All about Spina Bifida:
Definition
7 Types
Causes
Health Status
Incidence and epidemiology
Prevention
Social impact
Management
Detection
Diagnosis
Treatment
Antibiotics
Careful observation
Physical therapy
2. Spina bifida is a condition that affects the spine and is
usually apparent at birth. It is a type of neural tube defect
(NTD).
Spina bifida can happen anywhere along the spine if the
neural tube does not close all the way (usually lower
back), so the backbone that protects the spinal cord
doesn’t form and close as it should. This often results in
damage to the spinal cord and nerves.
Beneath this defect the meninges and spinal cord may or
may not be involved to varying degrees. The condition is a
result of failure of the mesenchyme, which grows in
between the neural tube and the surface ectoderm, to form
vertebral arches in the affected region.
In spina bifida, the spines and arches of one or more
adjacent vertebra failed to develop.
The condition occurs most frequently in the lower thoracic,
lumbar and sacral regions.
3. The neural tube develops into the
spinal cord and brain very early in
pregnancy.
It forms and closes the spinal cord
and nerves within protective
vertebrae (bones).
When there is an NTD present, the
spinal column does not close
completely as it is developing.
This occurs at around 23 days
gestation.
4. Types of Spina bifida are as follows:
1. Spina bifida oculta
2. Meningocele
3. Meningomyelocele
4. Myelocele
5. Syringomyelocele
6. Lipomeningocele
7. Spina bifida ventralis
Myelo” is the spinal cord
“meninges” is the protective covering
of the spinal cord
“cele” identifies swelling, or a sac
“lipo” means fat
“occult” means hidden
“Syrinx” means cyst
“Ventralis” means anterior
“bifida” means split
5. Most common and Most mild form of Spina bifida.
Occulta in Latin means Hidden, because a small
layer of skin covers the opening of your spinal
vertebrae.
when there is an opening in the spine, but nothing
comes out. It is skin covered and may not be
diagnosed at birth.
Spinal cord and tissue don’t protrude, so no damage
and no severe symptoms.
The spines and arches of one or more vertebra,
usually in the lumbar region, are absent, and the
vertebral canal remains open posteriorly. The
spinal cord and nerve roots usually are normal.
The defect is covered by the post vertebral muscles
and cannot be seen from the surface. A small tuft of
hair, dimple, birthmark or a fatty tumor may be
present over the defect.
6. Most cases are symptomless and are diagnosed by chance when the vertebral
column is X-rayed.
Spina bifida might cause physical and intellectual disabilities that range from
mild to severe. The severity depends on:
The size and location of the opening in the spine.
Whether part of the spinal cord and nerves are affected.
7. Meningocele is also called Meningeal
cyst.
When the meninges, but not the spinal
cord, come out of the opening in the
spine.
Hence, there is no damage to spinal
cord so no severe symptoms are
present.
The projecting meninges form a cystic
swelling beneath the skin and contain
cerebrospinal fluid, which
communicates with the subarachnoid
space.
The spinal cord and nerves usually are
normal.
8. Meningomyelocele is also called
Mylomeningocele.
When the spinal cord and protective
covering (meninges) both come out of
the opening in the spine.
The spinal cord or nerve roots are
adherent to the inner wall of the sac.
Generally, skin is intact. But in
severe form, the skin is absent. So,
spinal cord is exposed, and Hence
named “Open Spina Bifida” .
9. In Open Spina Bifida, spinal cord is prone to damage
and infections, resulting is:
Symptoms:
1. Loss of sensation
2. Paralysis
3. Bladder and Bowel movement problem
4. Seizure
5. Leg and foot deformities
Meningomyelocele is associated with ARNOLD
CHIARI II MALFORMATION.
ARNOLD CHIARI II MALFORMATION:
Cerebellum and brainstem tissue slips down into the
foramen Magnum
Leads to symptoms of cerebellum and Hydrocephalus
10. The neural tube fails to close in the
region of the defect. An oval row area
is found on the surface; this
represents the neural groove whose
lips are fused.
A rare disorder that presents as a
flat neural placode (at the level of
the skin of the back) that is exposed
to the environment.
The lack of expansion of the
subarachnoid space distinguishes
this lesion from myelomeningocele.
The central canal discharges clear
cerebrospinal fluid onto the surface.
11. This condition is rare.
A Meningomyelocele is present, and
in addition the central canal of the
spinal cord at the level of the Bony
defect is grossly dilated.
12. when fat tissue is caught in the
meninges and comes out of the
opening in the spine.
It is covered in skin and does not
require surgery at birth.
13. Much less common than other forms
of spina bifida
Meningeal sac protrudes 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
14. Health status
Incidence and epidimology
Causes
Management:
1. Detection (Diagnosis)
2. Surgery (Treatment)
3. Antibiotics
4. Careful observation
5. Physical therapy
Prevention
Social impact
15. Many afflicted infants are born dead. If the child is born alive, dead from infection
of the spinal cord may occur within a few days.
As a result of advances in medical and surgical care many infants with severe forms
of spina bifida now survive.
Unfortunately these children are likely to have a long life disabilities and
psychosocial problems.
The neurologic deficits alone may result in deformation of the limbs and spine, and
bladder, bowel and sexual dysfunction.
16. 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
17. Cause is unknown
Risk factor:
1. Folate deficiency (vit B9)(Developmental abnormalities occur at 4th week –
decrease intake of folic acid rich diet)
2. Obesity
3. Environmental and genetic factors
4. Poorly controlled diabetes
5. Medication that interferes with folate synthesis (e.g. anti.seizure medication).
19. Often done prenatally for detection of increased Alpha FetoProtein AFP in
mother’s serum.
Can be increased in other conditions
Other blood tests:
1. Human Chorionic gonadotropin HCG
2. Inhibin A
3. Estriol
Ultrasound
Amniocentesis ( Samples from amniotic sac )
20. Most cases of spina bifida occulta require no treatment. A
meningocele should be removed surgically within a few days
of birth. Infants with meningomyelocele should also be treated
surgically.
SURGERY:
During surgery, the surgeon will put the spinal cord and any
exposed tissues or nerves back into the correct place. The gap in
the spine is then closed and the hole sealed with muscle and
skin. Although this will repair the defect, unfortunately it cannot
reverse any nerve damage..
Prenatal surgery:
Close myelomeningocele
Can be dangerous
Postnatal surgery:
Within a few Days of birth
To minimize risk of meningitis
22. Not all people born
with spina bifida have
the same needs, so
treatment will be
different for each
person. Some people
have problems that are
more serious than
others.
Treatment for spina
bifida depends on the
extent of spinal cord
involvement
23. In some cases the spinal cord is exposed to the environment
Antibiotics are essential in preventing infection of the CNS
24. 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)
25. Spinal cord damage can cause muscle weakening
and wasting
Speech therapy may also be useful for some
individuals
The therapy programs should be designed to
parallel the normal achievement of gross motor
milestones.
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 dysfunction.
The physical therapist also provides caregivers
with instruction in handling and positioning
techniques and recommends orthotic positioning
devices to prevent soft tissue contractures.
26. 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
parapodium or swivel walker- for pregait training
Traditional long leg braces (eg, hip-knee-ankle-foot orthosis, knee-ankle-foot
orthosis) or the reciprocating gait orthosis [RGO]).
Occupational Therapy for ADLs
Recreational Therapy- to decrease the negative impact on normal development
and self esteem
27. Education:
Neural tube begins to close at day 22 after conception
Neural tube is usually fully closed by day 28 after conception
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%
28. Prognosis and any deficits are dependent upon level of involvement
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
Editor's Notes
If the neural tubes doesn’t get fused, there is a defect or a absence of vertebral arches because of failure of the mesoderm to organize.
Starting from 22nd day,Neural tubes completely closes around 28 days. So in practical terms the NTDs occur before the women know they’re expecting.
Spinal cord and meninges project through the vertebrae
Placode (plural placodes) A platelike thickening of the epithelial layer of an embryo from which an organ, especially a sense organ, develops
Main source of folic acid is cereal grains.
Valproic acid – treat siezures / convulsion, bipolar disorder, migraine.
Spina bifida happens in the first few weeks of pregnancy, often before a woman knows she’s pregnant. Although folic acid is not a guarantee that a woman will have a healthy pregnancy, taking folic acid can help reduce a woman’s risk of having a pregnancy affected by spina bifida. Because half of all pregnancies in the United States are unplanned, it is important that all women who can become pregnant take 400 mcg of folic acid daily.
The AFP is released from the fetus through amniotic fluid in the mother’s serum.