General Algorithm 1 for Hip Examination and Discussion on condition involving spine and LE

Hip Examination
Attitude
CDH
Broadening at Trochanteric region
Widening of perineum
Asymmetry or duplication of gluteal folds
(P. Kilisc termed DDH. )
Synovitis of Hip joint
            Mild FABER with lengthening
Arthritis
            F AD IR +/- shortening
Pure posterior dislcatoins
            F AD IR with apparent and true  shortening
Anterior Dislocation
            F AB ER with apparent lengthening –low type
            Marked ER + Full extension – High type
Trochanteric Fracture
            Marked ER outer part of foot touching bed –characteristic
Neck of Femur Fracture
            ER but not marked due to catch in capsule due to irritation by fracture end
            Late cases –variable flexion and adduction ,may be superadded except when patient ;has managed to walk even with abduction.
Inspection – Front , Side and Back



Fixed Bony Points
Soft tissue
Abnormal finding
Front
ASIS,Pubic Symphysis
Iliac Fossae , Inguinal ligament , Groin fold, Femoral Triangle (Scarps’s triangle ),front of thigh
Muscle wasting , Sinuses , Swelling , Obvious Pulsation , Level of ASIS,Abnormal skin condition
Side
Iliac Crest , Torachanters
Gluteal Bulge ,Supratrochanteric depression , Infractrochanteric depression , Lateral thigh muscle mass
Level of tip of greater trochanters in relation to ASIS
Back
Back of iliac Crest , PSIS (Dimple of Venus ) ,Ischial tuberosity
Gluteal bulges,Gluteal fods,back of thigh
Muscle wasting , Any swelling , Scars,Sinuses , Abnormal skin condition ,Contracture



Palpation of skin
Temperature , Skin – Smooth /Rough , Senstion , Sharp bony promineces

Deep
Pulsation , tenderness at iliac fossae ,
Anteriorly – Just below and lateral to mid inguinal point
Laterally – Over greater trochanter,
Direct pressure
Thrust pressure
            Points towards hip joint



Palpation to confirm findings of inspection

Touch tenderness – Fresh GT fracture , Inflammation at that area
Deep Pressure –Healing trochanteric fracture , trochanteric bursitits , ,fracture Neck of femur
Thrust Tenderness - Transmitted tenderness in NOF # , Fracture acetabulum , TB Hip , Characteristic inflammation at hip

Posteriorly – Centre – Line joining trochanteric tip to ischial tuberostiy
                        Centre – line joining ischial tuberosity to PSIS
Iliac Fossa – Base of ilica fossa infection
Medially – junction of groin with medial aspcet of thigh

Site of cold abscess
Base of Scarpa’s triangle
Gluteal region
Supratrochanteric region
Iliac Fossa
Anteromedial aspect of mid thigh even upto knee joint in that line

Lypmh Nodes
Movement
            Flexion /Extension
            Abduction /Adduction
Lower limb axis –mid inguinal point (ASIS practically ) , mid patella ,mid ankle ,2nd web space
            ER / IR
                        Patella horizontal , Great toe vertical upwards
            Fixed Deformities
            Flexion , Abduciton / Adduction , IR or ER
            Combination
                                    FADIR
                                    FABER
                                    FADER
            Cause
                        Persistent muscle spasm , posture to avoid pain or to conceal deformity
LLD , Destruction of joints,fibrotic contractures in periarticular soft tissue and surgical intervention leading to fixed deformity
(Limb can’t be brought to neutral positon but movement in same axis is possible )

Pathomechanics of Deformities
1)Normal ROM – Beyond that ligament holding hip joint don’t allow hip to move. Rather femur and pelvis move together.
2)If joint fixed in a postion (Abduction) , opposite motion is not possible (Adduction)
3)While testing , fix pelvis , when it moves, stop and bring limb back to just short of motion.Measure from zero position .
4)Compensation
To conceal deformity
Maintain equilibrium shifting ‘g’
Make up LLD
Stabilize unstable hip
            ER/IR
            First in extension then in flexion of knee

            Measurements
            LLD
                        Shortening Compensation
                                    -ASIS lower down – pelvis tilt
                                    -Equinus
                                    -Flexing opposite knee and hip
            Apparent Measurement
                        Assesses Extent of compensation of deformity , LLD
                        Method
Patient supine , comfortable posture ,affected limb in line of trunk ,Lower limbs parallel
Handle unaffected limbs to make it parallel
B/L affected cases to , apparent measurement not of much significance

Reference – Suprasternal notch , xiphisternum ,umbilicus,sharp bony point of medial malleolus

True LLD Measurement
ASIS to tip of Medial Malleolus while both LL in identical postion and pelvis squared,
Standing Position
            Gradually adduct limb, then patient raises heel where ASIS b/l at same level ,insert measured wooden block to get ASIS at level hieight of block equals LLD

Lying Position
Pre-requisites
Fully exposed
Bony points – mark with a pencil
ASIS , medial central or lateral central point of knee joint line (or tibial flare) , MM –inferoposterior portion
Sharp points on posterosuperior aspect of GT ,sharpest point on ischial tuberostiy
            Marked by flexing hip and knee joints at 90 degrees.
Square up pelvis , exagerrate noted deformity.
Limbs in identical position ,normal limb handled to make it identical to affected limb.
Use metal ends localizing bony points.
ASIS –slide from inguinal ligaments from Pubic Tubercle. First resistance is ASIS.
GT-Slide over gluteal medius till obstructed by a sharp bony resistance ,Make it or go above shaft.

Total Lenghts
Galeazi test – Hip at 60 degrees and Knee at 90 degrees , see Tibial tuberosity and if Femur are parellel.
Femur Parallel ,one knee receeding – Shortening from Femur
Femur not parallel and both tibial tuberosities at same level , shortening from Tibia
                        True LLD = Apparent Shortening
It Means          -                       No compensation


                        True Shortening > Apparent Shortening 
It Means          -                       part of shortening is compensated
                                                                                                 (Fixed Abduciton deformity )

                        True shortening < Apparent Shortening
It Means          -                       fixed adduction deformity

Shortening can be from Tibia and Femur. Galezi discerns shortening from tibia or femur. Then find out supratrochanteric or infratrochanteric femoral shortening .
            Supratrochanteric Shortening
            Square Pelvis Draw 3 lines connecting ASIS , GT , Vertical Imaginary line falling from ASIS perpendicular to line along shaft of femur and GT .








Interpretation
Any shortening at base suggests
            Riding up of trochanter may be due to shortening in Neck,Head or joint dislocation

Reverse of Bryant’s triangle suggest
            Trochanter going above the vertical line .
True Supratrochanteric shortening = Base of Reverse Bryant’s triangle + Base of
Opposite Braynt’s triangle
Shortening of Perpendicular Line
            Anterior sliding or tilting, IR of Greater trochanter / Head of Femur
            E.g. Posterior and Central Dislocation of Hip joint
            Flexion Contracture of hip following trochanteric fracture , distructive lesion of hip joint,
Shortening of Hypotenuse
            Approximation of trochanter towards central point of body
Central Dislocation , Old Neck of Femur Fracture, Neck Absorption,Protusion of Acetabulum ,Absent Head


Fallacies
B/l Involvement
Post Bone Grafting
Disarticulated Hip
Quantitative measurement 


Chiene's Test - Lines along GT and ASIS are parallel.




Morris Bitrochanteric test
GT tip to Sypmhysis pubis are bilaterally equal.

Schoemaker’s Line
GT to ASIS lines if projected meet at or above umbilicus.

B/L hip affections –the measurement are inconclusive.
Circumferential Measurement
Thigh – 15 cm above patella upper pole

Special Tests
SLRT
If acetabulum joint space ,head , neck and rest of Lower Limb are normal , patient can easily rasie leg upto 80-90 degrees.

Telescopic Test
Supine position ,knee and hip flexion 90 degrees , Put right hand at lower end of femur , pull and push.
Other hand palpate trochanter , putting thumb at ASIS , feel excursion .
Trendelenburg test
Ortolani’s test
Barlow’s test
Gauvin’s sign
Narath’s sign
Criag test
Galeazi test
 Ely’s test
Noble Compression test
Hart’s Sign – Limitation of Abduction seen in DDH.
Erichson’s Test - When iliac bone sharply compressed towards each other , pain in SI joint.
Flexibility of Hamstrings – POP angle < 20 degrees .
Per Rectal Examination
            Central fracture dislocation , fracture floor of acetabulum , Protusion Acetabulum
Peripheral Pulese ,Nerves
Sciatic Function- PF ,DF Ankle

Investigations
X-ray – AP –Shenton’s arc
            Capital Physis to neck
            Capital physis to acetabulum cup
            Lateral
Oblique projection – Central Fracture dislocation
Special Views .
Old Neck of Femur Fracture – 15 degrees abduction and 15 degrees IR to neutralize anteversion ,assess length of neck
Coxa Plana or LCPD
Keep hip movement abducted and IR – gives an assessment of containment of flattened in acetabulum.

Other Investigations
CT –Intervals to determine history of LCPD and progress in revascularization of necrosed femoral head and further to know about fate of ostochondral fragments in LCPD.
Aspiration
Biopsy
USG

           


            Fixed Flexion Deformities
Thomas Test
Note – Stop normal hip flexion when compensatory lordosis obliterates.
Criticism
Patient may be hurted in painful hip.
Obese /heavy patients may not be accurate .
B/L FFD – Difficult to perform
Anterior tilting of pelvis – inaccuracy
Ankylosed knee in extension causes difficulty.
Alternatives –
B/L contracture – test in prone position .Make angle of straight back with the thighs.
One side Knee contracture – bring patient in edge of table to perform the test.

Fixed Abduction Deformity
ASIS – at lower level
To measure , affected limb abducted till ipsilateral ASIS are in same horizontal line to that of opposite side .
In this position draw a vertical line (through Midbody or ASIS)
Anlge between this line and the limb is Fixed Abduction.

Alternative




Draw two lines perpendicular to Midline from ASIS and measure the angle .α is fixed abduction angle in above case .
Abduction is to compensate the short leg. 10 degrees abduction gives 1 cm gain in length.

Fixed Rotation Deformities
Normal Movements
Flexion /Extension
Abduction – Abduciton in flexion and extension
Normal – 70 degrees in adults.
Decreased in CDH,TB Hip , Perthes diseases.
Adduction – Normally crosses distal third of opposite limb.



Elisa Magar                  10 years Female                       Udayapur
Grade IV student
C/C – Limping on walking and shortening of left leg for 9 years
HOPI – Limping on walking noticed since age of walking .
Limping  not progressive , Associated with shortening of leg on left side.Shortening is progressively increasing.
On Movements
Sitting is Unaffected but
 She falls frequently while walking .
When she lies on ipsilateral side it is painful which is mild in intensity.

Functions
            No difficulty on wearing shoes , dresses
            Squatting is difficulty
            Walks only aout 100 metres
           
Trauma, fever , infections on body , no other constitutional symptoms.

Family and Socioeconoimc History
            Only daughter in family
Birth and Develoment history
            CS delivery at 10 /12 , Patan Hospital , Lalitpur Nepal
            Mother had taken regular Folic Acid , Iron and Calcium supplement during pregnancy
            Sitting – 10/12
            Growing – 12/12
            Babling – 12/12
            Walking – 2 years
Vaccination
            Complete as per Expanded Programme for Immunization schedule
Expectation
            Treat shortening , deformity of back .
Physcial Examination
G.C. Healthy looking , Well oriented to time plae and person
Conscious well oriented to time place and person
Co-operative
Not in distress
Build up good with BMI -
Sitting comfortably on bed
Pallor - , Icteric - , Lymph nodes – not palpable , cyanosis - , clubbing –
BP                    P – 109 / min               T – 98 degrees F          R

Systemic
Skin – Normal             Scalp – Normal hair colour and distribution
HEENT
Chest – b/l VBS with equal air entry with no added sounds
CVS – S1S2 MO
P/A – Soft  , non-tender with no any organomegaly
Musculoskeletal System
            Gait – Short leg gait with poor balance and tendency to fall
            Moves upper extremity rapidly to balance
            Knee on flexion on rt side
            Fast swing and stance phase
            Squat
            Inspection from fron side and back
            LLD , Rt leg shorter
            Thigh and calf wasting
            b/l forefoot adducted,
            ulcer on rt 5th toe base

Palpation
            Superficially – Temperature normal ,No soft tissue tenderness
            Deep – No soft tissue and bony tenderness
            Globular shape bony prominence lateral to ASIS
            Vascular sign of Narath’s sign +
Movement
Flexion /Extension only 70 degrees on affected side (Rt)
IR – 75/ 70 degrees

Knee /Ankle /Toes – Normal movements

Measurements
True – 9 cm LLD ,Supratrochanteric – 4 cm
                              Infratrochanteric – 2 cm
                             Tibia –                      3 cm
Wasting – Thigh -  3 cm
                  Calf – 2 cm
Neurological Examination
            HMF – Speech and cmmunication
                        Gait and Memory
                        Cranial Nerves – not tested
                       
Sensory
            L5 region – Absent
            S1             - Impaired
           
Motor
Bulk – Decreased on left side
Tone – Normal
Power – Normal

                                    Rt

Hip      Flexor                          3
            Extensor                      1
            Abductor                     2
            Adductor                     2
Knee    Flexor                          2
            Extensor                      3

Ankle   Flexor                                     0
            Extensors                     0
            Evertors                       0
            Invertors                      0


Toes
            Extensors                     0
            Flexors                                     0









Reflexes
            Superficial
                        Cremasteric - only in male child
                        Abdominal
                        Babinski’s –     Mute
            Deep
                        Knee
                        Ankle -            Absent

Special Tests
            Trendelenburg test
Telescopic Test


Examine the back as well. 

 


Above condition was the Hip Dislocation with Spina Bifida. Let's discuss about different conditions of spine related to this . 



Spina Bifida
1/200 in USA
Aetiology
 Unknown
Higher incidence in first born children , certain races and lower socioeconomic states
Theories
-Recessive inheritance with less than 1:4 ratio due to early fetal loss
-Environmental factors (Von-Recklinghausen )
-Hamartomatous growth
                                   
Association
-Low maternal folic acid uptake
-Maternal IDDM
-Maternal hyperthermia
Valporic acid
Classification
Spina Bifida Occulta
Incomplete closure of laminae without cystic distention of meninges
Spina Bifida Cystica
Meningocele –Cystic distention of meninges but no neurological loss (6 % of cases )
Myelomeningocele – Cystic distention of meninges with neurological loss (94 %), usually at lumbar or lumbosacral junction

Clinically determined by lowest active motor control .
Upper Thoracic
Lower Thoracic
Upper Lumbar
Lower Lumbar
Sacral

Flaccid Vs Spastic Paralysis
 Patients with L 4 functions have good chance of functional walking (Household or Community )
With L 5 Function – Good Chance of community walkers

Spina Bifida Occulta  (Spinal Dysraphism)
L5 /S1 and Cervical Axis most common .
Although skin intact , almost never normal as some midline anomaly suggest diagnosis.
Bulge ,tiny dimple , Pigmented Naevus , Faun’s tail
Neurological defect – occurs in a small percentage and depends on level and extent of  cord involvement

Tethetered Cord Syndrome  - Diastomatomyelia
Spina Bifida Cystica (Myelocele ,  Myelomeningocele )
Posture tells neurological level
LL Paralysis – complete or incomplete
85 % - Paraplegic foot deformity
Hip dislocation , Genu Recurvatum , Flexion Contracutre
 Genu Recurvatum +/- Talipes and claw toes
Scoliosis – 80 % of thoracic and high lumbar lesions
Ansesthetic Skin – leading to pressure sores
Lower Urinary tract dysfunction leading to renal failure
Latex Insensitivity in 60  %  due to surgical procedures multiple times , catheters ,shoes
Do surgery in latex free environment
 Hip Dislocation in 50 % cases of Spina Bifida
Vertical Talus
Other Vertebral Anomalies
Hemivertebrae,Abnormal bars , Uncommon (15-20%)
Mental Retardation
Developmental Delay
Bone Fragility , Pathologic Fracture
Upper Limb weaknesses and spasticity due to Arnold –Chiari Malformation
Syringomyelia leading to upper limb dysfunction
Only 30 % Patients with Mylomeningocele are functionally independent
29 % lack B/B control
Lipomeningocele
Defect contains lipoma that is involved with sacral nerves.

Diastomatomyelia
Osseous,Cartilaginous ,fibrous septum in central portion of spinal canal leads to complete or incomplete sagittal division of spinal cord into Two hemicords.
Upper lumbar levels involved.
F > M

Tethered Cord Sydrome
May recur after repair of myelomeningocele
Incidence of tethering 11-20 %
Occurs at 6-11 years.
Back , buttock , lower extremity pain , progressive motor deterioration , progressive foot deformities, rapid increase in severity of lordosis or scoliosis
Flaccid vs Spastic Paralysis\
Patients with  L 4 function have good chance of functional walking (household or community )
With L 5 function have good chance of community walkers
Diagnosis
MRI with Contrast
            R/O associated dermoid  cyst
Treatment
            Early release stops progression of spine deformity relieves pain

HydRocephalus
            90 % Require VP shunt
            Acute –bulging of fontanelle ,altered mental status,head-ache , Syrinx formation
            Scolisosis,,motor deterioration
Arnold-Chialri Malformation
            95 % children with myelomeningocele
            Herniation of posterior fossa structures (Cerebellum,brainstem ) through the foramen magnum
            Leading cause of death in infants
            Some deformities persist /progress and require surgical decompression
X-ray
            Gaps in neural arch
            Widening of spinal canal
            Bony spur of diastomatomyelia
            Other spinal deformities
                        Bars
                        Hemivertebra
                        Fused Ribs
                        Scoliosis
                        Lordosis
                        Kyphosis
Pathology
Failure of development of neural arch tube from arch at 4/52
Membrane or cord prolapsed
Skin over defect –Open /Close
Closed Defects associated with Dermal Cysts
                                                Lipoma of Cauda Equina
                                                Diastomatomyelia
                                                Partial or complete absence of sacrum / meningocele
Meningocele contains thecal sacs , no neural elements
Myelomeningocele – contains nerve roots and cord remnants adherent to sac protrude
Inv
New onset spasticity , progressive scoliosis –evaluate for cord tethering ,hydromelia and shunt function
Treatment
Team approach including paediatrician
Counseling
May elect not to treat for high lesion
Surgery
Hydrocephalus , tethered cord , bladder dysfunction
Forcible correction l- cant do  because bone are weak , skin is paretic.
Surgical Correction
After child at least several months old. Sequence of correction – Hip , Foot , Knee
High complication rate
            Infection , Pathological fracture, Pseudoarthrosis

Surgery to correct spine and limb deformities will be more rewarding when neurologically stable and free of UTI .
Treatment
Hip – Dislocation , common in L3 -4
Primary cause
            Paralysis – Abductors and Extensors with power in Adductor and Flexor
            Unilateral – open reduction , B/l – some recommend conservative treatment (controversial)


Open Reduction , tenotomy , or muscle transfer may be needed to reduce a dislocated hip.
Early Correction –avoids late bony changes .
Aim – Stiff enough to support on extension and flexible enough to support on sitting
Iliopsoas Transefer (Sharrad ) : Pass tendon through a hole in pelvis to GT to redirect pull of flexors.

Knee
Patients have Quadriceps weakness requiring KAFO.
Genu Vlagum
            Release of contracted Iliotibial band
Flexion deformity – release hamstring if not corrected by flexing FFD of hip. May  need extensive release of knee structures or Extension osteotomy
Genu Recurvatum
            May be due to Foot Equinus
Tibia
            Torsional deformities – secondary to muscle imbalance , Treratment – Osteotomy

Foot
            Goal – Platigrade foot with adequate  plantar skin , suitable for bracing
            Soft tissue procedure at 6 months.
Deformities include
            Equinus – Posterior Release
            Equinovarus – (Most common) – PMR
            Calcaneus – TA transfer to Os Calcis
            Valgus – Tendon transfer or subtalar arthrodesis
            Cavus – Plantar release , metatarsal +/- os calcis osteotomies
            Vertical talus –            
            Club foot – Most are rigid deformities requiring surgical correction
            Fixed Deformities
                        Bony procedures eg. TA ,Grice Arthrodesis , Dwyer Procedure

Spine
Scolisosis
Most common skeletal deformity in Myelomeningocele ,occurs in 80 %
Thoracic , Upper Lumbar most common
Lumbosacral
Rapidly progressing present
            MRI to r/o Tethered cord and neurosurgical evaluation for shunt
Causes
            Congenital Spinal Malformation
            Unequal Growth
            Embryological Causes
                        Failure of formation
                        Failure of segmentation
            Rigid Scoliosis
            Mechanical instability and paralysis due to absent cement

           

















Tethered cord and Hydromelia causes Neurological abnormalities. Surgical correction
                        High Pseudoarthrosis
                        Infection rate upto 25 %
Kyphosis
            15 % patients  
            Often severe with breathing and eating difficulty
Types
            Apex – L1 and L2
            Rigid lordosis in proximal adjacent lower dorsal spine.
                        Treatment – Excision of 1 or 2 vertebrae proximal to Apex
            Collapsing
                        Due to muscle imbalance
                        Corrected by manual pressure
                        Not associated with proximal lordosis
                        Aim – to avoid surgery as long as possible to allow for long spinal growth
                        Care with braces
Lordosis
            Excess lumbar lordosis due to other problems eg. FFD
            Tethered cord – more common in children with lesion at L4 -5
            Scoliosis at young age s/o tethering
            Treatment -      Khyphectomy
Early embryology
            Weak 1-2 – Zygote Formation , Bilaminar Embryo
            Weak 3-  8- Embryological period
            Weak 9-38 – Fetal Period

Development of Somite (3/52)
            Mesoderm forms paraxial mesoderm which differentiates into Somites on both the sides

            Somites
            Form Axial skeleton , vertebrae, Muscles
            Form in 20-30 days ,
            42-44 pairs , used to calculate age of embryo
            Differentiate into
                                    Ventromedial Sclerotome forming  Vertebrae and ribs
                                    Myotome forming muscles
                                    Dermatome forming skin
            At 4 weeks neural tube formed open in caudad and rostral ends.

           
                       
The above case was a  Spina Bifida with Hip Dislocation .
Discussion on Different conditions of Spine

 Spina Bifida
1/200 in USA
Aetiology
 Unknown
Higher incidence in first born children , certain races and lower socioeconomic states
Theories
-Recessive inheritance with less than 1:4 ratio due to early fetal loss
-Environmental factors (Von-Recklinghausen )
-Hamartomatous growth
                                   
Association
-Low maternal folic acid uptake
-Maternal IDDM
-Maternal hyperthermia
Valporic acid
Classification
Spina Bifida Occulta
Incomplete closure of laminae without cystic distention of meninges
Spina Bifida Cystica
Meningocele –Cystic distention of meninges but no neurological loss (6 % of cases )
Myelomeningocele – Cystic distention of meninges with neurological loss (94 %), usually at lumbar or lumbosacral junction

Clinically determined by lowest active motor control .
Upper Thoracic
Lower Thoracic
Upper Lumbar
Lower Lumbar
Sacral

Flaccid Vs Spastic Paralysis
 Patients with L 4 functions have good chance of functional walking (Household or Community )
With L 5 Function – Good Chance of community walkers

Spina Bifida Occulta  (Spinal Dysraphism)
L5 /S1 and Cervical Axis most common .
Although skin intact , almost never normal as some midline anomaly suggest diagnosis.
Bulge ,tiny dimple , Pigmented Naevus , Faun’s tail
Neurological defect – occurs in a small percentage and depends on level and extent of  cord involvement

Tethetered Cord Syndrome  - Diastomatomyelia
Spina Bifida Cystica (Myelocele ,  Myelomeningocele )
Posture tells neurological level
LL Paralysis – complete or incomplete
85 % - Paraplegic foot deformity
Hip dislocation , Genu Recurvatum , Flexion Contracutre
 Genu Recurvatum +/- Talipes and claw toes
Scoliosis – 80 % of thoracic and high lumbar lesions
Ansesthetic Skin – leading to pressure sores
Lower Urinary tract dysfunction leading to renal failure
Latex Insensitivity in 60  %  due to surgical procedures multiple times , catheters ,shoes
Do surgery in latex free environment
 Hip Dislocation in 50 % cases of Spina Bifida
Vertical Talus
Other Vertebral Anomalies
Hemivertebrae,Abnormal bars , Uncommon (15-20%)
Mental Retardation
Developmental Delay
Bone Fragility , Pathologic Fracture
Upper Limb weaknesses and spasticity due to Arnold –Chiari Malformation
Syringomyelia leading to upper limb dysfunction
Only 30 % Patients with Mylomeningocele are functionally independent
29 % lack B/B control
Lipomeningocele
Defect contains lipoma that is involved with sacral nerves.

Diastomatomyelia
Osseous,Cartilaginous ,fibrous septum in central portion of spinal canal leads to complete or incomplete sagittal division of spinal cord into Two hemicords.
Upper lumbar levels involved.
F > M

Tethered Cord Sydrome
May recur after repair of myelomeningocele
Incidence of tethering 11-20 %
Occurs at 6-11 years.
Back , buttock , lower extremity pain , progressive motor deterioration , progressive foot deformities, rapid increase in severity of lordosis or scoliosis
Flaccid vs Spastic Paralysis\
Patients with  L 4 function have good chance of functional walking (household or community )
With L 5 function have good chance of community walkers
Diagnosis
MRI with Contrast
            R/O associated dermoid  cyst
Treatment
            Early release stops progression of spine deformity relieves pain

HydRocephalus
            90 % Require VP shunt
            Acute –bulging of fontanelle ,altered mental status,head-ache , Syrinx formation
            Scolisosis,,motor deterioration
Arnold-Chialri Malformation
            95 % children with myelomeningocele
            Herniation of posterior fossa structures (Cerebellum,brainstem ) through the foramen magnum
            Leading cause of death in infants
            Some deformities persist /progress and require surgical decompression
X-ray
            Gaps in neural arch
            Widening of spinal canal
            Bony spur of diastomatomyelia
            Other spinal deformities
                        Bars
                        Hemivertebra
                        Fused Ribs
                        Scoliosis
                        Lordosis
                        Kyphosis
Pathology
Failure of development of neural arch tube from arch at 4/52
Membrane or cord prolapsed
Skin over defect –Open /Close
Closed Defects associated with Dermal Cysts
                                                Lipoma of Cauda Equina
                                                Diastomatomyelia
                                                Partial or complete absence of sacrum / meningocele
Meningocele contains thecal sacs , no neural elements
Myelomeningocele – contains nerve roots and cord remnants adherent to sac protrude
Inv
New onset spasticity , progressive scoliosis –evaluate for cord tethering ,hydromelia and shunt function
Treatment
Team approach including paediatrician
Counseling
May elect not to treat for high lesion
Surgery
Hydrocephalus , tethered cord , bladder dysfunction
Forcible correction l- cant do  because bone are weak , skin is paretic.
Surgical Correction
After child at least several months old. Sequence of correction – Hip , Foot , Knee
High complication rate
            Infection , Pathological fracture, Pseudoarthrosis

Surgery to correct spine and limb deformities will be more rewarding when neurologically stable and free of UTI .
Treatment
Hip – Dislocation , common in L3 -4
Primary cause
            Paralysis – Abductors and Extensors with power in Adductor and Flexor
            Unilateral – open reduction , B/l – some recommend conservative treatment (controversial)


Open Reduction , tenotomy , or muscle transfer may be needed to reduce a dislocated hip.
Early Correction –avoids late bony changes .
Aim – Stiff enough to support on extension and flexible enough to support on sitting
Iliopsoas Transefer (Sharrad ) : Pass tendon through a hole in pelvis to GT to redirect pull of flexors.

Knee
Patients have Quadriceps weakness requiring KAFO.
Genu Vlagum
            Release of contracted Iliotibial band
Flexion deformity – release hamstring if not corrected by flexing FFD of hip. May  need extensive release of knee structures or Extension osteotomy
Genu Recurvatum
            May be due to Foot Equinus
Tibia
            Torsional deformities – secondary to muscle imbalance , Treratment – Osteotomy

Foot
            Goal – Platigrade foot with adequate  plantar skin , suitable for bracing
            Soft tissue procedure at 6 months.
Deformities include
            Equinus – Posterior Release
            Equinovarus – (Most common) – PMR
            Calcaneus – TA transfer to Os Calcis
            Valgus – Tendon transfer or subtalar arthrodesis
            Cavus – Plantar release , metatarsal +/- os calcis osteotomies
            Vertical talus –            
            Club foot – Most are rigid deformities requiring surgical correction
            Fixed Deformities
                        Bony procedures eg. TA ,Grice Arthrodesis , Dwyer Procedure

Spine
Scolisosis
Most common skeletal deformity in Myelomeningocele ,occurs in 80 %
Thoracic , Upper Lumbar most common
Lumbosacral
Rapidly progressing present
            MRI to r/o Tethered cord and neurosurgical evaluation for shunt
Causes
            Congenital Spinal Malformation
            Unequal Growth
            Embryological Causes
                        Failure of formation
                        Failure of segmentation
            Rigid Scoliosis
            Mechanical instability and paralysis due to absent cement

           

















Tethered cord and Hydromelia causes Neurological abnormalities. Surgical correction
                        High Pseudoarthrosis
                        Infection rate upto 25 %
Kyphosis
            15 % patients  
            Often severe with breathing and eating difficulty
Types
            Apex – L1 and L2
            Rigid lordosis in proximal adjacent lower dorsal spine.
                        Treatment – Excision of 1 or 2 vertebrae proximal to Apex
            Collapsing
                        Due to muscle imbalance
                        Corrected by manual pressure
                        Not associated with proximal lordosis
                        Aim – to avoid surgery as long as possible to allow for long spinal growth
                        Care with braces
Lordosis
            Excess lumbar lordosis due to other problems eg. FFD
            Tethered cord – more common in children with lesion at L4 -5
            Scoliosis at young age s/o tethering
            Treatment -      Khyphectomy
Early embryology
            Weak 1-2 – Zygote Formation , Bilaminar Embryo
            Weak 3-  8- Embryological period
            Weak 9-38 – Fetal Period

Development of Somite (3/52)
            Mesoderm forms paraxial mesoderm which differentiates into Somites on both the sides

            Somites
            Form Axial skeleton , vertebrae, Muscles
            Form in 20-30 days ,
            42-44 pairs , used to calculate age of embryo
            Differentiate into
                                    Ventromedial Sclerotome forming  Vertebrae and ribs
                                    Myotome forming muscles
                                    Dermatome forming skin
            At 4 weeks neural tube formed open in caudad and rostral ends.




           
















           








































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