Congenital Spinal Deformities

Question 1

Neural Tube Defects

Answer 1

Spina Bifida

Syringomyelia

Question 2

Spina Bifida

split in 2

Answer 2

Definition: congential defect in the walls of the spinal canal caused by lack of union (non-fusion) of vertebral dorsal arches (lamina)

Question 3

Spina Bifida

Answer 3

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)

Question 4

Figure 1 Development of the Spinal Cord

Answer 4

1. early development of embryo’s head and brain-Neural fold begins to form 20 days
2. Neural Tube is closing 21 days
3. 28 days defect can begin to shoW

Question 5

Who is at higher risk?

Answer 5

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

Question 6

Spina Bifida Subclassifications

Answer 6

Spina Bifida Occulta

Spina Bifida Cystica

Question 7

Spina Bifida Occulta

Answer 7

1. Occulta=hidden, not visible
2. Most common/least severe
3. 5-10% of population
4. Simple failure of vertebrae to close without neurological defects
5. Usually no skin changes,
6. Leads to low back pain

Question 8

Associated Skin Defects

Answer 8

1. 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

Question 9

Spina Bifida with Cyst Subclassifications

Answer 9

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

Question 10

Common to all spina Bifida Cystica Deformities

Answer 10

1. 80% have 80 or higher IQ
2. Mental Retardation rare, but significant when occurs
3. Learning disorders possible
4. Visual and Speech Problems
5. Associated with other deformities
   »dislocated hips, Club feet, Scoliophosis
6. Osteoporosis
7. Kidney abnormalities
8. Changes in bowel and bladder
   >UTI or constipation problems
9. Emotional Disorders

Question 11

Meningocele

Answer 11

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

Question 12

Meningomyelocele

Answer 12

1. 90% of Spina Bifida cases
2. Open spine and skin with exposed cord and roots
3. 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%

Question 13

Myelocele

3rd level

Answer 13

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

Question 14

How is Spina Bifida Treated

Answer 14

1. 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
2. Spina Bifida occulta usually requires no treatment
3. Meningocele can usually be repaired with no paralysis

Question 15

Management of Myelomeningocele

Answer 15

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

Question 16

Spina Bifida: Prevention

Answer 16

1. There is a 75% reduction of spina bifida incidence in all women of childbearing age who take 0.4 milligrams (400 micrograms) folic acid prior to becoming pregnant and throughout the 1st trimester
   a. Women are encouraged to take a multivitamin containing 400 mcg of folic acid daily
2. Folic acid, a common water-soluble B vitamin, is essential for human body functioning.
   a. Folic acid requirements increase during periods of rapid growth – pregnancy and fetal development.
   b. In broccoli, spinach, egg yolks, & fruits (OJ)

Question 17

Syringomyelia

Answer 17

“Syrinx”=fusiform cyst in or beside the CENTRAL CANAL of the spinal cord

2. Damages central white matter causing pain and temprature sensory deficits (spinothalamic tracts crossing)
3. Dorsal Column O.K. (touch, vibration, proprioception, stereognosis, etc.)

Question 18

Syringomyelia

Answer 18

1. Dissection of CSF through the ependyma cells to form cavitation or “syrinx” within the substance of the spinal cord
2. Diagnosed by MRI in combination with S/S
3. Pain and temperature loss in “cape” distribution; preserved touch and position sense

Question 19

Syringomyelia: Etiology

Answer 19

Obstruction of CSF flow  redirecting it into the spinal canal ? Syrinx formation
1.Congenital development problems
Arnold Chiari I malformation
2.Trauma to spinal cord
Car accident, serious fall
Meningitis, hemorrhage, tumor
3.Sometimes unknown – inherited???

Question 20

Symptoms of Syringomyelia

Answer 20

Pain (extremity, cervical, occipital)
Weakness and atrophy (shoulder, arm, hand)
Spasticity
Stiffness
In back, shoulders, arms or legs
Headaches
Loss of extreme temperature sensation
Especially in hands
Syringomyelia may adversely affect sweating, sexual function, bladder/bowel control
Condition may lie dormant and undetected until symptoms become bothersome

Question 21

Syringomyelia: Treatment

Answer 21

A. Surgical Intervention
1. Surgically provide space for cerebellum and upper cervical cord without entering CNS (arnold Chiari I)
2. Remove tumor if present
3. Shunt to drain syrinx
4. Plug central Canal of cyst with gause–50% improve, 25% deteriorate, 25% unchanged
B. Avoid Straining
C. Without Symptoms, SM is usually not treated