2. Learning outcomes :
After studying this module, Students should be able to :
Define correctly what is the spina bifida .
List the possible causes of spina bifida
Describe the different types of spina bifida
Describe the clinical pictures of spina bifida
Demonstrate the dignostic and treatement measures .
Demonstrate the social impact of spina bifida.
Explain the preventive measures of spina bifida .
Discuss what information is required by mothers prior to
discharge.
3.
4.
5. “Split Spine” caused by incomplete
closure of the neural tube, usually in
the lumbar or sacral region
What is spina bifida?
6. Incidence and epidimology
Worldwide incidence is 1-2 cases in 1000
births
US incidence is 0.7 per 1000 live births
East coast higher than West coast
Slightly higher incidence in Caucasian
population
Irish immigrants also have a higher risk
Seen more in children born in late
summer and early fall
9. Types of spina bifida?
Several classifications that vary in
severity depending on location and
extent of opening
1-Spina bifida occulta
2-Spina bifida cystica
meningocele
myelomenigocele
3-Spina bifida ventralis
10. 1-Spina bifida occulta – “hidden”
The bony vertebra is open, but the spine is within the spinal canal
The skin may have a lipoma (small benign fatty tumor), some
discoloration (birthmark), or a small tuft of hair overlying the spinal
defect
Most patients with spina bifida occulta do not know they have it
There may be tethering of the spinal cord
11. Spina bifida occulta – tethered spinal cord
Often occurs later in life
Caused by limitations of movement of the spinal cord within the
spinal column
Patients often have low back pain, weakness in the legs, and/or
incontinence depending on the site of tethering
http://www.uwhealth.org/image
s/ewebeditpro2/upload/6144_Fi
gure_1.jpg
12. 2-Spina bifida cystica – meningocele
The bony vertebra is open, part of the meninges is protruding out of
the spinal canal
Since the spinal cord is not protruding, there is often normal
function
Some cases of tethering have been reported
13. Spina bifida cystica – myelomeningocele
The bony vertebra is open, part of the meninges and part or all of
the spinal cord is protruding out of the spinal canal
Since the spinal cord is protruding, it is often not fully developed
Involved nerve roots are often not developed resulting in weakness,
pain, and/or paralysis
14. Spina bifida cystica – myelomeningocele…. (continoue
Arnold Chiari malformation II is often associated with myelomeningocele and occurs
when the cerebellum is forced downward
This can result in life-threatening situations because the build-up of cerebrospinal
fluid can cause pressure on the brain
Patients with Arnold Chiari malformations often require placement of a shunt to
drain the excess fluid
http://www.thefetus.net/images/article-images/central_nervous_system/arnold_chiari_files/image001.jpg
15. Spina bifida ventralis – anterior opening
Much less common than other forms of spina bifida
Meningeal sac will protrude into the retroperitoneal space and impinge on
retroperitoneal organs such as the duodenum, ascending/descending colon, kidneys,
adrenal glands, pancreas, aorta, and inferior vena cava
http://myweb.lsbu.ac.uk/dirt/museum/margaret/871-3398-2082230.jpg
16.
17. causes spina bifida
- The exact cause of spina bifida is
unknown
- All research to date has indicated both
a genetic and environmental influence
- The developmental process that results
in spina bifida is well studied
18. Causes of spina bifida(continoue
Neural Tube Defect:
When the neural tube fails to properly close during the
4th
week embryogenesis.
20. What would prevent the neural tubes from
closing properly?
Folate seems to play a large role in the closing of the neural
tube – but it is unknown exactly how folate works in this process.
Folate influence was discovered by the increased incidence in
spina bifida seen in Irish babies born in late summer and early fall.
The lack of leafy green vegetables caused the mother to have low
levels of folate during conception.
Genetics also play a role in the development of spina bifida.
Mothers with one child with spina bifida have an increased risk
of additional children having spina bifida
Studies with folate-resistant mice
21. Valproic acid (Depakote)
Oral medication used to treat seizures/convulsions,
migraines, and bipolar disorder
Mechanism of action – thought to increase GABA
levels in the brain
Pregnant women taking Valproic acid have an
increased risk of having children with spina bifida
23. a-Primary clinical pictures
1-Abnormal nerve conduction, resulting in:
Somatosensory losses
Motor paralysis, including loss of bowel and bladder
control (all are below level of lesion)
Changes in muscle tone
24. Motor level
Lesion Level
Above L3
L4 and below
S1 and below
S3 and below
Spinal-related disability
Complete paraplegia and dermatomal para-
anesthesia, Bladder incontinence, Nonambulatory
Same as for above L3 except preservation of hip
flexors, hip adductors, knee extensors; Ambulatory
with aids, bracing orthopedic surgery
Same as for L4 and below except preservation of feet
dorsiflexors, and partial preservation of hip
extensors and knee flexors; Ambulatory with
minimal aids
Normal lower extremity motor function; Saddle
anesthesia; Variable bladder-rectal incontinence
26. 2-An enlarged head caused
by:
1- hydrocephalus
(“water on the brain”)
11-Arnolds Chairi
malformation 11
27. i. Hydrocephalus
VP Shunt done in 85-90% of MMC
(1/3 will require revision at some time)
Most commonly due to obstruction of
Cerebrospinal Fluid (CSF) Flow
28.
29.
30. Arnold-Chiari type II Malformation
Definition: Medulla, Pons, 4th
ventricle +/-
cerebellar vermis herniated into the cervical
spinal canal
Incidence: 80-90% of those with MMC
Symptomatic: ~20%
Should be managed and treated as an emergency
by (surgical decomperssion operation)
31. 3-Urinary and Bowel symptoms
Urinary symptoms:
In lumbosacral spina bifida cystica, few children attain
urinary incontinence
They require urodynamic testing, including
cystometrography, uroflowmetry, and EMG of the urinary
sphincter
Types of lesions
1. “Keepers”- UMN
2. “Leakers”- LMN
According to results and consulting urologist patient may
need intermittent catheterization, pharmacological
agents, implantation of an artificial urinary sphincter or a
combination.
33. Bowel symptoms
A. Constipation
B. Incontinence
Management techniques
-enemas -suppositories
-habit training - digital stimulation
-biofeedback -appendicostomy(ACE)
34. 4-Musculoskeletal deformities (scoliosis)
5-Joint and extremity deformities (joint contractures,
club foot, hip subluxations, diminished growth of non-weight
bearing limbs)
6-Abnormal or damaged nerve tissue which can lead to
loss of extroceptive and prorioceptive sensation .
37. Management of spina bifida
1-screening and dignosis
2-Treatement
3-followup and observation
4- prevention
38. 1-Screening and DiagnosisPrenatal Tests
AFP(alphafeotoprotien)
Ultrasound
Testing of Amniotic fluid(Amniocetesis)
Evaluation
Analysis of individual medical history
Physical examination
Evaluation of critical body systems
Imaging Studies
X-rays
Ultrasound
CT scan
MRI
Gait Analysis
40. Surgical treatment
Surgery
In some cases the spinal cord is exposed
to the environment or tethered
Surgery is performed in order to cover
the spinal cord with muscle and skin or to
untether the spinal cord
in utero surgery has also become a
viable option for some cases
41. Surgery (continue--
Surgery for spina bifida involves a variety of
neurosurgical, orthopedic, and urologic
procedures.
Surgical procedures include the following:
Closure of the defect over the spinal cord
Spinal deformity reconstruction
Lower-extremity deformity correction
43. Medical treatement
Antibiotics
- In some cases the spinal cord is
exposed to the environment
- Antibiotics are essential in preventing
infection of the CNS and urinary tract
system
44. Physical Therapy
General functional expectations have been
developed for patients in each lesion-level group
to help direct physical therapy goals within an
appropriate developmental context from
infancy through adulthood.
The therapy programs should be designed to
parallel the normal achievement of gross motor
milestones.
45. In managing the cases of newborns with
myelomeningocele, the physical therapist
establishes a baseline of muscle function.
As the child develops, the physical therapist
monitors joint alignment, muscle imbalances,
contractures, posture, and signs of progressive
neurologic dy sfunction.
The physical therapist also provides caregivers
with instruction in handling and positioning
techniques and recommends orthotic
positioning devices to prevent soft tissue
contractures.
46. Provide the infant with sitting opportunities to
facilitate the development of head and trunk
control.
Near the end of the first year of life, provide the
child with an effective means of independent
mobility in conjunction with therapeutic
exercises that promote trunk control and
balance.
For patients who are not likely to become
ambulatory, place emphasis on developing
proficiency in wheelchair skills
47. .
For patients who are predicted to ambulate,
pregait training should begin with use of a
parapodium or swivel walker.
Exercise or household-distance ambulation
may be pursued with use of traditional long
leg braces (eg, hip-knee-ankle-foot orthosis,
knee-ankle-foot orthosis) or the reciprocating
gait orthosis [RGO]).
49. Example of a Parapodium
Commonly used for
children with high lesions
(T12-L3)
Offers support to the
hips, knees, and ankles.
(See Tecklin for additional
descriptions and
illustrations of orthoses
used for various lesion
levels.)
50. Occupational Therapy
Children with spina bifida often have
impairment in fine motor skills and conducting
activities of daily living (ADL).
Initiate training early to compensate for these
deficits and progress along the developmental
sequence as closely as possible.
51. Upper-extremity stabilization and dexterous
hand use require adequate postural control of
the head and trunk.
In the first year of life, encourage development
of these postural mechanisms or substitute
passive support, if necessary, to promote eye-
hand coordination and manipulatory skills.
When adequate fine motor skills have been
achieved, the occupational therapist provides
instructions for use of adaptive equipment and
alternative methods for self-care and other ADL
for preschool- and school-aged children.
52. Recreational Therapy
Children with myelomeningocele often
experience restricted play and recreational
opportunities because of limited mobility and
physical limitations.
This inactivity decreases the potential for normal
development in all spheres and can exert a
negative impact on self-esteem.
53. For the infant and toddler with
myelomeningocele, recreational therapy
enhances opportunities for environmental
exploration and interaction with other children.
For the school-aged child, recreational therapy
provides opportunities for participation in
adapted sports and exercise programs, which can
result in long-term interest in personal fitness
and health.
54. Recreational and physical fitness goals include
socialization, weight control, and improved
fitness (eg, flexibility, strength, aerobic capacity,
cardiovascular fitness, coordination).
Recreational therapy is helpful for promoting
independence with adult living skills and often is
used to assist the patient with shopping for and
purchasing personal items, use of public
transportation, and development of appropriate
leisure activities.
55. Follow up and careful Observation
Children with myelomeningocele often have
hydrocephalus (blockage of CSF)
Children may present with paralysis,
blindness, MR, inability to speak, convulsions
Any changes in mental status or behavior
should be quickly brought to the attention of
the child’s physician(s)
56. Folate intake
Recommended that women of child-bearing
age take 400 micrograms of folate per day
Pregnant women should take 600 micrograms
of folate per day
Women with a previous child with spina
bifida should take 4000 micrograms of folate
per day
Folate can decrease the risk of spina bifida by
up to 75%
How can spina bifida be prevented?
57. Sources of folate
Vegetables and grains
Many foods are now
enriched with folate
Most multi-vitamins contain
folate
Some vitamins are
specifically formulated for
women
58. Social impact
Prognosis and any deficits are
dependent upon level of involvement
Estimates from 5-40 % of the world’s population
may have spina bifida occulta
Meningocele is not very common and often has
minimal impact once the cyst is removed
Myelomeningocele has the largest impact on
patients and their families
59. Social impact(continue….
Changes with time
Before antibiotics most children with
myelomeningocele died because of infections in
the CNS; those that survived were unlikely to
ever walk
During the 1990s, the discovery of the role of
folate in neural tube closure drastically
decreased the number of cases of
myelomeningocele
In the late 1990s, in utero surgery was
attempted to close neural tube defects
60. Social impact (continue….
Children born with spina bifida today
require some special treatment :
Multiple surgeries starting as early as 48 hours after birth
Physical therapy
Bowel and/or bladder surgery – helps prevent infection
and social stigmatism
Latex allergies are often present
In many cases, special centers are better
equipped to treat children with spina bifida
and have a variety of specialists on staff
61. Most children that are treated early will have normal
IQ and be able to attend public schools
Mobility is the biggest concern for many patients with
spina bifida – lack of mobility can lead to obesity and
scoliosis
With proper treatment, individuals will live well into
adulthood
Social impact (continue…
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