Perthes’ Disease
A 6 y.o boy presents with left leg pain and limping. Attached are plain radiographs taken at the ED.
- Describe the plain radiograph.
- What is your diagnosis?
- If the child had URTI a few days before, would this change your diagnosis?
- What is the management principle?
- This is an AP and frog leg view of the pelvis belonging to the index patient.
demonstrating avascular necrosis of the left hip with <50% height loss of the lateral pillar of the femoral epiphysis
Compared to contralateral hip, there are
- Decreased size of femoral head
- Irregularity of femoral head
- Flattening of epiphyses
- Sclerotic appearance
- Central part of epiphyseal osific centre appears lucent/ fragmented, indicating fragmentation stage of the disease process.
- Lateral part height reduced but < 50%
If according to Herring’s lateral pillar classification - Group B
The lateral pillar is defined as the lateral portion of the femoral head, on the anteroposterior radiograph, that is demarcated from the central portion of the head by a lucent line of fragmentation.
- Unilateral Perthes’ Disease
- If associated with recent URTI, will consider reactive synovitis if plain radiograph is normal. No, it wont change my Dx as this child’s xray is not normal.
- Management depents on age and severity of disease, disease stage- Classification, limitation in function.
Aims:
a) Symptomatic treatment
b) Hip containtment
Legg-Calve-Perthes (idiopathic avascular necrosis of the proximal femoral epiphysis) carries a different prognosis based on patient age and involvement of the lateral pillar. In children <8 years old and in children with lateral pillar Class A involvement, initial treatment is non-operative and consists of observation, activity restriction, and physical therapy. In children >8 years of age with group B or BC disease, treatment consists of containment procedures including proximal femoral varus osteotomies and/or pelvic osteotomies.
Child < 6 years
Pillar A & B
Non-operative: analgsia, activity modification, physio to stretch abductors.
Pillar C
Non-operative: Abduction brace
Child > 6
Pillar A & B
Non-operative: Abduction brace
Operative: Osteotomy
Child > 6
Pillar C
Non-operative: Abduction brace
Operative: Osteotomy
Outcome unaffected by treatment
Child > 9
Operative: Femoral/pelvic osteotomy
Ideally, operative treatment done before collaspe of femoral head occurs.
If collaspe has occured, considered late stage - B, C
If collaspe has occured, better outcome with Femoral/pelvic osteotomy.
DDH: Management
Outline the management principle for DDH that presented
a) > 1 year
b) > 18 months
Outline the management principle for DDH that presented
a) > 1 year
b) > 18 months
c) > 2-3 years
SCFE
Hemiepiphysiodesis
1.What is this implant? What are its features?
2.What is the indication for its use?
3. What is the principle for its use?
4. How to keep it as a sleeper plate?
1.What is this implant?
What are its features?
8 plate:
-low-profile and are of equal thickness, with a centre holes to allow accurate placement
- screws are titanium (or stainless steel), cannulated, and self-tapping
- plates and screws are painted and color-coded for ease of identification, but the surgeon may mix and match as dictated by the local anatomy
- not a locking plate; the principle is to deflect the physis (tension band) rather than overpower it
2.What is the indication for its use?
- is a tension band plate (TBP) for temporary guided growth/growth modulation of angular deformity.
- acts as a focal hinge at the perimeter of the physis with a longer lever arm, so as the physis grows, the screws toggle in the plate and pivot in the bone bringing about gradual correction and does not produce compression at the physis, thus preserving the growth potential.
- is typically removed after the deformity is corrected to prevent overcorrection.
- What is the principle for its use?
Principles of placement of 8-plate in hemiepiphysiodesis:
(i) Convex side
(ii) Mid-sagittal line
(iii) Subfascia, epiperiosteal (extraperiosteal)
(iv) Screw length cannot cross midline
(v) Non-rigid plate + 2 threaded screws
-> can toggle in the plate without backing out
-> longer moment arm as centre of rotation is outside the physis, acting as a focal hinge
-> applies tether only at periphery of physis; reduce risk of permanent physeal damage by not compressing physis
(vi) Can only hemiepiphysiodesis for 2 years, then allow physis to rest, otherwise will develop a physeal bar
- How to keep it as a sleeper plate?
After TBP removal, rebound deformity may occur, necessitating reinsertion of a new TBP.
Can keep previously inserted plate first.
Remove proximal screw (metaphyseal screw)only and keeping the plate and the epiphyseal screw behind
as physis grows away from diaphysis.
If needed further correction, can insert proximal screw again.
Deformity correction
- What is CORA?
- What is ACA?
- What are the rules of osteotomy?
- What is CORA?
is the intersection points of the anatomical and mechanical axes of both sides of deformity when there is a deformity in a long bone.
Simple malunited fracture usually has 1 CORA.
Bone with congenital bowing may have several CORAs.
https://abs.orthofix.it/blog/cora-center-of-rotation-of-angulation/#:~:text=The%20CORA%20(Center%20of%20Rotation,current%20osteotomy%20techniques%20and%20hardware.
- What is ACA?
- Rules of osteotomy
ACA-CORA
Rule 1. If the ACA is located on the concave side of angular deformity, secondary lengthening at the corticotomy develops after angular correction (A) and if the ACA is located on the convex side, secondary shortening develops (B).
Rule 2. If the ACA is not located on the transverse bisection line (tBL) of the CORA, the secondary translation develops after angular correction. The fragment on the opposite side to the ACA moves toward the convex side of the angular deformity.
Rule 3: If the osteotomy passes through the ACA, but the CORA is at a different level, there will be angulation but no translation of bone ends. The axis lines will be parallel, but translated
Deformity correction
What are the techniques used to correct deformities?
Techniques used to correct deformities
A) Acute correction
Indication:
- mild to moderate deformities, < 30 degrees
Options:
i) Plates and screws
A: suited for peri-articular deformities, less risk of spreading infection into the joint and causing joint stiffness (such as external fixator)
D: Invasive
ii) Intramedullar nail
A: minimally invasive, requires only percutaneous osteotomy, suitable for diaphyseal and rotational deformities.
D: Limited ability for correction with translation
B) Gradual correction
Options
i) Hemiepiphysiodesis
- technique to inhibit growth on one side of the physis to correct the deformity.
- temporary or permanent.
Temporary: 8 plate, staples.
Permanent: Drill hemiepiphysiodesis, Phemister bone block technique
ii) Distraction osteogenesis
-utilises Ilizarov’s principles to correct deformities using external fixators resulting in a
* stable construct using monolateral or circular fixator
* low energy osteotomy (presevation of blood supply)
* latency period of 5-7 days
* rate and rhythm of 1 mm distaction per day
Prosthesis
- Name this prosthesis.
- Label the following parts.
- Indication for prescribing this prosthesis.
- What are common complications from using this?
- How will you counsel the parent of a child using this prosthesis?
- What are the risk factors of failure of Pavlik Harness?
- What are the contraindications for its use?
- Name this prosthesis.
Pavlik Harness - Label the following parts.
- Indication for prescribing this prosthesis.
For neonates < 6 months old with confirmed DDH on USG and hips remained reduced in Pavlik harness. - What are common complications from using this?
- transient femoral nerve palsy (from excessive flexion of the hip)
-inferior dislocation
- AVN
- Pavlik disease- if hips are not reduced but kept in flexion and ABDuction leading to erosion of superior acetabulum and poorly developed posterolateral wall of acetabulum - How will you counsel the parent of a child using this prosthesis?
- To wear for 23 hours in a day.
- To wear untill hip stable as confirmed by repeated USG 3-4 weeks later, and if still reduced, is kept for 3-4 months untill hip is more stable. If repeat USG showed not reduced, abandoned PH, wait till child is older to do CMR and hip spica.
- Make sure hip not flexed to > 100 deg and free to ABDuct - What are the risk factors of failure of Pavlik Harness?
- older age group eg > 6 months
- bilateral dislocations
- coverage < 20% on USG
- Inability to reduce hip on manipulation - What are the contraindications for its use?
- teratologic hip dislocations
- spina bifida
- splasticity
DDH: Hip arthrogram
- Describe image on the left.
- What is the procedure being done?
- When is this procedure indicated?
- What additional procedures will accompany this operation?
- What is the safe zone of Ramsey?
- How should the hip be placed in hip spica?
- How long is duration on hip spica?
- What are common complications from hip spica?
- If failed CMR, what can be done?
- Describe image on the left.
Plain radiograph of pelvis of a skeletally immature child with DDH with a dislocated hip as highlighted by arthrogram which showed
- medial pooling > 7mm
- hour-glass configuration of the capsule
- Rose thorn sign Limbus - What is the procedure being done?
Closed reduction of a dislocated hip in a skeletally immature child with DDH, aided by arthrogram to confirm if the femoral head is concentrically reduced into the acetabulum.
- need to obtain < 5 mm constrast pooling medial to femoral head, no interposition of the limbus. - When is this procedure indicated?
6-18 months
Failed Pavlik harness - What additional procedures will accompany this operation?
i) Reduction using Ortolani maneuvre (hip flexion and ABDuction whilst elevating the greater trochanter)
ii) +/- Adductor tenotomy (if patient has an unstable safezone, if excessive ABDuction is required to maintain reduction, so done to avoid forceful reduction)
iii) Hip spica application - What is the safe zone of Ramsey?
- Measures angle between maximum ABD and minimum ABD in which the hip remains reduced at hip flexion of 90 deg.
- Range is usually 30 deg, with minimum 30 deg and maximum 60 deg.
- Should be 10-20 deg less than max ABDuction possible - How should the hip be placed in hip spica?
Hip flexion to 100 degrees maximum
Abduction in 45 degs
Neutral rotation - How long is duration on hip spica?
3 months/12 weeks
Change after 6 weeks
May continue after with abduction bracing - What are common complications from hip spica?
AVN (impingement of the posterior superior retinacular branch of the medial circumflex artery)
Redislocation
Residual acetabular dysplasia - If failed CMR, what can be done?
- perform open reduction when child reaches > 24/12 of age.
- via anterior approach (Smith-Peterson)- Most commonly used due to decreased risk of injury to medial femoral circumflex artery.
* with Capsulorrhaphy performed.
* Remove all possible anatomic blocks to reduction
* + adductor tenotomy if patient has unstable zone
* post op immobilisation in functional position
30 flexion, 30 abduction, 30 internal rotation
- Most commonly used due to decreased risk of injury to medial femoral circumflex artery.
DDH: Clinical presentation
1.Describe this plain radiograph.
2. How may a child with such pelvic radiograph present clinically?
3. If both hips are involved, how will his presentation differ?
1.Describe this plain radiograph.
Pelvic radiograph of a skeletally immature child with dislocated left hip due to DDH as evidenced by
- dysruption of Shenton’s line
- femoral head is NOT below the Hilgeriner line and NOT medial to the Perkin’s line
- small femoral head ossific nucleus
- shall acetabulum as acetabulum index is > 25
- How may a child with such pelvic radiograph present clinically?
Late presentation after child starts to weight bear
- LLD with affected side (left side) demonstrating short limb gait with toe walking, bending of knee at the longer limb,higher knee level, Trendelenburg gait, lurching towards affected side, externally rotated lower limb.
- Examination: shortening at femoral component, limited ABDuction, excessive IR and ER - If both hips are involved, how will his presentation differ?
Waddling gait with side to side lurching
Excessive lumbar lordosis
DDH: Open reduction
1. When is this indicated?
2. What are the structures you may encounter during Open Reduction?
- When is this indicated?
Failed CMR of hip
> 18 months - What are the structures you may encounter during Open Reduction?
Soft tissue
a) Extracapsular
-psoas tendon
-adductor
b) Intracapsular
- Capsule hourglass
- Elongated and hypertrophied ligamentum teres
- Inverted labrum/limbus
- Transverse acetabular ligament
- Pulvinar (fibro fatty tissue)
Bone
a) Femur
- Antverted
- Valgus
b) Acetabulum
- Dysplastic/shallow with AI >30 deg
DDH: Surgical options - Pelvic osteotomies
1. What are the types of Pelvic osteotomies used for treatment of DDH?
2. Include their indications.
- What are the types of Pelvic osteotomies used for treatment of DDH?
A) Reconstructive pelvic osteotomies
- Salter
- Triple innominate (Steele)
- PAO (Ganz)
- Pamberton
- Dega
- Dial
B)Salvage pelvic osteotomies
- Shelf
- Chiari
- Include their indications.
* > 4 years old (as decreased potential for acetabular remodelling as child ages)
* Radiographic changes showing significant dysplasia on the acetabular side (Increased Acetabular Index).
* For purpose of increasing anterior or anterolateral coverage.
DDH: Surgical options - Femoral osteotomies
1. When will you consider femoral osteotomies?
2. What are the common complications from performing femoral osteotomies?
- When will you consider femoral osteotomies?
Orthobullet
Indication:
2-4 years old with residual hip dysplasia
Consider use after femoral head is congruently reduced with satisfactory ROM
- To correct anatomical changes at the femur (femoral anteversion, coxa valga)
-To facilitate reduction
-To decrease the risk of AVN by relieving the tension produced by the reduction of a dislocated hip - What are the common complications from performing femoral osteotomies?
Cerebral palsy
- Define.
- How to classify?
- Cerebral palsy
-Groups of disorders
-That results from non-progressive brain damage
-Occurs during early development
-Characterised by abnormalities of movement and posture. - Classification
a) Type of motor disorders
i) Spasticity
- most commonest, increased muscle tone and hyper-reflexia.
ii) Hypotonia
- phase during early childhood before spasticity becomes obvious.
iii) Athetosis
-continuous involuntary writhing movements, exacerbated when frightened.
iv) Dystonia
-generalised increased in muscle tone and abnormal positions induced by activity.
v) Ataxia
-muscular incoordination during voluntary movements
vi) Mixed palsy
b) Topography
i) Hemiplegia
- one side of the body
- UL more affected than LL
ii) Diplegia
- both side of the body
- LL more affected than UL
iii) Monoplegia
- unusual if isolated, usually will have other areas involved as well.
- need to rule out neonatal brachial plexopathy
iv)Tetraplegia
Joint position
Define
a) Subluxation
b) Dislocation
a) Subluxation
- partial dislocation of a joint
- partial contact of the articulating surfaces of the bones
- in hips- more than 30% of uncovering of femoral head
b) Dislocation
-complete separation of 2 articulating bony surfaces
Cerebral palsy: Upper Limb
- What are the common deformities seen in upper limb?
- What are your goals in management?
- When to do operation?
- Describe examples of surgical procedures done for UL deformities in CP patients
- Common deformities
typically seen in child with
* Spastic hemiplegia
* Total body involvement
- shoulder internal rotation contracture
- forearm-pronation
- elbow flexion deformity
- wrist-flexion deformity
- thumb-in-palm deformity (adduction of thumb)
- finger-flexion deformity (clenched fingers)
- Goals in management
i) hygienic procedures
- indicated to maintain hygiene in patients with decreased mental and physical function
ii) functional procedures
- indicated in patients with voluntary control, IQ of 50-70 or higher, and better sensibility
Upper limb priorities
- improving resting position of limb
-restoring grasp
- Surgery 8 years and older
- Surgery options
A) Shoulder internal rotation contracture
Op: shoulder derotational osteotomy and/or subscapularis and pectoralis lengthening with biceps/brachialis lengthening capsulotomy
Indication: if severe contracture (>30 degrees) interfering with hand function
B) Elbow flexion deformity
Indication: Cannot extend to 90 degree, for FFD
Op: fractional lenghtening of biceps and brachialis tendon, lacertus fibrosis release + release of brachialis origin.
C) Forearm pronation deformity
Indication: Risk of radial head subluxation/dislocation
Op:
i) Release of pronator teres & transfer to anterolateral position to act as supinator.
ii) FCU tendon transfer to ECRB
D) Wrist flexion deformity
Indication: as a functional procedure in patients with voluntary control, IQ of 50-70 or higher, and better sensibility
Op:
i) FCU or FCR tendon lengthening (if wrist ulnarly deviate)
ii) FCU to ECRB transfer or FCU to EDC tendon transfer (if weak extensors)
which one?
with good grasp ability–> transfer FCU to EDC
with poor grasp ability –> transfer FCU to ECRB
iii) wrist arthrodesis
Indication:
to improve hygiene and function in patients with non-supple contractures who lack volitional control of the wrist/hand.
Op: wrist arthrodesis with excision of proximal carpal row
iv) Thumb in palm deformity
Indication:
functional procedure in patients with voluntary control, IQ of 50-70 or higher, and better sensibility.
Op: release of the adductor pollicis and 1st dorsal interroseous muscles, lengthening FPL, release thenar muscle, transfer of tendons (to reinforce ABDuction and extension), and stabilization of the MCP joint.
vi) Clenched/Clawed fingers
Indication: can unclenched with simultaneous flexion of wrist
Op: lenghtening of long flexor muscle, avoid wrist in extension if tendon transfer/fusion is undertaken
Cerebral palsy: Lower limb
- What are the typical clinical features of Spastic Hemiplegia & intervention that can be done.
More distal involvement
i) Foot/Ankle
Equinovarus deformity- dynamic or fixed
- Op:
equinus - muscle recession of gastroc/soleus complex
varus - if correctable/dynamic, can do tendon transfer by splitting the anterior tibialis tendon and transfer to lateral aspect of the foot.
ii) LLD
- hemiplegic limb always short irrespective of any joint contractures
- Op: epiphyseodesis of contralateral distal femoral +/- proximal tibia.
Cerebral palsy: Lower limb
- What are the typical clinical features of Spastic Diplegia & intervention that can be done.
More proximal involvement
A. Hips
i) Hip adduction deformity
- indicated for release if passive ABDuction < 20 deg
- attempt medial hamstring lenghtening –> if still not improve, release adductor via open tenotomy of adductor longus and division of gracilis.
ii) Hip flexion deformity
- indicated for release if FFD > 30 degrees
- attempt lenghtening of psoas tendon at pelvic brim in walking child.
- attempt release of psoas tendon at level of lesser trochanter in non-walking child.
iii) Hip internal rotation deformity
- usually associated with flexion and adduction (combination of two previous aspects)
- soft tissue –>adductor release, psoas lenghtening
- bone procedure –>derotation osteotomy of femur at subtroch +/- compensatory rotation osteotomy of tibia
iv) Hip subluxation -30% of CP
- occurs due to persistent flexion-adduction deformity which leads to femoral neck anteversion.
- weakened ABDuctors and lack of weight bearing –> risk of acetabular dysplasia and subluxation of joint.
- risk of dislocation is higher in non-walkers.
-prevention is correction of flexion-adduction deformity before 6 years old.
- types of intervention depends on age
eg.
* < 6 years: soft tissue procedures to correct flexion and ADDuction deformities.
* > 6 years: pelvic osteotomies for acetabular reconstruction +/- varus derotation osteotomy
* non walkers, long standing dislocation - excision of proximal end of femur if have discomfort
* adult, walking - degenerative symptoms, can offer THR.
B. Knee
i) Flexion deformity
- usually due to hamstring tightness
- aggravated by hip flexion or weakness of ankle plantar flexion
- if < 25: fractional lenghtening of hamstrings- medially
* Overlengthening can weaken hip extension and exacerbate hip flexion and lumbar lordosis.
- if > 25: extension osteotomy of distal femur
ii) External tibia torsion
Op: supramalleolar osteotomy
C. Foot
i) Equinus of foot
- patient will toe walk
- Op:
i) Fractional lenghtening of fascia/muscle of gastroc-soleus complex
ii) lenghtening of Achilles tendon
ii) + varus
Op: Split and anterior tibialis tendon transfer to lateral
iii) +valgus (equinovalgus/”rocker bottom” foot)
Cerebral palsy: Lower limb
- What are the typical clinical features of Total body involvement in CP & intervention that can be done.
Windswept hip
One side - ADDucted, flexed, IR
Contralateral side- ABDucted, ER, extended
If hip NOT subluxated, but tightness present- release adductors and psoas
If subluxation
- release adductors and psoas AND
- pelvic osteotomies, femoral varus derotation with shortening osteotomies
- opposite hip can benefit from release of hip ABDuctors and Extensors eg Gluteus maximus, iliotibial band
Cerebral palsy
Define
a) True equinus
b) Apparent equinus
c) Jump gait
d) Crouch gait
a) True equinus
defined by the foot position in relationship to the tibia being less than plantigrade
b) Apparent equinus
defined by a foot position that is normal in relationship to the tibia, however heel strike does not occur due to more proximal deviations (flexion of the knee most common)
c) Jump gait
Deformity includes hip flexion, knee flexion, and equinus ankle deformity ( could result in apparent ankle equinus)
d) Crouch gait
A combination of hip flexion, knee flexion, and excessive ankle dorsiflexion (the latter may be represented by flatfoot or calcaneus)
