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SPINA BIFIDA
DR KRISHNA NS
CON OCCUPATIONAL THERAPIST
COIMBATORE, TAMIL NADU
1
Content
 Introduction
 Neural Tube defect
 About Spina Bifida
 Incidence rate
 Etiology
 Diagnosis
 Types of Spina bifida
 Signs and symptoms
 Treatment
 Prognosis
 Conclusion
DR KRISHNA NS
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Neural tube defects
 Neural tube defects refers to a group of malformations of
the spinal cord, brain, and vertebrae.
 The resulting disorder vary in severity according to their
location, extent of bony opening and exposure of spinal
cord or brain
DR KRISHNA NS
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Cont.
 The 3 major NTDs are
 Spina Bifida
 Encephalocele
 Anencephaly
The most common of these is Spina Bifida.
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Encephalocele
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What is Spina Bifida?
 Spina bifida comes from 2 words
“Spina” meaning spine
“Bifida” meaning split or divided
Also called as Myelodysplasia
 It is a defect that occurs in the development of the infant’s spine.
 Here the backbone and spinal canal do not close before birth
 It occurs about 24-26 days after the mother becomes pregnant
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Video
DR KRISHNA NS
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What is there? In spina bifida
 It is usually diagnosed at birth by the presence of an external sac on
the infant’s back.
 The bones of the infant’s spine do not close over the spinal cord
(central tube of nerves).
 A soft, unprotected area will be present on the infant’s back.
DR KRISHNA NS
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Cont.
 This may be covered by skin, or more commonly be an open wound which may
bulge through the skin as dark bag.
 This bag is covered by a very thin layer of skin
which may leak liquid from the spinal cord and brain.
DR KRISHNA NS
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Incidence
 Spina Bifida is one type of neural tube defect causing neuromuscular
dysfunction
 The occurrence of spina bifida approaches 1 in every 1000 live births,
making it the 2nd most common birth defect after Down syndrome
 In India the prevalence is 3/1000 live births, the highest reported from the
northern states, namely Punjab, Haryana, Rajasthan and Bihar
DR KRISHNA NS
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Etiology
 Genetic predispositions due to numerous environmental factors
 Low levels of maternal folic acid during the first trimester of pregnancy.
 Obesity
 Improper dietary supplements
 For families in which spina bifida is already present, there is a 2% to 5% greater
chance than in the general population of having a second child born with the
defect.
DR KRISHNA NS
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How do we diagnose?
 Prenatal testing
 AFP (Alpha Feto Protein) is measured during the 16th – 18th week of pregnancy.
 It is typically found in fetal Spinal Fluid.
 In Spina Bifida this will leak from the open spine.
 Ultra sound Imaging
• The frontal bones of the fetal skull lose their normal convex shape and appear flattened when spina
Bifida is present, similar to the shape of a lemon. This is called as “Lemon Sign”
• Present before 24 weeks of gestation.
DR KRISHNA NS
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Cont.
 Aminocentesis – analysis AFP and an enzyme Specific for the
condition Acetylcholinesterase (ACH)
 ACH is found in fetal cerebrospinal fluid and its presence in
amniotic fluid suggests leaking from the open spine.
DR KRISHNA NS
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Types of Spina Bifida
 Spina Bifida can present in a number of different ways depending on the
extent the neural tube has failed to close early in development
1.Spina Bifida Occulta
2.Spina Bifida Cystica
2.a Meningocele
2.b Myelomeningocele
DR KRISHNA NS
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Types
DR KRISHNA NS
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DR KRISHNA NS
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DR KRISHNA NS
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SPINA BIFIDA OCCULTA MENINGOCELE MYELOMENINGOCELE
1. only bones over spinal
cord affected
1. lump contains only spinal fluid
not spinal cord
1. Lump contains spinal fluid
and spinal cord
1. Hairy patch or fatty lump
present
2. It can affect any level of
spine but usually in lower
part of back
---------
1. Lump may be open or
covered by skin
• Usually no disability 1. Usually no disability 1. Permanent disability which
varies from mild to severe
DR KRISHNA NS
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Signs and Symptoms
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Symptoms of spina bifida
A newborn may have a sac sticking out of the mid to lower back.
 Loss of bladder or bowel control
 Partial or complete lack of sensation
 Partial or complete paralysis of the legs
 Weakness of the hips, legs, or feet of a newborn
 Pressure sores and skin irritations
 Abnormal eye movement
DR KRISHNA NS
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Cont.
 Other symptoms may include:
 Swelling of the leg, heat or redness in the leg
 Development of pressure sores in butt, heels and knees.
 Build up of fluid inside the skull (hydrocephalus)
 Common Ortho problems
DR KRISHNA NS
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Complications
 Abnormal sensation or paralysis
 Chiari II malformation
 Hydrocephalus
 Meningitis
 Tethered cord syndrome
 Learning Disabilities
 Others follow
DR KRISHNA NS
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Common Signs of SBO
(in severe forms)
 loss of strength
 feeling and co-ordination in the movements of the legs
 mostly asymmetrical abnormalities of the feet such as, clubfeet and clawing of
the toes
 poor development of the muscles of the lower legs
 altered bladder and bowel function with retention of urine and constipation
(all these are caused due to abnormal development of spinal nerves)
DR KRISHNA NS
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Common Ortho problems
 Club foot-Children with spina bifida may be born with a foot turned inward
 Ankle equinus- Ankle deformity
 Ankle valgus
 Hip Dislocation
 Spinal Curvature problems
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What can happen as a result of spina bifida?
Difficulty in movement
 The ability to move is not the same for all children with spina bifida.
 Depending on where the spina bifida is on the child's back there will be differing amounts of
movement loss.
 The loss of movement present when the baby is born is permanent and usually does not
change.
Children may have either
 complete loss of muscle function and sensation below the level of their lesion or
 partial loss of muscle function and sensation below the level of their lesion.
DR KRISHNA NS
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Bones of the
backbone (spine)
Level of lesion Probable amount of
disability
DR KRISHNA NS
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Upper lumbar
level level
Sacral thoracic
level
Low lumbar and sacral
level
Mid lumbar level
Child will usually
not be able to
stand up by self
Will require
braces and
crutches to walk
Child will not be
able to stand up
by self
will require high
level bracing and
splinting to stand
child will usually stand at a
normal age
walking by himself may be
delayed
child may have some ability
to walk by himself with aids
he will require bracing
Will need a
wheelchair
Common
deformities are
hip and knee
dislocation
Will need W/C for
mobility
Common
deformities are
kyphosis, scoliosis,
hip dislocation,
knee contractures,
club feet
child may need braces for
ankles but not use crutches
to walk
common deformities are
club feet, foot and toe
deformities
he may not need a
wheelchair
common deformities are
hip flexion contractures,
hip dislocation, club feet,
calcaneous feet
DR KRISHNA NS
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Problem with bones
 SPINAL CURVATURES
 The spinal bones where the spina bifida is located will always remain split.
 This can affect the spine as the child grows and cause different types of
curvature.
 The three main types of curvatures are
1. Kyphosis
2. Scoliosis
3. Lordosis
DR KRISHNA NS
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Kyphosis Scoliosis Lordosis
The spine is curved out at
the back and produces a
hump
The spine bends sideways
and rotates causing a rib
hump on one side
The lumbar spine bows
inwards
This can be seen more easily
from behind when the child
bends forward.
DR KRISHNA NS
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LEG DEFORMITIES
 Although the cause of deformity in the individual circumstance is unproven and there are
unknown influences like:
 Muscle imbalance secondary to the neurological abnormality
 Habitually assumed intrauterine posture
 Habitually assumed posture after birth
 Co-existent congenital malformations
 Arthrogryphosis
 Tethering of neural tissue
DR KRISHNA NS
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Mixed deformity – right
clubfoot
Left knee contracture
Over straight knee Calcaneus deformity of
both feet
equinus deformity
DR KRISHNA NS
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Lack of feeling in skin
 Because of the nerve damage that occurs, children with spina bifida do not have normal
senses of feeling and pain in their body, legs and feet.
 The amount of lack of feeling will depend on where the spina bifida is located over the
spine and the amount of nerve damage in the spina bifida.
 Pressure sores can also occur if the child is sitting or lying in one position for a long time.
 These sores can become deep ulcers and take many months to heal. If a sore does
develop, care must be taken to keep the sore clean and prevent it from getting larger
DR KRISHNA NS
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POOR BLADDER AND BOWEL CONTROL
 In spina bifida, lack of control in passing urine and stools (incontinence) is due to the
damaged nerves which control the muscles of the bowel and bladder.
 These nerves come from very low in the spinal cord, so bladder and bowel problems are
common in children with spina bifida.
 There are two main types of problem
# bladder constantly voiding urine
# bladder that don’t empty
DR KRISHNA NS
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Due to hydrocephalus
 Many children with hydrocephalus have difficulty learning. Hydrocephalus does not
prevent the child from learning, but makes it harder for the child to learn.
 These difficulties can interfere with all kinds of learning including school work, activities
at home and with self-care activities.
 If hydrocephalus is not treated, brain damage will occur resulting in a greater loss of
intellectual function,
DR KRISHNA NS
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DR KRISHNA NS
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Prevention
 Folic acid supplements may help reduce the risk of neural tube defects such as
myelomeningocele. It is recommended that any woman considering becoming
pregnant take 0.4 mg of folic acid a day. Pregnant women need 1 mg per day.
 It is important to remember that folic acid deficiencies must be corrected before
becoming pregnant as the defects develop very early.
 Prospective mothers may be screened to determine the amount of folic acid in
their blood.
DR KRISHNA NS
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When to seek medical attention?
 Before Pregnancy
 During Pregnancy
 Right after birth
DR KRISHNA NS
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Treatment
 No permanent cure till now
 Regular check ups with physician
 Surgery (24 hours after birth)
 Medication
 Genetic counseling may be recommended.
 Occupational therapy and physiotherapy
DR KRISHNA NS
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Treatment cont.
Most children will require lifelong treatment for problems that result from
damage to the spinal cord and spinal nerves.
This includes:
 Catheters
 Braces
 High fiber diet
 Antibiotics may be used to treat or prevent infections such as meningitis or
urinary tract infections.
DR KRISHNA NS
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Braces – OT’st Proud in Splinting
 Foot orthosis (FO): The brace keeps the foot from turning downward, keeps the
arch of the foot from flattening, and prevents the ankles from rolling inward.
 Supramalleolar orthosis (SMO): The brace extends above the ankle bones to
provide increased support to the ankle while allowing the ankle to move.
 Ankle-foot orthosis (AFO): This brace gives maximum support to the foot and
ankle and extends up the calf to just below the knee. The brace can be solid at
the ankle to stop ankle motion. Some are hinged at the ankle to allow
some motion.
DR KRISHNA NS
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 Floor reaction or solteil braces: These are an ankle-foot brace that help the
child walk in a more normal position.
 Knee-ankle-foot orthosis (KAFO): This brace supports the knee. It has thigh cuffs
and jointed metal supports that extend from the thigh cuff to the ankle and
foot. The knee joints can be locked or unlocked to allow sitting in the brace.
 Hip-knee-ankle-foot orthosis (HKAFO): This brace come with a pelvic band to
support the hips, or with both a pelvic band and a chest strap if more support is
needed. Otherwise, it is identical to the KAFO. It is also called a “long-leg brace.”
DR KRISHNA NS
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DR KRISHNA NS
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Surgery
• Usually performed with in 24 hours after birth.
• They remove the infected area and replace it with muscle tissue and skin.
• Helps protect against hydrocephalus.
DR KRISHNA NS
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Complications
 Difficult delivery with problems resulting from a traumatic birth, including cerebral
palsy and decreased oxygen to the brain
 Frequent urinary tract infections
 Hydrocephalus
 Loss of bowel or bladder control
 Meningitis
 Permanent weakness or paralysis of legs
DR KRISHNA NS
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Prognosis
 Myelomeningocele can usually be surgically corrected. With treatment, length of life is not
severely affected. Neurological damage is often irreversible.
 Many individuals with spina bifida primarily use a wheelchair.
Life long issues include:
 more loss of function as well as orthopedic problems such as scoliosis
 foot or ankle deformities
 dislocated hips
 joint tightness or contractures
 Infections within the shunt system
DR KRISHNA NS
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quiz
1. What is spina bifida?
2. What is the most common type of SB?
3. Name 2 signs or symptoms of SB?
4. What are the ways of diagnosing SB?
5. Name 2 ways SB is treated?
DR KRISHNA NS
46

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Spina bifida overview Clinical Neuro by Dr Krishna NS (M.O.Th-Neuro)

  • 1. SPINA BIFIDA DR KRISHNA NS CON OCCUPATIONAL THERAPIST COIMBATORE, TAMIL NADU 1
  • 2. Content  Introduction  Neural Tube defect  About Spina Bifida  Incidence rate  Etiology  Diagnosis  Types of Spina bifida  Signs and symptoms  Treatment  Prognosis  Conclusion DR KRISHNA NS 2
  • 3. Neural tube defects  Neural tube defects refers to a group of malformations of the spinal cord, brain, and vertebrae.  The resulting disorder vary in severity according to their location, extent of bony opening and exposure of spinal cord or brain DR KRISHNA NS 3
  • 4. Cont.  The 3 major NTDs are  Spina Bifida  Encephalocele  Anencephaly The most common of these is Spina Bifida. DR KRISHNA NS 4
  • 6. What is Spina Bifida?  Spina bifida comes from 2 words “Spina” meaning spine “Bifida” meaning split or divided Also called as Myelodysplasia  It is a defect that occurs in the development of the infant’s spine.  Here the backbone and spinal canal do not close before birth  It occurs about 24-26 days after the mother becomes pregnant DR KRISHNA NS 6
  • 8. What is there? In spina bifida  It is usually diagnosed at birth by the presence of an external sac on the infant’s back.  The bones of the infant’s spine do not close over the spinal cord (central tube of nerves).  A soft, unprotected area will be present on the infant’s back. DR KRISHNA NS 8
  • 9. Cont.  This may be covered by skin, or more commonly be an open wound which may bulge through the skin as dark bag.  This bag is covered by a very thin layer of skin which may leak liquid from the spinal cord and brain. DR KRISHNA NS 9
  • 10. Incidence  Spina Bifida is one type of neural tube defect causing neuromuscular dysfunction  The occurrence of spina bifida approaches 1 in every 1000 live births, making it the 2nd most common birth defect after Down syndrome  In India the prevalence is 3/1000 live births, the highest reported from the northern states, namely Punjab, Haryana, Rajasthan and Bihar DR KRISHNA NS 10
  • 11. Etiology  Genetic predispositions due to numerous environmental factors  Low levels of maternal folic acid during the first trimester of pregnancy.  Obesity  Improper dietary supplements  For families in which spina bifida is already present, there is a 2% to 5% greater chance than in the general population of having a second child born with the defect. DR KRISHNA NS 11
  • 12. How do we diagnose?  Prenatal testing  AFP (Alpha Feto Protein) is measured during the 16th – 18th week of pregnancy.  It is typically found in fetal Spinal Fluid.  In Spina Bifida this will leak from the open spine.  Ultra sound Imaging • The frontal bones of the fetal skull lose their normal convex shape and appear flattened when spina Bifida is present, similar to the shape of a lemon. This is called as “Lemon Sign” • Present before 24 weeks of gestation. DR KRISHNA NS 12
  • 13. Cont.  Aminocentesis – analysis AFP and an enzyme Specific for the condition Acetylcholinesterase (ACH)  ACH is found in fetal cerebrospinal fluid and its presence in amniotic fluid suggests leaking from the open spine. DR KRISHNA NS 13
  • 14. Types of Spina Bifida  Spina Bifida can present in a number of different ways depending on the extent the neural tube has failed to close early in development 1.Spina Bifida Occulta 2.Spina Bifida Cystica 2.a Meningocele 2.b Myelomeningocele DR KRISHNA NS 14
  • 18. SPINA BIFIDA OCCULTA MENINGOCELE MYELOMENINGOCELE 1. only bones over spinal cord affected 1. lump contains only spinal fluid not spinal cord 1. Lump contains spinal fluid and spinal cord 1. Hairy patch or fatty lump present 2. It can affect any level of spine but usually in lower part of back --------- 1. Lump may be open or covered by skin • Usually no disability 1. Usually no disability 1. Permanent disability which varies from mild to severe DR KRISHNA NS 18
  • 19. Signs and Symptoms DR KRISHNA NS 19
  • 20. Symptoms of spina bifida A newborn may have a sac sticking out of the mid to lower back.  Loss of bladder or bowel control  Partial or complete lack of sensation  Partial or complete paralysis of the legs  Weakness of the hips, legs, or feet of a newborn  Pressure sores and skin irritations  Abnormal eye movement DR KRISHNA NS 20
  • 21. Cont.  Other symptoms may include:  Swelling of the leg, heat or redness in the leg  Development of pressure sores in butt, heels and knees.  Build up of fluid inside the skull (hydrocephalus)  Common Ortho problems DR KRISHNA NS 21
  • 22. Complications  Abnormal sensation or paralysis  Chiari II malformation  Hydrocephalus  Meningitis  Tethered cord syndrome  Learning Disabilities  Others follow DR KRISHNA NS 22
  • 23. Common Signs of SBO (in severe forms)  loss of strength  feeling and co-ordination in the movements of the legs  mostly asymmetrical abnormalities of the feet such as, clubfeet and clawing of the toes  poor development of the muscles of the lower legs  altered bladder and bowel function with retention of urine and constipation (all these are caused due to abnormal development of spinal nerves) DR KRISHNA NS 23
  • 24. Common Ortho problems  Club foot-Children with spina bifida may be born with a foot turned inward  Ankle equinus- Ankle deformity  Ankle valgus  Hip Dislocation  Spinal Curvature problems DR KRISHNA NS 24
  • 25. What can happen as a result of spina bifida? Difficulty in movement  The ability to move is not the same for all children with spina bifida.  Depending on where the spina bifida is on the child's back there will be differing amounts of movement loss.  The loss of movement present when the baby is born is permanent and usually does not change. Children may have either  complete loss of muscle function and sensation below the level of their lesion or  partial loss of muscle function and sensation below the level of their lesion. DR KRISHNA NS 25
  • 26. Bones of the backbone (spine) Level of lesion Probable amount of disability DR KRISHNA NS 26
  • 27. Upper lumbar level level Sacral thoracic level Low lumbar and sacral level Mid lumbar level Child will usually not be able to stand up by self Will require braces and crutches to walk Child will not be able to stand up by self will require high level bracing and splinting to stand child will usually stand at a normal age walking by himself may be delayed child may have some ability to walk by himself with aids he will require bracing Will need a wheelchair Common deformities are hip and knee dislocation Will need W/C for mobility Common deformities are kyphosis, scoliosis, hip dislocation, knee contractures, club feet child may need braces for ankles but not use crutches to walk common deformities are club feet, foot and toe deformities he may not need a wheelchair common deformities are hip flexion contractures, hip dislocation, club feet, calcaneous feet DR KRISHNA NS 27
  • 28. Problem with bones  SPINAL CURVATURES  The spinal bones where the spina bifida is located will always remain split.  This can affect the spine as the child grows and cause different types of curvature.  The three main types of curvatures are 1. Kyphosis 2. Scoliosis 3. Lordosis DR KRISHNA NS 28
  • 29. Kyphosis Scoliosis Lordosis The spine is curved out at the back and produces a hump The spine bends sideways and rotates causing a rib hump on one side The lumbar spine bows inwards This can be seen more easily from behind when the child bends forward. DR KRISHNA NS 29
  • 30. LEG DEFORMITIES  Although the cause of deformity in the individual circumstance is unproven and there are unknown influences like:  Muscle imbalance secondary to the neurological abnormality  Habitually assumed intrauterine posture  Habitually assumed posture after birth  Co-existent congenital malformations  Arthrogryphosis  Tethering of neural tissue DR KRISHNA NS 30
  • 31. Mixed deformity – right clubfoot Left knee contracture Over straight knee Calcaneus deformity of both feet equinus deformity DR KRISHNA NS 31
  • 32. Lack of feeling in skin  Because of the nerve damage that occurs, children with spina bifida do not have normal senses of feeling and pain in their body, legs and feet.  The amount of lack of feeling will depend on where the spina bifida is located over the spine and the amount of nerve damage in the spina bifida.  Pressure sores can also occur if the child is sitting or lying in one position for a long time.  These sores can become deep ulcers and take many months to heal. If a sore does develop, care must be taken to keep the sore clean and prevent it from getting larger DR KRISHNA NS 32
  • 33. POOR BLADDER AND BOWEL CONTROL  In spina bifida, lack of control in passing urine and stools (incontinence) is due to the damaged nerves which control the muscles of the bowel and bladder.  These nerves come from very low in the spinal cord, so bladder and bowel problems are common in children with spina bifida.  There are two main types of problem # bladder constantly voiding urine # bladder that don’t empty DR KRISHNA NS 33
  • 34. Due to hydrocephalus  Many children with hydrocephalus have difficulty learning. Hydrocephalus does not prevent the child from learning, but makes it harder for the child to learn.  These difficulties can interfere with all kinds of learning including school work, activities at home and with self-care activities.  If hydrocephalus is not treated, brain damage will occur resulting in a greater loss of intellectual function, DR KRISHNA NS 34
  • 36. Prevention  Folic acid supplements may help reduce the risk of neural tube defects such as myelomeningocele. It is recommended that any woman considering becoming pregnant take 0.4 mg of folic acid a day. Pregnant women need 1 mg per day.  It is important to remember that folic acid deficiencies must be corrected before becoming pregnant as the defects develop very early.  Prospective mothers may be screened to determine the amount of folic acid in their blood. DR KRISHNA NS 36
  • 37. When to seek medical attention?  Before Pregnancy  During Pregnancy  Right after birth DR KRISHNA NS 37
  • 38. Treatment  No permanent cure till now  Regular check ups with physician  Surgery (24 hours after birth)  Medication  Genetic counseling may be recommended.  Occupational therapy and physiotherapy DR KRISHNA NS 38
  • 39. Treatment cont. Most children will require lifelong treatment for problems that result from damage to the spinal cord and spinal nerves. This includes:  Catheters  Braces  High fiber diet  Antibiotics may be used to treat or prevent infections such as meningitis or urinary tract infections. DR KRISHNA NS 39
  • 40. Braces – OT’st Proud in Splinting  Foot orthosis (FO): The brace keeps the foot from turning downward, keeps the arch of the foot from flattening, and prevents the ankles from rolling inward.  Supramalleolar orthosis (SMO): The brace extends above the ankle bones to provide increased support to the ankle while allowing the ankle to move.  Ankle-foot orthosis (AFO): This brace gives maximum support to the foot and ankle and extends up the calf to just below the knee. The brace can be solid at the ankle to stop ankle motion. Some are hinged at the ankle to allow some motion. DR KRISHNA NS 40
  • 41.  Floor reaction or solteil braces: These are an ankle-foot brace that help the child walk in a more normal position.  Knee-ankle-foot orthosis (KAFO): This brace supports the knee. It has thigh cuffs and jointed metal supports that extend from the thigh cuff to the ankle and foot. The knee joints can be locked or unlocked to allow sitting in the brace.  Hip-knee-ankle-foot orthosis (HKAFO): This brace come with a pelvic band to support the hips, or with both a pelvic band and a chest strap if more support is needed. Otherwise, it is identical to the KAFO. It is also called a “long-leg brace.” DR KRISHNA NS 41
  • 43. Surgery • Usually performed with in 24 hours after birth. • They remove the infected area and replace it with muscle tissue and skin. • Helps protect against hydrocephalus. DR KRISHNA NS 43
  • 44. Complications  Difficult delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain  Frequent urinary tract infections  Hydrocephalus  Loss of bowel or bladder control  Meningitis  Permanent weakness or paralysis of legs DR KRISHNA NS 44
  • 45. Prognosis  Myelomeningocele can usually be surgically corrected. With treatment, length of life is not severely affected. Neurological damage is often irreversible.  Many individuals with spina bifida primarily use a wheelchair. Life long issues include:  more loss of function as well as orthopedic problems such as scoliosis  foot or ankle deformities  dislocated hips  joint tightness or contractures  Infections within the shunt system DR KRISHNA NS 45
  • 46. quiz 1. What is spina bifida? 2. What is the most common type of SB? 3. Name 2 signs or symptoms of SB? 4. What are the ways of diagnosing SB? 5. Name 2 ways SB is treated? DR KRISHNA NS 46