Congenital scoliosis البروفيسور فريح ابوحسان- استشاري جراحة العمود الفقري - الجنف الخلقي

Professor of Orthopaedicsp
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Professor Freih Abuhassan -
University of Jordan

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Congenital Scoliosisg
Classification:
According to the area of the spine
ff t daffected
– Cervical cervico-thoracicCervical, cervico-thoracic,
– Thoracic
– Lumbar
– Lumbo-sacral spine
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According to the pattern of deformity
Kyphoscoliosis–Kyphoscoliosis,
– Lordoscoliosis
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According to the basic type ofAccording to the basic type of
malformation
- Failure of formation
F il f t ti- Failure of segmentation
- Combination of the above (scramble eggs)( gg )
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Failure of formation.Failure of formation.
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F il f t tiFailure of segmentation
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Cervical spineCervical spine
= Klipple-Feil Syn.
= Sprengel’s def= Sprengel’s def.
Scramble eggs
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Questions
Is it hereditary?
No. Isolated hemivertebra carries noNo. Isolated hemivertebra carries no
risk to subsequent siblings.
What is the best Treatment?
Early evaluation.
Early aggressive treatmentEarly aggressive treatment.
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Factors affecting the progressionFactors affecting the progression
1- Specific anomalies.
A- unilateral unsegmented Bar. worst progression.
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B- Single hemivertebra or double unbalanced
Hemivertebra Progress slowely.
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2-Area of affected spine2 Area of affected spine
1- Cervico thoracic and lumbar less progressive
2- Thoracic curve progressivep g
3- Thoracolumbar curve progressive.
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Look for other anomaliesLook for other anomalies
1- Spina bifida
2-Neurological defeciet
e.g small size of the foote.g small size of the foot
3-Spinal dysraphism
4 O h li4-Other anomalies
e.g heart & kidney, facial asymmetry,g y, y y,
sprengle’s
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Diagnosis by SonogramDiagnosis by Sonogram
Can be done before birth
C b d t bi th ftCan be done at birth or soon after
Clinical diagnosis
DeformityDeformity
Associated congenital problems.
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Radiographic diagnosisRadiographic diagnosis
1 Si l PA d L t X1-Simple PA and Lat. X- rays.
2-CT scan with 3D reconstruction
3-Myelography
4-MRI4 MRI
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ManagmentManagment
C t b (Mil k )
Conservative
Cast or brace (Milwaukee)
Indications
A- Flexible long curve
B- Skeletal immaturityy
- Control compansatory curve
N id i ff i h i- No evidance in affection the prognosis
- Can be fitted to 2 years old child.
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W hi i l li iWatching congenital scoliosis
grow is not the solution.g
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Surgical treatmentSurgical treatment
1- Insitu ant. and post fusion
In minimal to moderate deformity
2- Ant and post unilateral epiphseodesis2 Ant. and post unilateral epiphseodesis
on the convex side.
3 St d ti f h f ll d3- Staged correction of the curve followed
by fusion
4- excision of the hemivertebra
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Post-operative radiographs
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After anterior-posterior resection, fusion
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VERTEBRAL EXCISIONVERTEBRAL EXCISION
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Posterior “in situ” fusion sets the stage for “Crank
Shaft Phenomenon”
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Post-operative correctionp
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SPINA BIFIDASPINA BIFIDA
S i Bifid f i l lSpina Bifida refers to incomplete closure
of the laminar arches of the spine.p
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GeneticsGenetics
not known.
However, the risk of occurrence in
first degree relatives is slightlyfirst-degree relatives is slightly
increased 3.2%.
The incidence of spina bifida occulta
i 2 3%is 2-3%.
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SignsSigns
Signs-local:
1 Di l h i h1- Dimple to hairy patch.
2- Vascular marking.g
3- Fatty mass (lipomeningocele).
4 E f th i4- Exposure of the meninges
(myelomeningocele)( y g )
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Signs-distant
1-Motor weakness,
2 Atrophy of calf or thigh2-Atrophy of calf or thigh
3-Neurogenic bladder.g
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ClassificationsClassifications
1 Si l ( lt )1- Simple (occulta)
At the L5, S1 with no neurologic deficit.At the L5, S1 with no neurologic deficit.
The only associated problem is a slightly
i d i k f d l li th iincreased risk of spondylolisthesis.
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2-Myelomeningocele:2-Myelomeningocele:
Bony defect, usually involving severaly y g
missing laminae, with exposed meninges
and usually some neurologic deficit atand usually some neurologic deficit at
the same level.
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3 Li i l3-Lipomeningocele
Caudal fatty mass arising from spinalCaudal fatty mass arising from spinal
canal, palpable under the skin, with
i t d l i d fi it b tassociated neurologic deficit but no
significant risk of hydrocephalusg y p
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Lipomeningocele
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CausesCauses
1 Unknown1- Unknown.
Involves either failure of closure of the
neural tube or its late rupture.
2 Folate def2- Folate def.
3- Congenital defect
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Overview of CareOverview of Care
At birth the child should be seen byAt birth, the child should be seen by
1-Neurologist,
2- Neurosurgeon,
3 Orthopaedic surgeon3-Orthopaedic surgeon
4- Urologist .g
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Also:Also:
= Latex exposure should be avoided.
= Genetic counseling should be offered
to the familyto the family
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SurgerySurgery
Clubfoot surgeryClubfoot surgery
Lengthen tendons and realign bones to
create a foot which will be flat on the ground.
Spine surgerySpine surgery
indicated if unbalanced and impairing
sitting: straighten and fuse spine using
implanted rods.p
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G l MGeneral Measures
1-Monitor motor strength and sensory
l l d d th h t lif i dlevel and record throughout life in order
to detect tethering or other complication.g p
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General MeasuresGeneral Measures
2-Treat other deformities by stretching ,
bracing or surgery.
3 Teach family how to protect skin3-Teach family how to protect skin.
4-Hip subluxation do not need surgery:
especially if high and bilateral in aespecially if high and bilateral in a
nonambulator
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Complications of Spina bifidaComplications of Spina bifida
1-Cord tether at site of openingp g
causing weakness with growth.
2 Fracture risk is higher with higher2-Fracture- risk is higher with higher
neurologic deficit.
Signs include low-grade fever, swelling,
and warmth without much painand warmth without much pain.
3-Pressure sore over insensate skin,
especially of ischium, foot or trochanter.
4-Renal failure due to poor self-care.4 Renal failure due to poor self care.
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NEUROMUSCULAR SCOLIOSISNEUROMUSCULAR SCOLIOSIS
Neuromuscular diseases are a group ofNeuromuscular diseases are a group of
disorders characterized
lack of normal function of the brain,
i l d i h lspinal cord, peripheral nerves,
neuromuscular junctions, or muscles.neuromuscular junctions, or muscles.
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Classification of neuromuscular spinal
deformity
NEUROPATHIC
UMNL
Cerebral palsyp y
Friedreich ataxia
Charcot-Marie-ToothCharcot-Marie-Tooth
Syringomyelia
S i l d tSpinal cord tumor
Spinal cord trauma
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deformity
LMNL
Poliomyelitis
Traumatic
Spinal muscle atrophy
Werdnig-HoffmannWerdnig Hoffmann
Kugelberg-Welander
Dysautonomia (Riley Day syndrome)Dysautonomia (Riley-Day syndrome)
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deformity
MYOPATHIC
Arthrogryposis
Muscular dystrophyMuscular dystrophy
Duchenne
Limb girdleLimb-girdle
Facio-scapulo-humeral
C it l h t iCongenital hypotonia
Myotonia dystrophica
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Neuromuscular curves
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Pelvic obliquityPelvic obliquity
1-Loss of sitting balanceg
2-Ribs impinge on the iliac crest
3-Decubitus ulcers over the ischium3 Decubitus ulcers over the ischium
4-Progressive pulmonary deficiet due to
chest deformitychest deformity
5-Hip contracture, subluxation, or dislocation
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Syringomyelia
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TreatmentTreatment
Brace is not effective at all.
Operation if the curve > 25 degree
If not involving the sacrum
i i itreat as idiopathic
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If involving the sacrum pelvic obliquityIf involving the sacrum pelvic obliquity
Fuse all the spine
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Congenital scoliosis البروفيسور فريح ابوحسان- استشاري جراحة العمود الفقري - الجنف الخلقي

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Congenital scoliosis البروفيسور فريح ابوحسان- استشاري جراحة العمود الفقري - الجنف الخلقي