifida Occulta
- Occulta=hidden, not visible
- Most common/least severe
- 5-10% of population
- Simple failure of vertebrae to close without neurological defects
- Usually no skin changes,
- Leads to low back pain
Associated Skin Defects
- 80% of people with spinal cord or spinal column abnormalities will have some type of skin abnormality over the defect
- Examples include:
- Hairy patch
- Fatty lump
- Hemangioma
- Birth mark
- Skin tract (tunnel) deep dimple
- Hypo-pigmented spot decreased skin color
Spina Bifida with Cyst Subclassifications
Spina Bifida Cystica: 3 types 1.Meningocele 2.Meningomyelocele 3.Myelocele 1.Meningo = brain/sc membranes/meninges 2.Myelo = spinal cord -- Also bone marrow, ex. Myeloblast 3.Cele = hernia
Common to all spina Bifida Cystica Deformities
- 80% have 80 or higher IQ
- Mental Retardation rare, but significant when occurs
- Learning disorders possible
- Visual and Speech Problems
- Associated with other deformities
»dislocated hips, Club feet, Scoliophosis - Osteoporosis
- Kidney abnormalities
- Changes in bowel and bladder
>UTI or constipation problems - Emotional Disorders
Meningocele
10% of Spina Bifida Cases
Meningeal cyst present without any nervous elements or deficits
Open spine with CSF-filled meningeal cyst on dura mater
Prognosis good - No spinal cord/root involvement
Skin is closed over deficit
No M/S limb deficit
No bowel/bladder problems
If not repaired surgically, may present new problems later in life
Meningomyelocele
- 90% of Spina Bifida cases
- Open spine and skin with exposed cord and roots
- Prognosis depends on location & degree of myelodysplasia
a. Cyst has both meninges and spinal cord in thoracic region
b. Cyst has both meninges and cauda equina in lumbar region
c. Paraplegia with bowel (S2-S3) and bladder (S2-S4) incontinence
d. Hydrocephalus( water in the brain–very frequent) 70-90% in children
- -MR without treatment
- -Symptoms due to Arnold-Chiari malformation 33%
Myelocele
3rd level
1% of all spina bifida cases
Neural folds are open and CSF WEEPS into the skin
Cyst is deep to the spinal Cord—-NOT BETWEEN THE CORD AND SKIN
How is Spina Bifida Treated
- A child with the most severe form is operated on within 24 hours after birth
- -surgery minimizes the risk of infection and preserves existing/remaining spinal cord function - Spina Bifida occulta usually requires no treatment
- Meningocele can usually be repaired with no paralysis
Management of Myelomeningocele
If known, deliver by caesarian section
Surgical closure of back if possible
Hydrocephalus shunting
Manage hydrocephalus, bowel/bladder, orthopedic consequences, psycho-social problems
Left untreated most children survive [with scarring 6 weeks post-natal]
Functioning relates to societies commitment for medical care, appropriate schooling, and transportation availability