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Blountā€™s Disease
Sarbdev Singh
Introduction
ā€¢ Symmetrical genu varum before the age of 2 years is rarely
pathological
When is Genu Varum Pathological?
ā€¢ Most likely if:
ā€¢ Present after 2 years of age
ā€¢ Progressive deformity beyond age of 2
ā€¢ Unilateral
ā€¢ Short stature
ā€¢ Severe
ā€¢ In a child with obesity
ā€¢ Early walker (before age of 1)
ā€¢ Mild deformities after the age of 2 years may resolve spontaneously
and probably represent extreme variants of physiological bowing
What causes
Genu Varum?
ā€¢ Injury of proximal tibial physis (infection,
radiation, or trauma)
ā€¢ Metabolic Diseases ā€“ Rickets, OI
ā€¢ Blountā€™s disease ā€“ infantile and adolescent
ā€¢ Generalized or focal osteochondrodysplasia, eg
hereditary multiple exostoses, achondroplasia,
focal fibrocartilaginous dysplasia
ā€¢ Tibia hemimelia
ā€¢ MED, SED
ā€¢ Family history of bow legs should raise suspicions of
ā€¢ Hypophosphatemic rickets
ā€¢ Hereditary multiple exostoses
ā€¢ Bone/cartilage dysplasia
Tibia vara / Blountā€™s Disease
ā€¢ A progressive pathologic varus deformity of the proximal tibia
ā€¢ Disease is centered at the medial aspect of proximal tibial physis
ā€¢ Aetiology ā€“ unknown
ā€¢ Thought to be an overload phenomenon on proximal medial tibial physis ā€“
supraphysiological compressive forces from severe obesity
ā€¢ Tends to produce VARUS + FLEXION + INTERNAL ROTATION deformity
(3-D)
1. Infantile tibia vara (Blount disease) : < 3 years, common
2. Adolescent tibia vara : >10 years old
ā€¢ Clinical features
ā€¢ obese
ā€¢ Lateral thrust gait
ā€¢ Deformity
ā€¢ Genu varum - acute proximal
ā€¢ Internal tibial torsion
ā€¢ Procurvatum (flexion deformity)
ā€¢ Unilateral or bilateral
ā€¢ LLD (mostly unilateral)
ā€¢ Cover up test (infantile)
ā€¢ Patient supine & lower extremities fully extended with the
patella up.
ā€¢ Physiologic bowing is indicated by a valgus proximal tibia,
while a Blount's patient will have a neutral or varus proximal
tibia.
*A toddler may appear bow-legged if both knees and hips are flexed
when walking; a gentle repositioning of the hips and knees with patella
facing up will correct it (NEVER COMMENT ON ANGLE IF FFD PRESENT)
When are radiographs
required?
ā€¢ Most children referred DO NOT require
ā€¢ Unilateral bowing
ā€¢ Features not associated with physiological
bowing (as mentioned before)
ā€¢ Varus progresses
*Radiographic features are only present in
Blountā€™s after age 18months (*Staheli)
ā€¢ Imaging
ā€¢ Long limb standing view
ā€¢ Tibia AP and lateral views
*Patella facing forward
ā€¢ Findings
ā€¢ Sharp varus angulation in metaphysis
ā€¢ Widening and irregular physeal line medially
ā€¢ Medially slopped & irregular ossified epiphysis
ā€¢ Prominent beaking of medial epiphysis with lucent cartilage island within
beak
ā€¢ Lateral subluxation of proximal end tibia
*problem ā€“ lack of reproducibility and that it was not intended for use in determining the prognosis
or type of treatment, as pointed out by the author. (*Staheli)
II
III IV V VI
ā€¢ Drennan metaphyseal-diaphyseal angle (MDA)
ā€¢ Angle between
ā€¢ Line connecting metaphyseal beak and
ā€¢ Line perpendicular to the longitudinal axis of the
tibia
ā€¢ Meeting at most prominent lateral point
ā€¢ >16ā€™ abnormal, 95% chance progression
ā€¢ >10 : Diagnosis of Blountā€™s in Probable (Staheli)
ā€¢ <10ā€™ : 95% chance of resolution
ā€¢ Tibiofemoral angle
ā€¢ Mechanical axis
What indicates progression is likely?
ā€¢ Severity of the deformity at presentation
ā€¢ Obesity ā€“ BMI >22
*BMI > 22 + MDA > 10 --- Strongly suggestive of progression
OR
MDA > 16
*Selvadurai Nayagam
What problems need to be managed?
ā€¢ Tibial Deformity
ā€¢ Varus + Internal Tibial Torsion + Flexion
ā€¢ Joint Deformity
ā€¢ Medial tibial hemiplateau depression
ā€¢ Mostly in late stage
ā€¢ Results in recurrence & early degenerative
joint disease (DJD)
ā€¢ Physeal Bar
ā€¢ LLD
ā€¢ Mainly in unilateral with significant physeal
growth retardation
ā€¢ Limb lengthening not a great option with
underlying joint instability & deformity
ā€¢ Close f/up for ideal chance for an
epiphysiodesis of opposite extremity.
ā€¢ Recurrence
ā€¢ Obesity, Ligament laxity, Langenskiold stage IV
above, presence of bone bar and
undercorrection (Staheli)
ā€¢ Hence why DIAGNOSE EARLY & INTERVENE!!
ā€¢ Neurovascular complications
ā€¢ High risk of compartment syndrome & peroneal
nerve palsy
ā€¢ Anterior compartment release recommended
during index procedure
*Randall Loder
Our Aim of treatment?
1. Restore normal tibial alignment
2. Restore normal mechanical axis
3. Correct joint deformity
ā€¢ If articular surface is defective ā€“ need to correct
4. Equalize limb length
ā€¢ Differing knee levels predisposes to early DJD
5. Maintain correction, prevent recurrence
ā€¢ Best chance of avoiding recurrence ā€“ early correction of deformity!
6. Prevent neurovascular complications
*Randall Loder
Treatment Options (Non-operative)
ā€¢ Mainstay treatment for infantile blountā€™s of
Lā€™skiold stage I & II
ā€¢ Above knee non articulated KAFO ā€“ custom
moulded to provide 3 point fixation
ā€¢ Takes ~ 12-18months of use to resolve with
success rates of 90% (when presenting
deformity is mild to moderate)
ā€¢ If fail by age of 4 OR progresses to Lā€™skiold
stage III --- immediate tibial osteotomy is
indicated (Randall Loder)
*Selvadurai
ā€¢ No data to confirm effectiveness
ā€¢ Only for younger than 3 & Stage I or II
ā€¢ Multiple problems associated :
ā€¢ Use during daytime can be difficult &
uncomfortable leading to poor compliance
ā€¢ Moulding is difficult & corrective force
over knee may not be tolerated
ā€¢ Locks knee in extension ā€“ unphysiological
& uncomfortable & stretches ligaments
ā€¢ May affect sleep patterns causing irritable
child/behavior changes
*Staheli
Proximal Tibial Osteotomy
ā€¢ Most widely used surgery
ā€¢ Infantile Blountā€™s ā€“ MUST be below tibial tuberosity
1. Acute corrective
ā€¢ With internal fixator
ā€¢ Kwiring in younger
ā€¢ Plating in older
ā€¢ With external fixator ā€“preferred for ability to fine tune deformity
post op.
ā€¢ Often with prophylactic anterior compartment fasciotomy
2. Gradual corrective
ā€¢ With external fixator
ā€¢ Less risk of compartment syndrome
Physeal elevating osteotomy
ā€¢ Significant depression of medial tibial epiphysis - Langenskiƶld V / VI
ā€¢ Sparing physis
ā€¢ When child approaching
skeletal maturity
ā€¢ Evidence of premature
physeal arrest
*Medial Plateau Elevation
ā€¢ Tilted ā€˜pitched roofā€™ appearance of the tibial Joint line --- misleading
ā€¢ Could be an unossified cartilage appearing as incomplete medial tibial
condyle
ā€¢ MRI or arthrogram will show true joint level
*comparison of xray of knee taken in supine & weight bearing!
ā€¢ In true underdeveloped medial tibial condyle --- clinically significant
laxity to valgus stressing can be exhibited
*Selvadurai
Physeal Bar Resection
ā€¢ Young patients who has developed physeal bar ā€“ resection can be
considered along with repeat osteotomy
ā€¢ Results are often disappointing
ā€¢ In patients with at least 4 years of growth remaining (Orthobullets)
ā€¢ Interpositional material - fat
Growth modulating
ā€¢ Proximal lateral tibia
hemiepiphyseodesis
ā€¢ ā‰„ 2 years of growth remaining
ā€¢ 2 types
I. Temporary
ā€¢ Extraperiosteal 8 plate or staples
ā€¢ Slight overcorrection to valgus to
account for rebound growth upon
removal
II. Permanent
ā€¢ Timing is crucial
Complications
ā€¢ Proximal tibial recurvatum
ā€¢ injury to proximal tibial physis at level of tibial tubercle ļƒ  hyperextension instability of knee
ā€¢ Anterior tibial artery injury
ā€¢ Proximal tibial osteotomy must be performed distal to tibial tubercle, near the level of trifurcation of popliteal
artery
ā€¢ 29% injured in osteotomy procedure
ā€¢ Compartment syndrome
ā€¢ Prophylactic fasciotomy of all compartment should be performed during all osteotomy procedures
ā€¢ Postop neurovascular surveillance for 1st 48H
ā€¢ Peroneal nerve palsy
ā€¢ Infections
ā€¢ Iatrogenic fractures
ā€¢ Loss of correction
ā€¢ Recurrence
ā€¢ Progression
Thank you

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blount disease pptx

  • 2. Introduction ā€¢ Symmetrical genu varum before the age of 2 years is rarely pathological
  • 3. When is Genu Varum Pathological? ā€¢ Most likely if: ā€¢ Present after 2 years of age ā€¢ Progressive deformity beyond age of 2 ā€¢ Unilateral ā€¢ Short stature ā€¢ Severe ā€¢ In a child with obesity ā€¢ Early walker (before age of 1) ā€¢ Mild deformities after the age of 2 years may resolve spontaneously and probably represent extreme variants of physiological bowing
  • 4. What causes Genu Varum? ā€¢ Injury of proximal tibial physis (infection, radiation, or trauma) ā€¢ Metabolic Diseases ā€“ Rickets, OI ā€¢ Blountā€™s disease ā€“ infantile and adolescent ā€¢ Generalized or focal osteochondrodysplasia, eg hereditary multiple exostoses, achondroplasia, focal fibrocartilaginous dysplasia ā€¢ Tibia hemimelia ā€¢ MED, SED
  • 5. ā€¢ Family history of bow legs should raise suspicions of ā€¢ Hypophosphatemic rickets ā€¢ Hereditary multiple exostoses ā€¢ Bone/cartilage dysplasia
  • 6. Tibia vara / Blountā€™s Disease ā€¢ A progressive pathologic varus deformity of the proximal tibia ā€¢ Disease is centered at the medial aspect of proximal tibial physis ā€¢ Aetiology ā€“ unknown ā€¢ Thought to be an overload phenomenon on proximal medial tibial physis ā€“ supraphysiological compressive forces from severe obesity ā€¢ Tends to produce VARUS + FLEXION + INTERNAL ROTATION deformity (3-D) 1. Infantile tibia vara (Blount disease) : < 3 years, common 2. Adolescent tibia vara : >10 years old
  • 7.
  • 8.
  • 9. ā€¢ Clinical features ā€¢ obese ā€¢ Lateral thrust gait ā€¢ Deformity ā€¢ Genu varum - acute proximal ā€¢ Internal tibial torsion ā€¢ Procurvatum (flexion deformity) ā€¢ Unilateral or bilateral ā€¢ LLD (mostly unilateral) ā€¢ Cover up test (infantile) ā€¢ Patient supine & lower extremities fully extended with the patella up. ā€¢ Physiologic bowing is indicated by a valgus proximal tibia, while a Blount's patient will have a neutral or varus proximal tibia. *A toddler may appear bow-legged if both knees and hips are flexed when walking; a gentle repositioning of the hips and knees with patella facing up will correct it (NEVER COMMENT ON ANGLE IF FFD PRESENT)
  • 10. When are radiographs required? ā€¢ Most children referred DO NOT require ā€¢ Unilateral bowing ā€¢ Features not associated with physiological bowing (as mentioned before) ā€¢ Varus progresses *Radiographic features are only present in Blountā€™s after age 18months (*Staheli)
  • 11. ā€¢ Imaging ā€¢ Long limb standing view ā€¢ Tibia AP and lateral views *Patella facing forward ā€¢ Findings ā€¢ Sharp varus angulation in metaphysis ā€¢ Widening and irregular physeal line medially ā€¢ Medially slopped & irregular ossified epiphysis ā€¢ Prominent beaking of medial epiphysis with lucent cartilage island within beak ā€¢ Lateral subluxation of proximal end tibia
  • 12. *problem ā€“ lack of reproducibility and that it was not intended for use in determining the prognosis or type of treatment, as pointed out by the author. (*Staheli)
  • 14.
  • 15. ā€¢ Drennan metaphyseal-diaphyseal angle (MDA) ā€¢ Angle between ā€¢ Line connecting metaphyseal beak and ā€¢ Line perpendicular to the longitudinal axis of the tibia ā€¢ Meeting at most prominent lateral point ā€¢ >16ā€™ abnormal, 95% chance progression ā€¢ >10 : Diagnosis of Blountā€™s in Probable (Staheli) ā€¢ <10ā€™ : 95% chance of resolution ā€¢ Tibiofemoral angle ā€¢ Mechanical axis
  • 16. What indicates progression is likely? ā€¢ Severity of the deformity at presentation ā€¢ Obesity ā€“ BMI >22 *BMI > 22 + MDA > 10 --- Strongly suggestive of progression OR MDA > 16 *Selvadurai Nayagam
  • 17. What problems need to be managed? ā€¢ Tibial Deformity ā€¢ Varus + Internal Tibial Torsion + Flexion ā€¢ Joint Deformity ā€¢ Medial tibial hemiplateau depression ā€¢ Mostly in late stage ā€¢ Results in recurrence & early degenerative joint disease (DJD) ā€¢ Physeal Bar ā€¢ LLD ā€¢ Mainly in unilateral with significant physeal growth retardation ā€¢ Limb lengthening not a great option with underlying joint instability & deformity ā€¢ Close f/up for ideal chance for an epiphysiodesis of opposite extremity. ā€¢ Recurrence ā€¢ Obesity, Ligament laxity, Langenskiold stage IV above, presence of bone bar and undercorrection (Staheli) ā€¢ Hence why DIAGNOSE EARLY & INTERVENE!! ā€¢ Neurovascular complications ā€¢ High risk of compartment syndrome & peroneal nerve palsy ā€¢ Anterior compartment release recommended during index procedure *Randall Loder
  • 18. Our Aim of treatment? 1. Restore normal tibial alignment 2. Restore normal mechanical axis 3. Correct joint deformity ā€¢ If articular surface is defective ā€“ need to correct 4. Equalize limb length ā€¢ Differing knee levels predisposes to early DJD 5. Maintain correction, prevent recurrence ā€¢ Best chance of avoiding recurrence ā€“ early correction of deformity! 6. Prevent neurovascular complications *Randall Loder
  • 19. Treatment Options (Non-operative) ā€¢ Mainstay treatment for infantile blountā€™s of Lā€™skiold stage I & II ā€¢ Above knee non articulated KAFO ā€“ custom moulded to provide 3 point fixation ā€¢ Takes ~ 12-18months of use to resolve with success rates of 90% (when presenting deformity is mild to moderate) ā€¢ If fail by age of 4 OR progresses to Lā€™skiold stage III --- immediate tibial osteotomy is indicated (Randall Loder) *Selvadurai ā€¢ No data to confirm effectiveness ā€¢ Only for younger than 3 & Stage I or II ā€¢ Multiple problems associated : ā€¢ Use during daytime can be difficult & uncomfortable leading to poor compliance ā€¢ Moulding is difficult & corrective force over knee may not be tolerated ā€¢ Locks knee in extension ā€“ unphysiological & uncomfortable & stretches ligaments ā€¢ May affect sleep patterns causing irritable child/behavior changes *Staheli
  • 20.
  • 21. Proximal Tibial Osteotomy ā€¢ Most widely used surgery ā€¢ Infantile Blountā€™s ā€“ MUST be below tibial tuberosity 1. Acute corrective ā€¢ With internal fixator ā€¢ Kwiring in younger ā€¢ Plating in older ā€¢ With external fixator ā€“preferred for ability to fine tune deformity post op. ā€¢ Often with prophylactic anterior compartment fasciotomy 2. Gradual corrective ā€¢ With external fixator ā€¢ Less risk of compartment syndrome
  • 22.
  • 23. Physeal elevating osteotomy ā€¢ Significant depression of medial tibial epiphysis - Langenskiƶld V / VI ā€¢ Sparing physis ā€¢ When child approaching skeletal maturity ā€¢ Evidence of premature physeal arrest
  • 24. *Medial Plateau Elevation ā€¢ Tilted ā€˜pitched roofā€™ appearance of the tibial Joint line --- misleading ā€¢ Could be an unossified cartilage appearing as incomplete medial tibial condyle ā€¢ MRI or arthrogram will show true joint level *comparison of xray of knee taken in supine & weight bearing! ā€¢ In true underdeveloped medial tibial condyle --- clinically significant laxity to valgus stressing can be exhibited *Selvadurai
  • 25. Physeal Bar Resection ā€¢ Young patients who has developed physeal bar ā€“ resection can be considered along with repeat osteotomy ā€¢ Results are often disappointing ā€¢ In patients with at least 4 years of growth remaining (Orthobullets) ā€¢ Interpositional material - fat
  • 26. Growth modulating ā€¢ Proximal lateral tibia hemiepiphyseodesis ā€¢ ā‰„ 2 years of growth remaining ā€¢ 2 types I. Temporary ā€¢ Extraperiosteal 8 plate or staples ā€¢ Slight overcorrection to valgus to account for rebound growth upon removal II. Permanent ā€¢ Timing is crucial
  • 27.
  • 28.
  • 29. Complications ā€¢ Proximal tibial recurvatum ā€¢ injury to proximal tibial physis at level of tibial tubercle ļƒ  hyperextension instability of knee ā€¢ Anterior tibial artery injury ā€¢ Proximal tibial osteotomy must be performed distal to tibial tubercle, near the level of trifurcation of popliteal artery ā€¢ 29% injured in osteotomy procedure ā€¢ Compartment syndrome ā€¢ Prophylactic fasciotomy of all compartment should be performed during all osteotomy procedures ā€¢ Postop neurovascular surveillance for 1st 48H ā€¢ Peroneal nerve palsy ā€¢ Infections ā€¢ Iatrogenic fractures ā€¢ Loss of correction ā€¢ Recurrence ā€¢ Progression

Editor's Notes

  1. lateral view in full extension is preferred!!