DR/HN/1
Spina Bifida
Dr Hasan Nugud
Consultant
Paediatric Surgeon
Spina Bifida
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Dr/HN/2
Spina Bifida (SB), is a neural tube
defect (NTD), that occurs when the
vertebrae (arches) and /or spinal
cord of the fetus fail to fuse in the
mid line with or without protrusion
(herniation) and dysplasia of the
spinal cord ,its associated
membranes and distal nerves ,
resulting in varying degrees of CNS
damage .
Spina Bifida
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Dr/HN/3
Spina bifida (SB) itself means split
spine.
When the brain itself is not
completely developed the condition
is called anencephally.
When a portion of it “the spine”, is
abnormally formed , spina bifida
results,
The damage is permanent.
Spina Bifida
Calssification :
1-Spina bifida occulta,
2-Spina bifida cystica;
a-Meningocele,
b-Myelomeningocele,
3-Rare types :
-Amelia,
-Rachischisis
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Spina Bifida
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Spina bifida is actually one of a
number of conditions called NTD
that occur when the CNS of the
fetus fails to fold and fuse at some
point along it length.
This can occur anywhere from the
brain to the end of the spinal
cord.The bones (ver- tebral
arches) of the spinal column which
surround the developing spinal
cord do not close .
Spina Bifida
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Spina Bifida Occulta :
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The vertebrae are bifid without a
menigocele or myelomeningocele
below the intact skin, commonly
affects the 5th lumbar and first
sacral vertebrae
Spina Bifida
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Spina Bifida Occulta :
The term suggests that the lesion
is hidden below the intact skin, but
it is not always so, sometimes the
presence a “signature mark” as a
superficial clue may over lie the
anomaly such as; a patch of a dark
hair,pigmented naevus,
angiomatus area, lipoma, simple
dimple or sinus.
Spina Bifida
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Spina Bifida
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Spina Bifida
Amelia :
The spinal cord may be absent, split or
disorganized,
Amelia occurs with gross spina bifida
associated with anencephaly
 Rachischisis:
The most severe form. Incomplete
failure of fusion of the neural plates
Neural tube lies open. No sac is
present, falttend spinal cord red down
the back.
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Spina Bifida
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During the first 28 days of
pregnancy the brain and spinal
cord of an embryo form,
During this period (28 days), spina
bifida occurs, before most women
even know that they are pregnant,
Occurs in 1/750-1000 pregnancies.
Spina Bifida
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Etiology :
There is no single known cause of
SB. Research continues into the
effects of factors such as heredity ,
nutrition, environment, pollution,
and physical damage to the
embryo.
The risk of SB occuring in
subsequent children is certainly
increased.
Spina Bifida
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Alfa Feto Protein (AFP):
AFP is produced by all fetuses but
higher in cases of open CNS(NTD)
Pregnant woman’s blood can be
tested for AFP as early as the 16th
week of gestation,
Amniocentesis at 14-17th weeks of
gestation is more accurate ,(disadvantage of miscarriage).
Spina Bifida
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AFP : (cont…)
AFP testing can detect up to 85%
of these cases, but this test is not
specific for SB and can be
elevated in many other similar
abnormalities , therefore, further
testing is recommended.
Spina Bifida
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AFP : (cont..)
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Amniocentesis is offered to
mothers with increased risk of
having babies with a birth defect,
(previous spina bifida, or taking
drugs for siezures, or over 35
years of age or positive family
history) .
Spina Bifida
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USS Scan :
Uss at 18-20
weeks of
gestation will
show the
defect in the
spinal bones
and certain
changes in the
brain in 90%
on NTD cases.
Spina Bifida
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Spina Bifida Occulta :
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Neural abnormalities are uncommon
The bony diffect is of no clinical
significance,
Few patients may develop progressive
neurological signs during spurt growth
which occurs most often between 8 and
14 years of age.
Lipoma
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Spina Bifida
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Spina Bifida Occulta : (cont...)
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Progressive deformity of the feet or
changes in micturation are very
suggestive and myelography is indicated
when these occur,
In case of objective neurological signs or
myelographic evidence of cord or root
displacement ,patient should be
explored.
Local lesion removal for cosmetic reason
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Spina Bifida
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Spina Bifida Occulta : (cont..)
Dermal sinus ;
Should be completely excised
before infection supervenes to
remove the risk of meningitis when
the track happens to communicate
with or is in close proximity to the
subarachnoid space. If already infected, the sinus should be excised
after the antibiotic infection control
Spina Bifida
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Split Notochord Syndrome :
Occasionally , embryos develop
with partial duplication of
notochord, yolk sac herniates
between them,
Diastematomyelia is due to fusion
of the medial pedicles of a pair of
hemi-vertevrae to form a bony spur
which lies between the split halves
of spinal cord.
Spina Bifida
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Vesicointestinal fissure, or extrophy
of the cloaca is usually associated
with a myelomeningocele and is a
severe variation of the split
notochord syndrome.
Split notochord with yolk sac hernia,
Split notochord with yolk sac fistula,
Split notochord with gut fistula,
Split notochord with
diastematomyelia
Spina Bifida
Different
variants of
split
notochord
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Spina Bifida
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Spina Bifida Cystica :
Meningocele;
About 5-6% of SB cystica,
A failure of the vertebral arch to
form and a protrusion of the
meninges through the gap to form
a simple meningeal sac lined by
arachnoid membrane and dura
containing CSF and only
occasionally nerve tissue.
Spina Bifida
Spina Bifida Cystica
Meningocele
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Spina Bifida
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Menigocele : (cont..)
A cystic swelling with a cover which
may vary from a thin translucent
membrane to normal skin,
In the great majority the cord is
normally formed and there is
neurological defect, but each case
must be watched with care because
of associated spinal cord dysplasia.
Spina Bifida
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Meningocele : (cont..)
The cyst may be large to several
inches in diameter while the neck
is often narrow,
The cyst may be tense, specially if
the baby is crying or when
pressure is applied to the
fontanelle, otherwise soft and
fluctuant,
Spina Bifida
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Meningocele : (cont..)
The skin over the sac is generally
intact, but it is occasionally
ulcerated, which is, however , more
typical of myelomeningocele,
A lipoma round the base of the sac
may give the false impresion of a
wide base,
Radiology will show that the interpeduncular gap is not so extensive.
Spina Bifida
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Meningocele : (cont..)
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Hdrocephalus is rare,
Normal mental state,
Usually, no neurol. abnormalities.
If the skin is intact no urgency ,
The repair can be done at any
convinient time during infancy.
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Spina Bifida
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Meningocele : (cont..)
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If the sac is ulcerated it should be
repaired immediately after birth, before significant infection supervenes,
But if this opportunity is missed
epithelium should be allowed to
cover the sac, which is then excised
at a convenient time later in infancy.
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Spina Bifida
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SB Cystica :
Myelomeningocele;
94% of spina bifida cystica ,
The most serious variety,
Consists of a bifid spine with protrusion and dysplasia of the meninges and spinal cord and is always
acoompanied by neurological signs
Spina Bifda
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Myelomeningocele : (cont..)
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The spinal cord remains exposed
and the surface (neural plague),
looks like granulation tissue,
At the upper end the central canal
may be recognized as a fistula
discharging cerebro- spinal- fluid
(CSF).
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Spina Bifida
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Myelomeningocele : (cont..)
This tissue is surrounded by a thin
translucent membrane
representing the meninges
(arachnoid membrane with nervous
tissue visible on the surface known
as , neural plague), and
circumferentially an area of illformed skin is almost always
present.
Spina Bifida
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Myelomeningocele : (cont..)
The base of the lesion is broad and
the separated bony pedicles of the
spine can be felt on both sides,
The extent of the paralysis does
not necessarily relate accurately to
the level of the lesion because
dysplasia in the spinal cord may be
more extensive than the overt
lesion .
Spina Bifida
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Myelomeningocele : (cont..)
The commonest forms in the lumbar
region but can occur anywhere
along the neuroaxis including the
brain itself (encephalocele) ,
Even grosser forms may occur in
which the entire cord lies open, untubed on the surface.
These babies are usually stillborn
with other multiple congenital
anomalies.
Spina Bifida
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Myleomeningocele : (cont..)
Motor loss ;
There is a flaccid paralysis of the
lower motor neuron type .
The extent depending on the level
of the neurological lesion,
Upper neuron paralysis is rare and
present in few cases of
hydrocephalus or meningitis.
Spina Bifida
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Myelomeningocele: (cot..)
Motor Loss ;
The motor loss can be assessed
by observing the infant’s voluntary
(not reflex) movements and the
determination of the level is helpful
in assessing the probable extent of
the ultimate disability.
Spina Bifida
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Myleomeningocele : Motor Loss ;
Grouping according to lesion level,
(some overlap is inevitable),
Group I –Cervical & upper thoracic
Group II--Lower thoracic,
Group III-Upper Lumbar;
Group IV-Lower lumbar & up. sacral
Group V--Lower Sacral
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Myelomeningocele : Motor Loss ;
Group I : ( < 1% ),
Small group with extensive paralysis
of the lower extrimities,
Paralysis of variable proportion of the
trunck and upper extrimities,
Spastic paralysis of legs in children
with normal sp.cord below lision level
Spina Bifida
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Myelomeningocele : Motor Loss ;
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Group II : ( 27 % ),
Complete paraplegia of the legs ,
Some paralysis of the lower trunck,
Pralysis of the psoas major muscle
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Spina Bifida
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Myelomeningocele : Motor Loss ;
Group III : ( 23 % ) ,
The psoas major muscle is active
and its actions are unopposed resulting in flexion contractures of the hip,
The adductors and extensors of the
hip are paralyzed and so are the
muscles controlling knees, ankles
and feet
Spina Bifida
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Myelomeningocele : Motor Loss ;
Group IV : ( 45% ),
All group III muscles are active plus
the power of quadriceps femurs
muscle to hold the knee in full ext.
Some power in the dorsiflexors of
the foot, particularly tibialis anterior,
The calf muscles are paralyzed
causing talipes calcaneus. (cont..)
Spina Bifida
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Myleomenigocele : Motor Loss ;
Group IV : ( cont..)
In the lowest lesions in this group
there is some power in the
extensors and adductors of the hip
and in the flexors of the knee,
Group V : ( 4% ),
There is no orthopedic disabilities.
Spina Bifida
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Myelomenigocele : Sensory Loss ;
Corresponds closely to the level of
the motor loss,
Normal sensation level is usually
one segment higher than the lower
level of normal motor power,
Sensation loss is most important in
the feet, buttocks and the perineum
( risk of pressure sores ) .
Spina Bifida
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Myelomeningocele : ( cont..)
Neurogenic Bladder ;
The sphincters and pelvic muscles
are nearly always affected ,
Almost in every case the parasympathatic sacral roots (S2,3,4) to the
viscera is lacking causing paralysis
of detrusor, int. and ext. urethral
musculature, pelvic floor, anal
sphincters.
Spina Bifida
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Myelomeningocele : ( cont..)
Clinical signs observable in the first
day of life ;
An expressible bladder,
Patulous anus (open, level with the
buttocks for the natal cleft is
absent.
Perineal anaesthesia ,(no reaction
of pain to pin pricks ).
Continuous drippling of urine .
Spina Bifida
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Myelomeningocele : ( cont..)
Faecal Incontinence ;
Although present, seldom produces
overt disturbances in infants ,
In todlers and elder children (no
control and soiling occurs ),
Constipation masks lack of control,
Faecal impaction and often anal
prolaps is common in infants &
todlers
Spina Bifida
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Myelomeningocele : (cont..)
Associated anomalies;
Orthopedic deformities like
kyphosis , lordosis, scoliosis ,
Paralytic dyslocation of hips,
flexion contracture of the hips and
knees and,
Talipes,
Spina Bifida
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Myelomeningocele : (cont..)
Anomalies of the renal tract
include;
Neurogenic bladder,
VUR, with or without megaureters,
The kidney may be dysplastic,
cystic or duplicated,
Less common like ectopia vesica,
hypospadias, urethra diverticulum
or multiple vesical sacculations.
Spina Bifida
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Myelomeningocele : ( cont..)
Complications ;
Meningitis from infection of ulcerated sac specially if ruptured or after
repair, and acounts for 1/3 of all
deaths from spina bifida cystica,
Pressure sores on feet , sacral and
perineal areas,
UTI ,(stasis in neurogenic bladder),
Spina Bifida
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Myelomeningocele : (cont..)
Complications ; ( cont..)
Mental retardation , related to the
presence of hydrocephalus, ( in
77% intelligence is normal, 21%
retarded but educable, 2% grossly
retarded ),
Paralytic squint ( hydrocephalus),
optic atrophy (blidness), deafness .
Spina Bifida
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Myelomeningocele : ( cont..)
Assessment :
The type , level , size of spina bifida
and state of the sac (intact,
ruptured)
The persence of hydrocephalus,
The presence of meningitis,
Level of orthopedic disability,
Urinary tract abnormalities or UTI.
Spina Bifida
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Myelomeningocele : ( cont..)
Assessment ;
The presence of other congenital
anomalies,
The family,
All the above points and the
predicted disabilities should be fully
discussed and explained to
parents.
Spina Bifida
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Myelomeningocele : ( cont..)
Investigations :
Routine lab. Investigations, swabs,
AP and Lateral views of vertebral
column including the pelvis.
Brain and abdominal USS,
CSF ( during surgery ) .
Spina Bifida
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Myelomeningocele : ( cont…)
Treatment :
The aim of treatment is to produce
an ambulent , dry , free of smell of
urine and feaces , functioning to
the best of his itellectual and
physical potential ,educable and
capable of employment and
independent living .
Spina Bifida
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Myelomeningocele : ( cont..)
Early operation reduces the
incidence of meningitis , shortens
the hospital stay, relieves the
parents from long term dressings,
encourages acceptance of the
baby by the parents,
Early repair of the sac has no influence on the development of hydrocephalus or neurological
disabilities.
Spina Bifida
MyeloMeningocele
repair
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Spina Bifida
Thoraco-lumbar
myelomeningocele Repair
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Spina Bifida
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Myelomeningocele : ( cont..)
Treatment at birth (immediate repair)
Group IV and V ,
Active treatment is given for all
subsequent problems e.g. shunting of
hydrocephalus, urologic , orthopedic
etc.
Because of adverse factors further
treatment is differed.
Spina Bifida
Lumbosacral
myelomeningocele Repair
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Spina Bifida
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Although the spinal opening is surgically
repaired shortly after birth, the nerve
damage is permanent. This results in
varying degrees of paralysis of the lower
limbs, depending largely on the location
and severity of the lesion. Even with no
lesion there may be improperly formed or
missing vertebrae , and accompanying
nerve damage. There is no cure for
either of these conditions as nerve tissue
cannot be replaced or repaired.
Spina Bifida
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In addition to the physical and mobility
difficulties , most individuals with SB or
hydrocephalus will have some form of
learning disability. This means that they
are likely to have learning problems in
school, in spite of having average or
above average intelligence.
Other conditions found in some SB
patients are Chiari Malformation and
Tethered Cord Syndrome .
Spina Bifida
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Treatment for the variety of effects
of SB includes surgery, medication
physiotherapy, and the use of
assistive devices. Many will need
support to walk such as braces,
splints, crutches. Many will need
wheel chairs, and almost all will
have some form of bladder and
bowel dysfunction.
Spina Bifida
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These are conditions they outgrow. They
learn to control and live with them,
Ongoing therapy, medical care, and/or
surgical treatments will be necessary to
prevent and manage complications
throughout an individual’s life,
Research is greatly needed to develop
better methods of meeting the
challenges and complications posed by
spina bifida.
Spina Bifida
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How can SB be prevented ?
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Research indicates that addition of the bvitamin, folic acid, to the diet of women
of children bearing age may significantly
reduce the incidence NTDs such as SB.
Because NTD occurs before a woman is
likely to know she is pregnant, all women
capable of becoming pregnant should
consume 0.4mg of folic acid daily,
especially those with previous NTD
affected pregnancy or a close family
history of NTDs.
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Spina Bifida
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Allow the individual to reach his or
her potential, however, the cooperation of society , and the coordiation of services – medical,
nursing , social, educational and
community – is vital,
Education materials to parents,
families, and individuals with SB or
hydrocephalus as well as to educators, care givers, other
professionals.