Spina bifida
Presenter Dr Narayanan sk
Chairpersons Dr Ravishanker
Dr Manjunath
Spinal Dysraphism
• Spinal Dysraphism is a generalised term
for a group of congenital abnormalities
that can cause progres...
Spina bifida
• Spina bifida latin term for split spine.
• Congenital disorder in which 2 halves of the
posterior vertebral...
Embryology
In the third and fourth week
of intra uterine life the neural
groove appears as a dorsal
Focal thickening cause...
By about 21 st day cell
adhesion causes formation
of Neural tube
Initial fusion occurs near
the center of embryo,then
it p...
The embryonic origin of spina bifida , if the
abnormality occurs between 26 -28 days ,
during the phase of closure of neur...
The exact mechanisms by which humans
develop this abnormality remain elusive
The proposed theories are
•Primary failure of...
Types of spina bifida
• Spina bifida occulta
• Spina bifida cystica
 Meningocele
 Myelomeningocele
Spina bifida occulta
• Mildest form
• Incomplete formation of posterior arch of the spinal
column
• Usually incidental fin...
Spina bifida cystica
Two types:
• Meningocele- protrusion of only the dura and
arachnoid through the defect in the verteb...
Spina bifida cystica
Myelomeningocele-
• 10 times more frequent
• portion of the spinal cord or the nerve roots are
displa...
Incidence
• 1 in 1000 live births
• Female: male ratio= 1.2:1
• Mainly seen in Hispanic women
• Incidence decreased by abo...
Etiology
• Exact cause not known
• Proposed theories are :
• exposure to fungus Phytophthora infestans
by excessive consum...
Folic acid deficiency-
• 80-100% reduction in neural tube defects with
administration of folic acid.
• Daily dose 0.4 mg.
...
Associated conditions
•Hydrocephalus – dilatation of
ventricles of brain from excessive CSF
• Seen in 80-90% of meningomye...
Hydrosyringomyelia
• Accumulation of CSF in the enlarged central
canal of the spinal cord.
• Can cause three problems in p...
Arnold chiari malformation
• Caudal displacement of posterior lobe of
cerebellum
• Causes dysfunction of lower cranial ner...
Tethered spinal cord
• Usually a MRI finding
• Usual clinical findings are loss of motor
function, development of spastici...
Urological dysfunction
• Almost all children with meningomyelocele have
bladder dysfunction.
• Chronic renal failure and s...
Latex allergy
• Incidence 3.8 – 38%
• Manifests in form of local rashes or mucosal
irritation
• Latex skin prick test and ...
Classification –based on level of lesion
Thoracic level
• Most frequent deformity encountered by
surgeons are spinal defo...
Lower lumbar level
• Hip adductor strenghth and quadriceps power to
provide active hip extension
• Weakness of foot, ankl...
Multidisciplinary care
 Management must be integrated to treat the child as a
whole and provide the family the necessary ...
• Psychologist to help parents cope with many
challenges and stresses related to child’s
diability.
• Urologist to monitor...
Orthopaedic management
Orthotic management
• Principle component of management
• Main goal is to stabilise joints in the absence of lower extremi...
Lower lumbar and sacral levels
• Good quadriceps function
• Should be able to function with AFO
• First prescription shou...
Braces
Ankle- Foot Orthroses
• Polypropylene AFO are
mainstay of lower
extremity bracing
• Usually used in sacral level
l...
Knee ankle foot
orthroses
• Usually used in
upper lumbar
lesion where
quadriceps action
is weak.
• KAFO consists of
AFO c...
Hip knee ankle foot
orthroses
• Used in patients with
inadequate hip strength
or in patients who
cannot bear weight with
...
Reciprocating gate
orthroses
• Sophisticated form of
HKAFO
• Unique feature is two long
leg components are
connected by a...
• Other mobility aids are standing frame,
parapodium, wheel chair.
Surgical management of specific
problems
Foot
• 75 % of children with myelomeningocele have
foot deformities that can seriously limit
function.
• The main goal of ...
Calcaneal deformity
• Seen in 1/3 rd of children with
meningomyelocele.
• Level of lesion L5-S1
• Due to active anterior l...
• Muscle imbalance
simple tenotomy of
ankle dorsiflexors and
evertors
• “Anterolateral
release”
• tendons of the extensor...
• Combined with transfer of tibialis
anterior tendon to calcaneum.
Equinus deformity
• Seen with high lumbar or thoracic level lesions.
• Equinus deformity can be prevented by gentle
passiv...
Club foot
• Seen in 30% of meningomyelocele.
• Irrespective of level of lesion.
• Characterised by :
 Severe rigidity
 S...
• Ideal age for surgery : 10-18 months
• Radical posteromedial- lateral release through
Cincinnati incision.
• Naviculecto...
VO procedure
• Verebely- Ogston procedure
• Decancellation procedure- removing of
cancellous bone from talus and cuboid.
•...
Varus deformity
• Isolated varus deformity of hind foot is
very rare.
• Imbalance between invertors and evertors
should be...
Cavo varus deformity
• Occurs in sacral level lesion.
• Cavus is the primary deformity which produces
varus.
• Rigidity of...
Radical plantar release
• Release of the plantar
aponeurosis,
abductor hallucis,
short flexors of
calcaneum is done.
• If the deformity is rigid despite radical
plantar release, closing wedge osteotomy
is done.
• Triple arthrodesis rarely ...
Supination deformity
• Occurs in L5-S1 level lesion.
• Mainly due to unopposed action of anterior tibial
muscle.
• Supple ...
Hind foot valgus
• Initially hind foot valgus can be controlled
by a well fitted orthroses but as the child
becomes taller...
Grice extraarticular arthrodesis
• Extraarticular fusion of
subtalar joint with bone
graft is done.
• For rigid fixation a...
Vertical talus
• Seen in 10% children with meningomyelocele.
• Characterised by malalignment of hind foot and mid
foot.
• ...
Pes cavus deformity
• Seen in sacral level lesion.
• Painful callosities under the metatarsal
head and difficulties with s...
Toe deformity
• Claw or hammer toe occurs in sacral lesions.
• For flexible claw defomities, simple tenotomy of
flexors at...
Extensor hallucis
longus tendon is
attached to neck of
first metatarsal;
interphalangeal joint is
arthrodesed and fixed
by...
Ankle
 valgus deformity
• Common deformity in ambulatory patients, irrespective
of level of lesion.
• Deformities arise f...
• Operative treatment is indicated when ankle
valgus deformity causes problems with
orthrotic fitting and cannot be reliev...
Hemiepiphysiodesis of distal tibial
epiphysis
• Done in skeletally immature patients.
• Medial tibial physis is closed by ...
Supramalleolar varus derotation
osteotomy
• Recommended in children older than 10
years.
• Seen in low lumbar level lesion...
Distal 1/3rd fibular oblique osteotomy is done
followed by medial based wedge osteotomy above
the epiphysis.
Size of the w...
Knee
Flexion contractures
• More common than extention contractures
• Seen in thoracic or lumbar level lesions
• contract...
• Knee flexion upto 20 degrees ---effective
bracing and standing program
• 20- 30 degree----Radical flexor release
can be ...
Radical flexor release
Minimal procedure – partial division of
supracondylar extention osteotomy
of femur
Extention contractures
• Due to unopposed quadriceps action
• Serial casting successful in some patients
• Surgical manage...
V-Y quadriceps lengthening
A, Detachment of rectus
femoris tendon from rectus
femoris, vastus medialis, and
vastus lateral...
Valgus or varus deformity
• Supracondylar or tibial osteotomy with
internal fixation can be done
• Hemiepiphysiodesis can ...
Hip deformities
• Mainly due to:
▫ Muscle imbalance
▫ Congenital dysplasia
▫ Habitual posture
Flexion contracture
• Seen in high lumbar or thoracic level lesion.
• Caused due to unopposed action of hip flexors,
habit...
Flexion abduction external rotation
contracture
• Seen in thoracic level lesion.
• The occurrence of this deformity may be...
External rotation contracture
• Seen in low lumbar lesions.
• Initially bracing & physiotherapy.
• If persists subtrochan...
Abduction contracture
• Seen in high level lesions.
• Fascial release indicated when abduction
contracture causes pelvic o...
Adduction contracture
• Seen in high level lesions.
• Due to spasticity and contracture of
adductor muscles.
• Surgery ind...
Hip subluxation/dislocation
• Seen in 50% of children with meningomyelocele.
• Three types:
• Developmental treated by Pa...
Paralytic
• Most common type- 50-70%
• Seen in low level lesions L3-L4.
• Due to imbalance between adduction &
abduction f...
Reduction
• Controversial
• Should be atttempted only where child will
derive considerable benefit from the
procedure.
• N...
Correction of bony abnormality
• Femoral deformity corrected by proximal
femoral resection & inter position
arthroplasty.
...
• Acetabular deformity corrected by :
• Pemberton osteotomy
• Daga osteotomy
• Shelf procedure
• Steel triple innominate o...
Chiari osteotomy An osteotomy of the pelvis is
performed at the superior margin of the
acetabulum, and the pelvis inferio...
Muscle balancing procedures
•Simple release of ilioposas tendon with adductor
release
Adductor longus tendon is sepereted...
• Posterior transfer of adductor muscle mass on the
ischium to convert it into more of hip extensor.
• Transfer of iliopso...
• Sharrard procedure:
• 1959, posterior
transfer of iliopsoas
tendon.
• Transfer of iliopsoas
muscle through a hole
in the...
• Transfer of external oblique muscle:
• Thomas and colleagues described transfer of
external oblique abdominis to greater...
Pelvic obliquity
• Common
• Three types–
• Infrapelvic  contracture of adductors and TFL
• Supra pelvic uncompenseted sc...
• Pelvic
osteotomy
Preoperative
determination of size
of iliac wedge to be
removed and
transferred. ,
Exposure of ilium. ,...
Spinal deformities
• Most common skeletal deformity
• Higher the lesion, greater the incidence.
• Deformity may be in the ...
• Overall goals are to correct deformity, preserve
sitting balance, maintain level pelvis, preserve
respiratory function, ...
Scoliosis
• Most common and usually progressive
• Incidence related to level of lesion.
▫ 100% T12
▫ 80% L2
▫ 70% L3
▫ 60%...
• Indications for spinal fusion include a
progressive increase in angular deformity that
cannot be controlled by bracing, ...
• 2nd stage posterior procedure is done
7 days after the anterior procedure.
• Choice of instrumentation includes:
▫ Squar...
Kyphosis
• Seen in 10% patients with meningomyelocele.
• Associated with thoracic and lumbar lesions
• Apex deformity vari...
Hyperlordosis
• Less common deformity
• Can lead to difficulty in sitting,
intertriginous skin breakdown,
difficulty with ...
Hemimyelodysplasia
• Rare manifestation characterised by
significantly assymetric involvement of lower
extremities with on...
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spina bifida

  1. 1. Spina bifida Presenter Dr Narayanan sk Chairpersons Dr Ravishanker Dr Manjunath
  2. 2. Spinal Dysraphism • 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.
  3. 3. Spina bifida • 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 lesion.
  4. 4. Embryology 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.
  5. 5. 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 superfical ectoderm separates and forms the neural arch and paraspinal muscles
  6. 6. 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 diastematomyslia
  7. 7. 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
  8. 8. Types of spina bifida • Spina bifida occulta • Spina bifida cystica  Meningocele  Myelomeningocele
  9. 9. Spina bifida occulta • Mildest form • Incomplete formation of posterior arch of the spinal column • Usually incidental finding • Seen in LS spine, commonest S1 • Occasionally patient may have fatty deposit, hemangioma or tuft of hair
  10. 10. Spina bifida cystica Two types: • Meningocele- protrusion of only the dura and arachnoid through the defect in the vertebral lamina forming a cystic swelling usually in lumbosacral area • Spinal cord remains in the vertebral canal
  11. 11. Spina bifida cystica Myelomeningocele- • 10 times more frequent • portion of the spinal cord or the nerve roots are displaced through the spina bifida defect into the sac
  12. 12. Incidence • 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
  13. 13. Etiology • Exact cause not known • Proposed theories are : • exposure to fungus Phytophthora infestans by excessive consumption of potatoes • Use of anti seizure drugs(sodium valproate) • Irradiation • Genetic – 6-8% • Couple with one affected infant- risk of subsequent sibling acquiring major CNS malformation is 1 in 14
  14. 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
  15. 15. Associated conditions •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.
  16. 16. Hydrosyringomyelia • Accumulation of CSF in the enlarged central canal of the spinal cord. • Can cause three problems in patients with myelomeningocele • Increase in level of paralysis • Progressive scoiliosis • Weakness of hands and upper extremities
  17. 17. Arnold chiari malformation • Caudal displacement of posterior lobe of cerebellum • 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 problem
  18. 18. 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 urologic function. • MRI evaluation should be performed.
  19. 19. Urological dysfunction • Almost all children with meningomyelocele have bladder dysfunction. • 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 and capacity.
  20. 20. Latex allergy • Incidence 3.8 – 38% • Manifests in form of local rashes or mucosal irritation • Latex skin prick test and assay for latex specific immunoglobulin E in serum • Non exposure to latex is recommended in all patients with meningomyelocele.
  21. 21. Classification –based on level of lesion Thoracic level • 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
  22. 22. 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 Sacral level • Weakness of peroneus and intrinsic muscles of foot • Partial paralysis and insensate skin can lead to foot problems and neurogenic ulcers • Not all patients have one of these classic levels of paralysis.
  23. 23. Multidisciplinary care  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.  Consists of: • 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 spinal orthrosis. • Physical therapist- to aid lower extremity functional assessment, bracing needs and ROM exercises for daily activities. • Nurse to teach about skin care and self catheterisation
  24. 24. • Psychologist to help parents cope with many challenges and stresses related to child’s diability. • 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 support • Experienced neurodevelopmental paediatrician to oversee the whole process. • Finally an orthopaedician to produce a stable posture for sitting or standing.
  25. 25. Orthopaedic management
  26. 26. Orthotic management • 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
  27. 27. 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 be reinstituted.
  28. 28. Braces Ankle- Foot Orthroses • Polypropylene AFO are mainstay of lower extremity bracing • Usually used in sacral level lesions. • Main function is to protect foot and toes during weight bearing and to stabilise the ankle joint.
  29. 29. Knee ankle foot orthroses • Usually used in upper lumbar lesion where quadriceps action is weak. • KAFO consists of AFO component, thigh cuff and knee hinge.
  30. 30. Hip knee ankle foot orthroses • Used in patients with inadequate hip strength or in patients who cannot bear weight with KAFO • Consists of two long leg braces (KAFO) connected by a pelvic band and a free hip joint.
  31. 31. Reciprocating gate orthroses • Sophisticated form of HKAFO • 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 controlateral hip extension allowing energy efficient stride.
  32. 32. • Other mobility aids are standing frame, parapodium, wheel chair.
  33. 33. Surgical management of specific problems
  34. 34. Foot • 75 % of children with myelomeningocele have foot deformities that can seriously limit function. • The main goal of orthopedic treatment is to have a plantigrade, mobile, braceable foot. • Manipulation and casting should be used with caution. • 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 of recurrence.
  35. 35. Calcaneal deformity • Seen in 1/3 rd of children with meningomyelocele. • Level of lesion L5-S1 • Due to active anterior leg muscles and inactive posterior muscles. • Supple deformity manipulation and splinting in neutral position.
  36. 36. • Muscle imbalance simple tenotomy of ankle dorsiflexors and evertors • “Anterolateral release” • tendons of the extensor hallucis longus, extensor digitorum communis ,anterior tibial, peroneus brevis and longus tendons are exicised
  37. 37. • Combined with transfer of tibialis anterior tendon to calcaneum.
  38. 38. Equinus deformity • 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  Open  Percutaneus • Careful postoperative casting for few weeks, followed by fitting of orthroses. • Long flexors must be released if toe deformity presentmay result in pressure sores.
  39. 39. Club foot • Seen in 30% of meningomyelocele. • Irrespective of level of lesion. • Characterised by :  Severe rigidity  Supination varus deformity  Rotation malalignment of calcaneum and talus  Subluxation of calcaneocuboid & talonavicular joint • Recurrence of deformity is common after adequate surgical correction. • Manipulation and casting techniques are difficult due to absence of pain and protective sensation which can lead to fractures and pressure sores.
  40. 40. • Ideal age for surgery : 10-18 months • Radical posteromedial- lateral release through Cincinnati incision. • Naviculectomy • Talectomy • 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.
  41. 41. VO procedure • Verebely- Ogston procedure • Decancellation procedure- removing of cancellous bone from talus and cuboid. • Leaves a hollow shell of bone and more space for correction. • Foot is manipulated into neutral position and because of the collapse of talus and cuboid correction of residual deformity.
  42. 42. Varus deformity • Isolated varus deformity of hind foot is very rare. • 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.
  43. 43. Cavo varus deformity • Occurs in sacral level lesion. • Cavus is the primary deformity which produces varus. • Rigidity of the varus deformity should be determined before planning for surgery. • Supple deformity radical plantar release without hind foot bone surgery is done.
  44. 44. Radical plantar release • Release of the plantar aponeurosis, abductor hallucis, short flexors of calcaneum is done.
  45. 45. • If the deformity is rigid despite radical plantar release, closing wedge osteotomy is done. • Triple arthrodesis rarely indicated as a salvage procedure. • Subtalar, calcaneocuboid and talonavicular joint are fused.
  46. 46. Supination deformity • Occurs in L5-S1 level lesion. • Mainly due to unopposed action of anterior tibial muscle. • Supple deformity simple tenotomy of anterior tibial tendon. • 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 metatarsal. • 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.
  47. 47. 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.
  48. 48. 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 calcaneum.
  49. 49. Vertical talus • Seen in 10% children with meningomyelocele. • Characterised by malalignment of hind foot and mid foot. • Two types:Developmental and Congenital Both requires surgical correction once the deformity is partially corrected by manipulation and serial casting. • 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.
  50. 50. 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.
  51. 51. Toe deformity • 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.
  52. 52. Extensor hallucis longus tendon is attached to neck of first metatarsal; interphalangeal joint is arthrodesed and fixed by medullary wire and by suturing distal end of extensor hallucis longus tendon to soft tissues over proximal phalanx.
  53. 53. Ankle  valgus deformity • 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 orthroses. • To evaluate valgus ankle deformity, 4 factors must be determined. 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.
  54. 54. • Operative treatment is indicated when ankle valgus deformity causes problems with orthrotic fitting and cannot be relieved by orthroses. • Tenodesis of tendoachilles to fibula above the physis if the valgus tilt is between 10-25 degrees. Valgus deformity is due to lateral compartment paralysis, so underdevelopment of fibula will be present, pulling down the fibula during weight bearing and dorsiflexion
  55. 55. Hemiepiphysiodesis of distal tibial epiphysis • Done in skeletally immature patients. • Medial tibial physis is closed by direct surgical ablation or stapling or using maleolar screws. • Growth arrest 0f the medial side combined with continued growth in the lateral side corrects the deformity. • Advantage is immediate weight bearing.
  56. 56. Supramalleolar varus derotation osteotomy • Recommended in children older than 10 years. • 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.
  57. 57. Distal 1/3rd fibular oblique osteotomy is done followed by medial based wedge osteotomy above the epiphysis. Size of the wedge depends on valgus to be corrected. Fixation with plate and screws
  58. 58. Knee Flexion contractures • 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 equinus; 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 syndrome; o hip flexion contracture or calcaneal deformity in the ambulatory patient. Knee flexion contractures
  59. 59. • 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
  60. 60. Radical flexor release Minimal procedure – partial division of
  61. 61. supracondylar extention osteotomy of femur
  62. 62. Extention contractures • Due to unopposed quadriceps action • Serial casting successful in some patients • Surgical management consist of oV-Y quardriceps lengthening oCapsular release oPostrerior displacement of the hamstring muscle
  63. 63. 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 position.
  64. 64. Valgus or varus deformity • Supracondylar or tibial osteotomy with internal fixation can be done • Hemiepiphysiodesis can be done using staples if recoganised early
  65. 65. Hip deformities • Mainly due to: ▫ Muscle imbalance ▫ Congenital dysplasia ▫ Habitual posture
  66. 66. Flexion contracture • 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 osteotomy.
  67. 67. Flexion abduction external rotation contracture • 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.
  68. 68. External rotation contracture • Seen in low lumbar lesions. • Initially bracing & physiotherapy. • If persists subtrochanteric medial rotation osteotomy.
  69. 69. Abduction contracture • Seen in high level lesions. • Fascial release indicated when abduction contracture causes pelvic obliquity & scoliosis which inteferes with function or bracing. • Fascial release fascia over gluteus medius, minimus & tensor fascia lata is divided and fasciotomy done.
  70. 70. Adduction contracture • Seen in high level lesions. • Due to spasticity and contracture of adductor muscles. • Surgery indicated when it causes pelvic obliquity & inteferes with sitting & walking. • Adductor release done.
  71. 71. Hip subluxation/dislocation • Seen in 50% of children with meningomyelocele. • Three types: • Developmental treated by Pavlik harness, traction, closed reduction & hip spica application. • Teratological seen in high level lesions, initially acetabulum dyplastic, with head of femur displaced proximally, should not be treated initially.
  72. 72. Paralytic • Most common type- 50-70% • Seen in low level lesions L3-L4. • Due to imbalance between adduction & abduction forces. • Principles of treatment: • Obtain concentric reduction • Correct bony abnormality • Seek to balance flexor-adductor & extensor- abductor imbalance. • Main goal – maximal function rather than radiograph reduction.
  73. 73. Reduction • Controversial • Should be atttempted only where child will derive considerable benefit from the procedure. • Not always successful stiff painful hip • If required anterior open reduction with capsulorrhaphy.
  74. 74. Correction of bony abnormality • Femoral deformity corrected by proximal femoral resection & inter position arthroplasty. • Done in severely retarded multiply handicapped with dislocated hips and severe adduction contraction.
  75. 75. • 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 paralytic dislocation.
  76. 76. 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 medially
  77. 77. Muscle balancing procedures •Simple release of ilioposas tendon with adductor release Adductor longus tendon is sepereted from brevis and exiced Adductor brevis is separated from pectineus and lesser trochanter Identified ilioposas released
  78. 78. • 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.
  79. 79. • Sharrard procedure: • 1959, posterior transfer of iliopsoas tendon. • Transfer of iliopsoas muscle through a hole in the ilium underneath the femoral nerve with reinsertion of the tendon to the posterior greater trochanter through a drill hole and reattachment of iliacus portionto the outer wall of the ilium.
  80. 80. • Transfer of external oblique muscle: • Thomas and colleagues described transfer of external oblique abdominis to greater trochanter. • Modified by Phillips and Lindseth adductors, external oblique & tensor fascia lata transferred. • Adductors sutured to ischium. • External oblique with aponeurosis to greater trochanter. • TFL rotated externally and sutured to iliac crest proximally and tendon of gluteus distally • mcKay described tendon transfer with femoral osteotomy.
  81. 81. Pelvic obliquity • Common • 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 position • Suprapelvic—scoliosis by orthoses and spinal fusion • Pelvic--- obliquty is 20 degree pelvic osteotomy is done • Upto 40 degree gets corrected
  82. 82. • Pelvic osteotomy Preoperative determination of size of iliac wedge to be removed and transferred. , Exposure of ilium. , After bilateral osteotomies and removal of wedge from low side, deformity is corrected. , Transferred iliac wedge is fixed with two Kirschner wires.
  83. 83. Spinal deformities • 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 or mixed. • Congenital spinal deformities are managed as in any other patient. • 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- 12 years. • Non operative treatment is not usually successful.
  84. 84. • 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 infections.
  85. 85. Scoliosis • 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°.
  86. 86. • 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 procedures. • Anterior procedure is done initially. • Anterior release with discectomy & intervertebral fusion • Anterior release with strut grafting • Anterior fusion • Correction with Zielke instrumentation.
  87. 87. • 2nd stage posterior procedure is done 7 days after the anterior procedure. • Choice of instrumentation includes: ▫ Square ended ▫ Contoured ▫ Harrington rods with sacral hooks ▫ Spinal instrumentation.
  88. 88. Kyphosis • 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.
  89. 89. Hyperlordosis • Less common deformity • Can lead to difficulty in sitting, intertriginous skin breakdown, difficulty with self catheterisation in females because of posterior rotation of perineum. • Treatment by combination of anterior and posterior spinal release and posterior instrumentation. • In rigid deformities, postural reduction in traction after spinal release, before definitive instrumentation may improve the deformity.
  90. 90. Hemimyelodysplasia • 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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