Presenter Dr Narayanan sk
Chairpersons Dr Ravishanker
• Spinal Dysraphism is a generalised term
for a group of congenital abnormalities
that can cause progressive neurological
damage and therefore the deterioration of
neural and physical function. The
abnormalities are also sometimes known
as neural tube defects.
• Spina bifida latin term for split spine.
• Congenital disorder in which 2 halves of the
posterior vertebral arch has failed to fuse.
• This can lead to hernial protrusion of spinal cord
and its meninges
• Results in variable neurological defects
depending on the location and severity of the
In the third and fourth week
of intra uterine life the neural
groove appears as a dorsal
Focal thickening caused by
the proliferation of
ectodermal cells.Theses cells
increase in number and
height .A groove is formed in
the sagital plane .the groove
deepens causing the lateral
portion to face eacher.
By about 21 st day cell
adhesion causes formation
of Neural tube
Initial fusion occurs near
the center of embryo,then
it proceeds in both the
directions and the cephalic
end closes first.The
separates and forms the
neural arch and paraspinal
The embryonic origin of spina bifida , if the
abnormality occurs between 26 -28 days ,
during the phase of closure of neural tube its is
known as NEURALIZATION DEFECT
It includes myelomeningocele and anencephaly
If the abnormality occurs between 28 -48 days
,during the phase of canalization it is known as
POST NEURALIZATION DEFECT
It includes meningocele, lipomenigocele and
The exact mechanisms by which humans
develop this abnormality remain elusive
The proposed theories are
•Primary failure of closure of the neural tube
•Secondary rupture of the neural tube due to
increase in csf pressure
Spina bifida occulta
• Mildest form
• Incomplete formation of posterior arch of the spinal
• Usually incidental finding
• Seen in LS spine, commonest S1
• Occasionally patient may have fatty deposit,
hemangioma or tuft of hair
Spina bifida cystica
• Meningocele- protrusion of only the dura and
arachnoid through the defect in the vertebral
lamina forming a cystic swelling usually in
• Spinal cord remains in the vertebral canal
Spina bifida cystica
• 10 times more frequent
• portion of the spinal cord or the nerve roots are
displaced through the spina bifida defect into
• 1 in 1000 live births
• Female: male ratio= 1.2:1
• Mainly seen in Hispanic women
• Incidence decreased by about 80% due to
prenatal screening using USG and
measurement of maternal serum α
fetoprotein & administration of folate
before and after pregnancy
• Exact cause not known
• Proposed theories are :
• exposure to fungus Phytophthora infestans
by excessive consumption of potatoes
• Use of anti seizure drugs(sodium valproate)
• Genetic – 6-8%
• Couple with one affected infant- risk of
subsequent sibling acquiring major CNS
malformation is 1 in 14
Folic acid deficiency-
• 80-100% reduction in neural tube defects with
administration of folic acid.
• Daily dose 0.4 mg.
• max dose 1mg/day
• Up to 4mg/day to women who have given birth
to a prior affected child
•Hydrocephalus – dilatation of
ventricles of brain from excessive CSF
• Seen in 80-90% of meningomyelocele
• Patients with thoracic and upper lumbar lesions
have a higher incidence than those with lower
lumbar and sacral lesions.
• Accumulation of CSF in the enlarged central
canal of the spinal cord.
• Can cause three problems in patients with
• Increase in level of paralysis
• Progressive scoiliosis
• Weakness of hands and upper extremities
Arnold chiari malformation
• Caudal displacement of posterior lobe of
• Causes dysfunction of lower cranial nerves
which results in weakness and paralysis of vocal
cords and difficulty in feeding
• Placement of VP shunt often resolves this
Tethered spinal cord
• Usually a MRI finding
• Usual clinical findings are loss of motor
function, development of spasticity in lower
limbs, development of scoliosis before 6 years
age in absence of vertebral anomalies, back pain
and increased lumbar lordosis and changes in
• MRI evaluation should be performed.
• Almost all children with meningomyelocele have
• Chronic renal failure and sepsis from UTI were
the most common causes of delayed mortality
• Mainstay of treatment is clean intermittent
catheterisation to prevent development of
hydronephrosis to maintain bladder compliance
• Incidence 3.8 – 38%
• Manifests in form of local rashes or mucosal
• Latex skin prick test and assay for latex specific
immunoglobulin E in serum
• Non exposure to latex is recommended in all
patients with meningomyelocele.
Classification –based on level of lesion
• Most frequent deformity encountered by
surgeons are spinal deformities
• No active hip flexion, no voluntary muscle
control of lower extremity
Upper lumbar level
• Hip dysplasia and dislocation due to imbalance
of flexors and adductors
• No extension/ abduction
Lower lumbar level
• Hip adductor strenghth and quadriceps power to
provide active hip extension
• Weakness of foot, ankle, hip abductors and
extensors lurching gait imposing great deal of
stress on unbraced knee
• Weakness of peroneus and intrinsic muscles of
• Partial paralysis and insensate skin can lead to
foot problems and neurogenic ulcers
• Not all patients have one of these classic levels of
Management must be integrated to treat the child as a
whole and provide the family the necessary support.
Best assessed and treated by multidisciplinary clinics.
• Administrative or registered nurse to function as
patient’s advocate. Needs addressed over time including
educational, vocational and sexual counselling.
• Orthotist – to provide and repair lower extremity and
• Physical therapist- to aid lower extremity functional
assessment, bracing needs and ROM exercises for daily
• Nurse to teach about skin care and self catheterisation
• Psychologist to help parents cope with many
challenges and stresses related to child’s
• Urologist to monitor genitourinary function and
maximise bladder control
• Neurosurgeon to monitor shunt dysfunction and
development of tethered cord
• Social worker to assist family in finding financial
• Experienced neurodevelopmental paediatrician
to oversee the whole process.
• Finally an orthopaedician to produce a stable
posture for sitting or standing.
• Principle component of management
• Main goal is to stabilise joints in the absence of lower extremity
muscle function and facilitate weight bearing and ambulation.
• Specific protocols by spinal level
Thoracic and upper lumbar levels
• Unable to pull themselves to a standing position
• Usually full lower extremity bracing is required ( HKAFO)
• When patient is ready for upright position standing frames or
wheel standers can be given.
• Wheel standers allows children significant amount of mobility
on flat surfaces.
• Once they are comfortable with upright position, then it can be
converted to HKAFO
Lower lumbar and sacral levels
• Good quadriceps function
• Should be able to function with AFO
• First prescription should always be KAFO,
after initial adaptation and ambulation
efforts, can be converted to AFO.
• Frequent falls, loss of confidence and
recurrent knee effusions KAFO should
Ankle- Foot Orthroses
• Polypropylene AFO are
mainstay of lower
• Usually used in sacral level
• Main function is to protect
foot and toes during weight
bearing and to stabilise the
Knee ankle foot
• Usually used in
• KAFO consists of
thigh cuff and knee
Hip knee ankle foot
• Used in patients with
inadequate hip strength
or in patients who
cannot bear weight with
• Consists of two long leg
connected by a pelvic
band and a free hip
• Sophisticated form of
• Unique feature is two long
leg components are
connected by a spring
loaded cable, flexion of the
hip with advancement of
the limb produces passive
extension allowing energy
• Other mobility aids are standing frame,
parapodium, wheel chair.
• 75 % of children with myelomeningocele have
foot deformities that can seriously limit
• The main goal of orthopedic treatment is to
have a plantigrade, mobile, braceable foot.
• Manipulation and casting should be used with
• Study by Frawley et al showed calcaneal
deformity to be the most common, followed by
equinus, valgus, club foot and vertical talus.
• All major foot deformities have high frequency
• Seen in 1/3 rd of children with
• Level of lesion L5-S1
• Due to active anterior leg muscles and inactive
• Supple deformity manipulation and splinting
in neutral position.
• Muscle imbalance
simple tenotomy of
ankle dorsiflexors and
• tendons of the extensor
tibial, peroneus brevis
and longus tendons are
• Combined with transfer of tibialis
anterior tendon to calcaneum.
• Seen with high lumbar or thoracic level lesions.
• Equinus deformity can be prevented by gentle
passive manipulation, bracing and splinting.
• If deformity persists:
• Tendoachilles lengthening
• Careful postoperative casting for few weeks,
followed by fitting of orthroses.
• Long flexors must be released if toe deformity
presentmay result in pressure sores.
• Seen in 30% of meningomyelocele.
• Irrespective of level of lesion.
• Characterised by :
Supination varus deformity
Rotation malalignment of calcaneum and talus
Subluxation of calcaneocuboid & talonavicular joint
• Recurrence of deformity is common after adequate
• Manipulation and casting techniques are difficult due to
absence of pain and protective sensation which can lead
to fractures and pressure sores.
• Ideal age for surgery : 10-18 months
• Radical posteromedial- lateral release through
• Talar enucleation( VO procedure)
• Difficulty in wound closure is common and
rotational flaps can be done for primary closure.
• Recent studies show wound can be left open as
much as necessary with foot in correct position
and meticulous postoperative care.
• Verebely- Ogston procedure
• Decancellation procedure- removing of
cancellous bone from talus and cuboid.
• Leaves a hollow shell of bone and more space for
• Foot is manipulated into neutral position and
because of the collapse of talus and cuboid
correction of residual deformity.
• Isolated varus deformity of hind foot is
• Imbalance between invertors and evertors
should be evaluated.
• Lateral closing wedge osteotomy of
calcaneum is done and calcaneum
should be translated laterally if
possible to increase the correction.
Cavo varus deformity
• Occurs in sacral level lesion.
• Cavus is the primary deformity which produces
• Rigidity of the varus deformity should be
determined before planning for surgery.
• Supple deformity radical plantar release
without hind foot bone surgery is done.
Radical plantar release
• Release of the plantar
short flexors of
calcaneum is done.
• If the deformity is rigid despite radical
plantar release, closing wedge osteotomy
• Triple arthrodesis rarely indicated as a
• Subtalar, calcaneocuboid and
talonavicular joint are fused.
• Occurs in L5-S1 level lesion.
• Mainly due to unopposed action of anterior tibial
• Supple deformity simple tenotomy of anterior
• If some amount of gastrocnemius-soleus activity is
present with no spasticity, the anterior tibial tendon
can be transferred to the mid foot in line with 3rd
• Split anterior tibial transfer can be done with the
lateral half of tendon inserted in the cuboid.
• Osteotomy of 1st cuneiform or base of 1st metatarsal
may be required for residual deformity.
Hind foot valgus
• Initially hind foot valgus can be controlled
by a well fitted orthroses but as the child
becomes taller and heavier the control of
deformity becomes difficult and then
surgical treatment is indicated.
• Hind foot valgus > 10 mm of lateral shift
of calcaneum is significant.
• Grice extraarticular arthrodesis is the
classical treatment of this problem.
Grice extraarticular arthrodesis
• Extraarticular fusion of
subtalar joint with bone
graft is done.
• For rigid fixation and
hold of the graft, a k-
wire or screw can be
used anteriorly from the
talar neck into the
• Seen in 10% children with meningomyelocele.
• Characterised by malalignment of hind foot and mid
• Two types:Developmental and Congenital
Both requires surgical correction once the deformity
is partially corrected by manipulation and serial
• Ideal age for surgery : 12-18 months
• Posteromedial or lateral release should be performed.
• Anterior tibial tendon can be resected or transferred
into talar neck.
• If required, subtalar arthrodesis can be done.
Pes cavus deformity
• Seen in sacral level lesion.
• Painful callosities under the metatarsal
head and difficulties with shoe wear.
• For isolated cavus deformity with no hind
foot varus, radical plantar release is done.
• If varus deformity is done, medial subtalar
release is done followed by immobilisation
in POP cast.
• Claw or hammer toe occurs in sacral lesions.
• For flexible claw defomities, simple tenotomy of
flexors at the level of proximal phalanx is done.
• For rigid claw deformity, partial resection of IP
joint or arthrodesis.
• If cavus deformity is present, Jones procedure
can be performed- tendon suspension method.
longus tendon is
attached to neck of
interphalangeal joint is
arthrodesed and fixed
by medullary wire and
by suturing distal end
of extensor hallucis
longus tendon to soft
tissues over proximal
• Common deformity in ambulatory patients, irrespective
of level of lesion.
• Deformities arise from distal tibia or subtalar joint.
• Common sequelae is skin irritation or breakdown over
the medial malleolus from excessive pressure over the
• To evaluate valgus ankle deformity, 4 factors must be
1. Skeletal maturity
2. Degree of fibular shortening
3. Degree of valgus tilt in ankle mortise
4. Lateral shift of calcaneum in relation to weight bearing
axis of tibia.
• Operative treatment is indicated when ankle
valgus deformity causes problems with
orthrotic fitting and cannot be relieved by
• Tenodesis of tendoachilles to fibula above the
physis if the valgus tilt is between 10-25
Valgus deformity is due to
lateral compartment paralysis,
so underdevelopment of fibula
will be present, pulling down
the fibula during weight
bearing and dorsiflexion
Hemiepiphysiodesis of distal tibial
• Done in skeletally immature patients.
• Medial tibial physis is closed by direct
surgical ablation or stapling or using
• Growth arrest 0f the medial side
combined with continued growth in the
lateral side corrects the deformity.
• Advantage is immediate weight bearing.
Supramalleolar varus derotation
• Recommended in children older than 10
• Seen in low lumbar level lesions.
• Severe fibular shortening 10-20mm
• Valgus tilt > 20 degrees
• External tibial torsion.
• Postoperatively above knee casts for 3
weeks followed by below knee casts for 2
weeks then full weight bearing.
Distal 1/3rd fibular oblique osteotomy is done
followed by medial based wedge osteotomy above
Size of the wedge depends on valgus to be
Fixation with plate and screws
• More common than extention contractures
• Seen in thoracic or lumbar level lesions
• contractures of 20 degree are common at birth
• Becomes fixed causes
o the typical position assumed when supine—hips in abduction,
flexion, and external rotation; knees in flexion; and feet in
o gradual contracture of the hamstring and biceps muscles, with
contracture of the posterior knee capsule from quadriceps
weakness and prolonged sitting
o spasticity of the hamstrings that may occur with the tethered cord
o hip flexion contracture or calcaneal deformity in the ambulatory
patient. Knee flexion contractures
• Knee flexion upto 20 degrees ---effective
bracing and standing program
• 20- 30 degree----Radical flexor release
can be done
• 30-45 degree supracondylar extention
osteotomy of femur is done
• If hip contractures are present both should
be operated at the same time
Radical flexor release
Minimal procedure – partial division of
• Due to unopposed quadriceps action
• Serial casting successful in some patients
• Surgical management consist of
oV-Y quardriceps lengthening
oPostrerior displacement of the hamstring
V-Y quadriceps lengthening
A, Detachment of rectus
femoris tendon from rectus
femoris, vastus medialis, and
vastus lateralis muscles; vastus
medialis and lateralis muscles
are separated from iliotibial
band, lateral hamstrings, medial
hamstrings, and sartorius
muscles. B, When knee is flexed,
hamstring muscles and tensor
fasciae latae slip posterior to
knee axis, restoring normal
function. Quadriceps muscles
are repaired in lengthened
Valgus or varus deformity
• Supracondylar or tibial osteotomy with
internal fixation can be done
• Hemiepiphysiodesis can be done using
staples if recoganised early
• Mainly due to:
▫ Muscle imbalance
▫ Congenital dysplasia
▫ Habitual posture
• Seen in high lumbar or thoracic level lesion.
• Caused due to unopposed action of hip flexors,
habitual posture & spasticity of hip flexors.
• Flexion contracture of 20-30 degrees can be
accomodated by lumbar lordosis and knee flexion.
• >30 degrees anterior hip release of sartorius,
rectus femoris, iliopsoas, tensor fascia lata &
anterior hip capsule.
• In this procedure, upto 60 degrees gets corrected.
• If flexion persistent subtrochanteric extension
Flexion abduction external rotation
• Seen in thoracic level lesion.
• The occurrence of this deformity may be
decreased by the use of night splints and range
of motion exercises.
• Complete hip release
done only when
deformity interferes with bracing.
External rotation contracture
• Seen in low lumbar lesions.
• Initially bracing & physiotherapy.
• If persists subtrochanteric medial
• Seen in high level lesions.
• Fascial release indicated when abduction
contracture causes pelvic obliquity &
scoliosis which inteferes with function or
• Fascial release fascia over gluteus
medius, minimus & tensor fascia lata is
divided and fasciotomy done.
• Seen in high level lesions.
• Due to spasticity and contracture of
• Surgery indicated when it causes pelvic
obliquity & inteferes with sitting &
• Adductor release done.
• Seen in 50% of children with meningomyelocele.
• Three types:
• Developmental treated by Pavlik harness,
traction, closed reduction & hip spica
• Teratological seen in high level lesions,
initially acetabulum dyplastic, with head of
femur displaced proximally, should not be
• Most common type- 50-70%
• Seen in low level lesions L3-L4.
• Due to imbalance between adduction &
• Principles of treatment:
• Obtain concentric reduction
• Correct bony abnormality
• Seek to balance flexor-adductor & extensor-
• Main goal – maximal function rather than
• Should be atttempted only where child will
derive considerable benefit from the
• Not always successful stiff painful hip
• If required anterior open reduction with
Correction of bony abnormality
• Femoral deformity corrected by proximal
femoral resection & inter position
• Done in severely retarded multiply
handicapped with dislocated hips and
severe adduction contraction.
• Acetabular deformity corrected by :
• Pemberton osteotomy
• Daga osteotomy
• Shelf procedure
• Steel triple innominate osteotomy
• Chiari osteotomy- preferred, as concentric
reduction is not required & the osteotomy
does not redirect the acetabulum
posteriorly. It is in the direction of the
Chiari osteotomy An osteotomy of the pelvis is
performed at the superior margin of the
acetabulum, and the pelvis inferior to the
osteotomy along with the femur is displaced
Muscle balancing procedures
•Simple release of ilioposas tendon with adductor
Adductor longus tendon is sepereted from brevis and exiced
Adductor brevis is separated from pectineus and lesser trochanter
Identified ilioposas released
• Posterior transfer of adductor muscle mass on the
ischium to convert it into more of hip extensor.
• Transfer of iliopsoas tendon- 1st described by Mustard
in 1952.anterolateral transfer.mobilising the iliopsoas
tendon with the lesser trochanter into the pelvis, re
rooting it through a large trough in the ilium and
through the gluteal muscles and re inserting the tendon
into the greater trochanteric.
• Sharrard procedure:
• 1959, posterior
transfer of iliopsoas
• Transfer of iliopsoas
muscle through a hole
in the ilium
femoral nerve with
reinsertion of the
tendon to the posterior
through a drill hole
and reattachment of
iliacus portionto the
outer wall of the ilium.
• Transfer of external oblique muscle:
• Thomas and colleagues described transfer of
external oblique abdominis to greater
• Modified by Phillips and Lindseth adductors,
external oblique & tensor fascia lata transferred.
• Adductors sutured to ischium.
• External oblique with aponeurosis to greater
• TFL rotated externally and sutured to iliac crest
proximally and tendon of gluteus distally
• mcKay described tendon transfer with femoral
• Three types–
• Infrapelvic contracture of adductors and TFL
• Supra pelvic uncompenseted scoliosis
resulting in boney deformity of LS spine
• Pelvic boney deformity of sacrum and SI joint
• Infrapelvic –splinting , ROM exercises and
• Suprapelvic—scoliosis by orthoses and spinal
• Pelvic--- obliquty is 20 degree pelvic osteotomy
• Upto 40 degree gets corrected
determination of size
of iliac wedge to be
Exposure of ilium. ,
removal of wedge
from low side,
wedge is fixed with
two Kirschner wires.
• Most common skeletal deformity
• Higher the lesion, greater the incidence.
• Deformity may be in the form of scoliosis, lordosis or
kyphosis which may be either congenital, developmental
• Congenital spinal deformities are managed as in any other
• Developmental spinal deformity treated according to
severity, evidence of progression and skeletal maturity.
• By using spinal braces for developmental deformities, the
progression of the curve can be retarded until child is 10-
• Non operative treatment is not usually successful.
• Overall goals are to correct deformity, preserve
sitting balance, maintain level pelvis, preserve
respiratory function, increase the trunk height.
• Preoperatively surgeon must ensure shunt
function is stable, no UTI, weight bearing skin
of pelvis and upper thigh are free of pressure
sores, and skin over spine should be normal.
• Postoperatively patient must be managed in
ICU until stable.
• Urinary tract must be kept clean to avoid
• Most common and usually progressive
• Incidence related to level of lesion.
▫ 100% T12
▫ 80% L2
▫ 70% L3
▫ 60% L4
▫ 25% L5
▫ 5% S1
• Thoracic lumbosacral orthrosis used if
curve is upto 30°.
• Indications for spinal fusion include a
progressive increase in angular deformity that
cannot be controlled by bracing, unacceptable
deformities and progressive thoracic lordosis.
• Most cases require anterior and posterior
• Anterior procedure is done initially.
• Anterior release with discectomy &
• Anterior release with strut grafting
• Anterior fusion
• Correction with Zielke instrumentation.
• 2nd stage posterior procedure is done
7 days after the anterior procedure.
• Choice of instrumentation includes:
▫ Square ended
▫ Harrington rods with sacral hooks
▫ Spinal instrumentation.
• Seen in 10% patients with meningomyelocele.
• Associated with thoracic and lumbar lesions
• Apex deformity varies from T12-L5, most common L2
• Anterior and posterior procedures are recommended.
• Anterior done first consists of discectomies throughout
the deformity with fusion accomplished by intervertebral
arthrodesis or strut grafting.
• Posterior procedure is done 7 days later and involves long
fusion with instrumentation to include the sacrum.
• Harrington or Lugue instrumentation is recommended.
• Goal of surgery is to align the trunk vertically over
sacrum and pelvis.
• Less common deformity
• Can lead to difficulty in sitting,
intertriginous skin breakdown,
difficulty with self catheterisation in
females because of posterior rotation of
• Treatment by combination of anterior
and posterior spinal release and
• In rigid deformities, postural reduction
in traction after spinal release, before
definitive instrumentation may improve
• Rare manifestation characterised by
significantly assymetric involvement of lower
extremities with one leg being significantly
affected and the controlateral leg being normal
or nearly normal.
• This condition is referred as hemi spina bifida.
• There may be limb length inequality due to
paralysis induced growth inhibition on the
affected side which may require orthotic
management, epiphyseodesis or lenghthening.
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