Spina Bifida - Dr Hasan Nugud Bifida.pdf · · 2015-04-23Spina Bifida Spina Bifida Occulta : ... must be watched with care because of associated spinal cord dysplasia. Dr/HN/25
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Spina Bifida
Dr Hasan Nugud
Consultant
Paediatric Surgeon DR/HN/1
Spina Bifida
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 .
Dr/HN/2
Spina Bifida
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.
Dr/HN/3
Spina Bifida
Calssification :
1-Spina bifida occulta,
2-Spina bifida cystica;
a-Meningocele,
b-Myelomeningocele,
3-Rare types :
-Amelia,
-Rachischisis
Dr/HN/4
Spina Bifida
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 . Dr/HN/5
Spina Bifida
Spina Bifida Occulta :
The vertebrae are bifid without a
menigocele or myelomeningocele
below the intact skin, commonly
affects the 5th lumbar and first
sacral vertebrae
Dr/HN/6
Spina Bifida
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. Dr/HN/7
Spina Bifida
Dr/HN/8
Spina Bifida
Dr/HN/9
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.
Dr/HN/10
Spina Bifida
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.
Dr/HN/11
Spina Bifida
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. Dr/HN/12
Spina Bifida
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 ,(disad- vantage of miscarriage).
Dr/HN/13
Spina Bifida
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.
Dr/HN/14
Spina Bifida
AFP : (cont..)
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) .
Dr/HN/15
Spina Bifida
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.
Dr/HN/16
Spina Bifida
Spina Bifida Occulta :
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
Dr/HN/17
Spina Bifida
Spina Bifida Occulta : (cont...)
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 Dr/HN/18
Spina Bifida
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 inf-ected, the sinus should be excised after the antibiotic infection control
Dr/HN/19
Spina Bifida
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. Dr/HN/20
Spina Bifida
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 Dr/HN/21
Spina Bifida
Different
variants of
split
notochord
Dr/HN/22
Spina Bifida
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.
Dr/HN/23
Spina Bifida
Meningocele
Spina Bifida Cystica
Dr/HN/24
Spina Bifida
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.
Dr/HN/25
Spina Bifida
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,
Dr/HN/26
Spina Bifida
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 inter-
peduncular gap is not so extensive. Dr/HN/27
Spina Bifida
Meningocele : (cont..)
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.
Dr/HN/28
Spina Bifida
Meningocele : (cont..)
If the sac is ulcerated it should be repaired immediately after birth, be- fore 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.
Dr/HN/29
Spina Bifida
SB Cystica :
Myelomeningocele;
94% of spina bifida cystica ,
The most serious variety,
Consists of a bifid spine with prot- rusion and dysplasia of the menin- ges and spinal cord and is always acoompanied by neurological signs
Dr/HN/30
Spina Bifda
Myelomeningocele : (cont..)
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).
Dr/HN/31
Spina Bifida
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 ill-
formed skin is almost always
present. Dr/HN/32
Spina Bifida
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 . Dr/HN/33
Spina Bifida
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, un- tubed on the surface.
These babies are usually stillborn with other multiple congenital anomalies.
Dr/HN/34
Spina Bifida
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.
D/HN/35
Spina Bifida
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.
Dr/HN/36
Spina Bifida
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 Dr/HN/37
Spina Bifida
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 Dr/HN/38
Spina Bifida
Myelomeningocele : Motor Loss ;
Group II : ( 27 % ),
Complete paraplegia of the legs ,
Some paralysis of the lower trunck,
Pralysis of the psoas major muscle
Dr/HN/39
Spina Bifida
Myelomeningocele : Motor Loss ;
Group III : ( 23 % ) ,
The psoas major muscle is active and its actions are unopposed resul- ting 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 Dr/HN/40
Spina Bifida
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..)
Dr/HN/41
Spina Bifida
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. Dr/HN/42
Spina Bifida
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 ) .
Dr/HN/43
Spina Bifida
Myelomeningocele : ( cont..)
Neurogenic Bladder ;
The sphincters and pelvic muscles are nearly always affected ,
Almost in every case the parasym- pathatic sacral roots (S2,3,4) to the viscera is lacking causing paralysis of detrusor, int. and ext. urethral musculature, pelvic floor, anal sphincters. Dr/HN/44
Spina Bifida
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 . Dr/HN/45
Spina Bifida
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 Dr/HN/46
Spina Bifida
Myelomeningocele : (cont..)
Associated anomalies;
Orthopedic deformities like
kyphosis , lordosis, scoliosis ,
Paralytic dyslocation of hips,
flexion contracture of the hips and
knees and,
Talipes, Dr/HN/47
Spina Bifida
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.
Dr/HN/48
Spina Bifida
Myelomeningocele : ( cont..)
Complications ;
Meningitis from infection of ulcera- ted 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), Dr/HN/49
Spina Bifida
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 .
Dr/HN/50
Spina Bifida
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. Dr/HN/51
Spina Bifida
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.
Dr/HN/52
Spina Bifida
Myelomeningocele : ( cont..)
Investigations :
Routine lab. Investigations, swabs,
AP and Lateral views of vertebral
column including the pelvis.
Brain and abdominal USS,
CSF ( during surgery ) .
Dr/HN/53
Spina Bifida
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 . Dr/HN/54
Spina Bifida
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 influ- ence on the development of hydro- cephalus or neurological disabilities. Dr/HN/55
Spina Bifida
Myelo-
Meningocele
repair
Dr/HN/56
Spina Bifida
Thoraco-lumbar
myelomeningo-
cele Repair
Dr/HN/57
Spina Bifida
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.
Dr/HN/58
Spina Bifida
Lumbosacral
myelomeningo-
cele Repair
Dr/HN/59
Spina Bifida
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. Dr/HN/60
Spina Bifida
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 .
Dr/HN/61
Spina Bifida
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. Dr/HN/62
Spina Bifida
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. Dr/HN/63
Spina Bifida
How can SB be prevented ?
Research indicates that addition of the b-vitamin, 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.
Dr/HN/64
Spina Bifida
Allow the individual to reach his or her potential, however, the co- operation of society , and the co- ordiation 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 educa- tors, care givers, other professionals.
Dr/HN/65
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