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
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
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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