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CTEV-ETIOLOGY ,PATHOANATOMY
,CLASSIFICATION ,IMAGING
Dr.Sushil Pokhrel (ms ortho resident
Congenital club foot
-Rigid deformity present at birth characterized
by ankle equinus , hind foot varus ,midfoot
cavus, forefoot adduction,usually calf muscle
underdevelopment
History of club foot
• Club foot – was first described in Egyptian
tomb painting
• First written description of club foot given by
Hippocrates in 400 B.C.
Epidemology
- 1-2 per 1000 birth
- Male> female
- 50 % cases are bilateral
- In unilateral cases –right>left foot
- Single parent affected have 3-4% while both
affected parent 30 % chance of club foot
- Certain ethnicities such as the Hawaiians and
Maori more affected and lowest in Chinese
Etiology
Etiology of idiopathic clubfoot is multifactorial
and modulated significantly by developmental
aberrations early in limb bud development
Primary germplasm defect :
- defect at germplasm cartilaginous talar
anlage produces the dysmorphic neck and
navicular subluxation < 7 weeks
Developmental delay :Bohm et al.
-arrest in fetal foot development
-characteristic dysmorphic talar head and the
medial dislocation of the navicular have never
been observed at any stage of normal fetal
development
Intra-uterine enviromental causes :
smoking increase 1.5x for light smoker
3.9 x heavy smoker
more in who smoked in first trimester
- many factors have been associated :
Environmental
factor
•Early amniocentesis or CVS
•Amniotic fluid leakage and
oligohydramnios
•Cigratte smoking
•Seasonal viral infections
•Elevated maternal homocysteine
•Methylenetetrahydrofolate
reductase (MTHFR) gene
polymorphisms
Conclusions: Smoking, maternal obesity, family history, amniocentesis, and some
selective serotonin reuptake inhibitor exposures are the most clinically relevant
exposures associated with increased odds of clubfoot, with family history representing
the greatest risk
Neuromuscular unit abnormalitis :
-alter musculotendinous position and pressure on
tarsal bone relative to each other
-congenital fiber type disproportion with atrophy of type
I fibers found in both peroneal and triceps surae
histopathologic specimens
- supported by fact that increase incidance : peripheral
neuropathy ,myelopathic abnormalitis ,central cerebral
lesion
Heredity :
- autosomal dominant with incomplete
penetrance
-first degree relative has 20-30 times higher
incidance than in normal popupation
-in twin study ,monozygotic twins has
concordance rate of 33% as compared to
dizygotic twin has 3%
Gene and molecular abnormalitis:
- missense mutation in number of gene have
been idetified :
N-acetylation genes, NAT1 and NAT2
CYP1A1,
HOXA, HOXD, and IGFBP3,
CAND2 and WNT7a,
TBX4,caspases genes
Transcription factor PITX1
Retractin fibrosis
Zimny et .-identified myofibroblast like cells
in medial ligamentous complex and tibialis
posterior tendon insertion
- myofibroblast like cells seemed to create
disorder of ligaments resembling fibromatosis
-Ippolito and Ponseti, who identified an increase in
collagen fibers and fibroblastic cells in the
ligaments and tendons of a clubfoot.
Syndromic
•Arthrogryposis
•Diastrophic dysplasia
•Streeter dysplasia (constriction
band syndrome)
•Freeman-Sheldon syndrome
•Möbius syndrome
•Chromosomal deletion
•Autosomal trisomy
•Smith-Lemli-Opitz syndrome
•Larsen syndrome
•TARP syndrome
•Chondrodysplasia punctata
•Myotonic dystrophy
•Spinal muscular atrophy
•Neural tube defect
Pathoanatomy
• Scarpa in 1803 reported medial and plantar
displacement of the navicular, cuboid, and
calcaneus around talus
• Scarpa, Adams in 1866,and Elmslie in 1920,
emphasized the midtarsal subluxation -
navicular and cuboid displaced medially,with
plantar and medial rotation of the calcaneus
-Talonavicular subluxation and dislocation of
the head of the talus out of“socket”
(acetabulum pedis)
• Finally Ponseti, in defining clubfoot, has
emphasized the cavus component which is
due to pronation of forefoot in relation to
hindfoot
Talus (astragalus)
1.Head and neck deviated medially
and plantarward
1
2
2.Short neck , small body,head –
neck to body angle is decreased
3.Body of talus at superior
articular surface at ankle is
obliquely positioned into
equinous
3
Anterior part of talus displaced
from ankle joint onto dorsum
4.Radiographic appearance of the
ossification center of the talus is
delayed
6
6 Articular surface of head
articulates with medially displaced
navicular
Anterior and medial facets of the
subtalar joint are absent,fused, or
significantly misshapen
4
Herzenberg and colleagues, commented that the body of the
talus appeared to be externally rotated within the mortise
Talus is inverted ,plantarflex and adducted
Os calcis(calcaneus)
Altered position, small
Planterflexed into equinus,
also inverted to varus
Anterior articular surface is
medially deviated and
deformed
Calcaneus slips beneath the
head and neck of the talus
anterior to the ankle joint
Sustentaculum tali is
usually underdeveloped
Cuboid
-cuboid is displaced
medially on the
anterior end of the
calcaneus
Navicular
-Positioned medially and plantarward
-Medial tuberosity of the navicular
may be hypertrophied
Cueiforms and MT
-abnormal positioning is due to altered
position of navicular ,os calcis and talus
First MTP Joint
Metatarsal I is more in Plantar flexion than the
rest of the Metatarsals.
Resisting Talocalcaneal joint realignment
Posterior talocalcaneal
Peroneal tendon seath
Calcaneofibular
Superior peroneal retinaculum
Resisting realignment of TN joint
- posterior tibial tendon
-deltoid ligament (tibial navicular)
-calcaneonavicular ligament (spring ligament)
-Talonavicular capsule
- dorsal talonavicular ligament
-bifurcated (Y) ligament
-inferior extensor retinaculum,
- cubonavicular oblique ligament.
Bifurcate ligament
IR of the CalcaneoCuboid joint
• causes contracture of
-bifurcated (Y) ligament
- long plantar ligament
-plantar calcaneocuboid ligament
-navicular cuboid ligament
-inferior extensor retinaculum
-dorsal calcaneocuboid ligament
-cubonavicular ligament
Bifurcate ligament
Due to contarcture
Kinematic coupling
• Subtalar joint axis is mobile oblique axis
• Movements of one tarsal bone affects
movements of other
• Calcaneal adduction,inversion and flexon
occur together
• Calcaneal abduction ,eversion and extension
occur together
• Fore foot abduction causes calcaneal
abduction around talus
Calcaneo-Pedis Block
The entire forefoot moves with the calcaneus
around talus
 Abduction pressure on forefoot,
causes movement of calcaneum
into abduction
 As abduction is coupled with
eversion and extension of
calcanaeum :hind foot deformity correct
Clinical features
Types of club foot
1.Idiopathic club foot
isolated , unilateral or bilateral deformity
no underlying neuromuscular disorder
2.Newborn neuromuscular club foot
congenital myelopathy ,myelo-meningocele
3.Club foot with arthrogryposis
at least one other joint contracture should be present
4.Syndromal club foot
Mutliple connective tissue abnormalities eg diastrophic
dysplasia
Carroll et al . 2012 based on etiology
classified
• Postural
bening ,resolves with streching ,full pasive ROM
• Idiopathic
congenital club foot with variable severity
• Neurogenic –actually neuromuscular
associated with underlying neurologic or muscular disorder
• Syndromal
Skeletal dysplasia and connective tissue disorder tend to be
very rigid
Classification of clubfoot
based on management (ponsetti)
• Typical clubfoot:
1-Positional clubfoot
2-Delayed treated clubfoot
3-Recurrent typical clubfoot
4-Alternatively treated typical clubfoot
• Atypical clubfoot
1-Rigid or resistant atypical clubfoot:
stiff, short, chubby, with a deep crease in the sole of the foot and behind the ankle, and
have shortening of the first metatarsal with hyperextension of the metatarsal
phalangeal joint
2-Syndromic clubfoot
3-Neurogenic club foot
4-Acquired clubfoot
5-Teratologic clubfoot
-congenital tarsal synchondrosis
Differential diagnosis
Clinical index of severity
Harrold and walker (1983)
Grade I
foot could held at or beyond neutral position
Garde II
foot couldn’t be manually reduced to neutral but fixed
equinous or angle of varus was 20 or less
Garde III
fixed deformity more than 20 equinous or varus
Pirani score
• Consists of
- Midfoot contracture score (MFCS)-max 3
- Hindfoot contracture score (HFCS)-max 3
• Documentation on
- initial visit
- changing cast
- seperately for each foot
• Prognostic value , severity and monitor progress
of treatment
Mid foot contracture score(MFCS)
Hind foot contracture score(HFCS)
Conclusions: Higher Pirani scores were associated with late relapses, but HFCS is a
stronger predictor of potential late relapse. Close follow-up is advised for patients at risk.
Dimeglio’s Adduction
Muscle condition
Ponseti and Smoley Classification
Ankle
Dorsiflexion
(degrees)
Heel
Varus(Degrees)
Adduction of
fore part of
foot (degrees)
Tibial
Torsion(degrees)
Result
>10 0 0-10 0 Good
0-10 0-10 10-20 Moderate Acceptable
0 >10 >20 Severe Poor
Catterall’s system
Tendon contracture criteria
Imaging
• X ray : atypical, global neurologic or genetic,
resistant to non operative treatments
• In non ambulatory : simulated weight bearing
AP and stress dorsiflexion lateral both feet
• For older standing AP and Lateral
• Talocalcaneal angle decreases on both views
with increase in severity, on lateral parallel
• -
Fig: talocalcaneal angle on AP
Normal-30-55°Club foot
Fig talocalcaneal angle in stress dorsiflexon lateral
Club foot Normal-20-50°
• Tibiocalcaneal
angle : negative
Normal:10-40°
Talus–first metatarsal
angle : negative
Normal-5-15
Ultrasound
Prenatal assesment
-upto 86 %
between 20-23 weeks
Post natal assesment
-assessing medial malleolar navicular dsiatance
and calcaneo cuboid relationship
- used for post tenotomy tendon healing
Approch
Summary
• Club foot has equinus,varus,cavus and adduction deformity
• Mutiple etiology ,retraction fibrosis (crimp) latest accepetd theory
• Rotatory subluxation of TCN joint complex with talus in plantar flexon and
at subtalar complex in medial rotataion and inversion
• Calnaneal abduction is kinameticaly coupled with eversion
• Abduction of forefoot is associated with abduction and eversion of hind
foot around talus (calacneo pedal block)
• Typical – classic club foot can be unilateral or b/l otherwise normal infant
• Atypical club foot –associated with other problem.
• Rigid club foot have stiff, short ,chubby ,deep posterior and medial crease
with extenion of 1st MTP joint and small 1st MT in otherwise normal infant
• Pirani score consists of HFCS and MFCS ,higher score associated with
increase no.cast and high tenotomy rate
Referances
• Pediatric orthopedic deformities vol 2 springer
• Cambell 13 e
• Tachdjian pediatric orthopaedics 4e
• Ponseti method manual 3e
• Related articles ,internet

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Club foot

  • 2. Congenital club foot -Rigid deformity present at birth characterized by ankle equinus , hind foot varus ,midfoot cavus, forefoot adduction,usually calf muscle underdevelopment
  • 3. History of club foot • Club foot – was first described in Egyptian tomb painting • First written description of club foot given by Hippocrates in 400 B.C.
  • 4. Epidemology - 1-2 per 1000 birth - Male> female - 50 % cases are bilateral - In unilateral cases –right>left foot - Single parent affected have 3-4% while both affected parent 30 % chance of club foot - Certain ethnicities such as the Hawaiians and Maori more affected and lowest in Chinese
  • 5. Etiology Etiology of idiopathic clubfoot is multifactorial and modulated significantly by developmental aberrations early in limb bud development Primary germplasm defect : - defect at germplasm cartilaginous talar anlage produces the dysmorphic neck and navicular subluxation < 7 weeks
  • 6. Developmental delay :Bohm et al. -arrest in fetal foot development -characteristic dysmorphic talar head and the medial dislocation of the navicular have never been observed at any stage of normal fetal development
  • 7. Intra-uterine enviromental causes : smoking increase 1.5x for light smoker 3.9 x heavy smoker more in who smoked in first trimester - many factors have been associated :
  • 8. Environmental factor •Early amniocentesis or CVS •Amniotic fluid leakage and oligohydramnios •Cigratte smoking •Seasonal viral infections •Elevated maternal homocysteine •Methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms
  • 9. Conclusions: Smoking, maternal obesity, family history, amniocentesis, and some selective serotonin reuptake inhibitor exposures are the most clinically relevant exposures associated with increased odds of clubfoot, with family history representing the greatest risk
  • 10. Neuromuscular unit abnormalitis : -alter musculotendinous position and pressure on tarsal bone relative to each other -congenital fiber type disproportion with atrophy of type I fibers found in both peroneal and triceps surae histopathologic specimens - supported by fact that increase incidance : peripheral neuropathy ,myelopathic abnormalitis ,central cerebral lesion
  • 11. Heredity : - autosomal dominant with incomplete penetrance -first degree relative has 20-30 times higher incidance than in normal popupation -in twin study ,monozygotic twins has concordance rate of 33% as compared to dizygotic twin has 3%
  • 12. Gene and molecular abnormalitis: - missense mutation in number of gene have been idetified : N-acetylation genes, NAT1 and NAT2 CYP1A1, HOXA, HOXD, and IGFBP3, CAND2 and WNT7a, TBX4,caspases genes Transcription factor PITX1
  • 13. Retractin fibrosis Zimny et .-identified myofibroblast like cells in medial ligamentous complex and tibialis posterior tendon insertion - myofibroblast like cells seemed to create disorder of ligaments resembling fibromatosis -Ippolito and Ponseti, who identified an increase in collagen fibers and fibroblastic cells in the ligaments and tendons of a clubfoot.
  • 14. Syndromic •Arthrogryposis •Diastrophic dysplasia •Streeter dysplasia (constriction band syndrome) •Freeman-Sheldon syndrome •MĂśbius syndrome •Chromosomal deletion •Autosomal trisomy •Smith-Lemli-Opitz syndrome •Larsen syndrome •TARP syndrome •Chondrodysplasia punctata •Myotonic dystrophy •Spinal muscular atrophy •Neural tube defect
  • 15. Pathoanatomy • Scarpa in 1803 reported medial and plantar displacement of the navicular, cuboid, and calcaneus around talus • Scarpa, Adams in 1866,and Elmslie in 1920, emphasized the midtarsal subluxation - navicular and cuboid displaced medially,with plantar and medial rotation of the calcaneus
  • 16. -Talonavicular subluxation and dislocation of the head of the talus out of“socket” (acetabulum pedis) • Finally Ponseti, in defining clubfoot, has emphasized the cavus component which is due to pronation of forefoot in relation to hindfoot
  • 17. Talus (astragalus) 1.Head and neck deviated medially and plantarward 1 2 2.Short neck , small body,head – neck to body angle is decreased 3.Body of talus at superior articular surface at ankle is obliquely positioned into equinous 3 Anterior part of talus displaced from ankle joint onto dorsum
  • 18. 4.Radiographic appearance of the ossification center of the talus is delayed 6 6 Articular surface of head articulates with medially displaced navicular Anterior and medial facets of the subtalar joint are absent,fused, or significantly misshapen 4 Herzenberg and colleagues, commented that the body of the talus appeared to be externally rotated within the mortise Talus is inverted ,plantarflex and adducted
  • 19. Os calcis(calcaneus) Altered position, small Planterflexed into equinus, also inverted to varus Anterior articular surface is medially deviated and deformed Calcaneus slips beneath the head and neck of the talus anterior to the ankle joint Sustentaculum tali is usually underdeveloped
  • 20. Cuboid -cuboid is displaced medially on the anterior end of the calcaneus Navicular -Positioned medially and plantarward -Medial tuberosity of the navicular may be hypertrophied Cueiforms and MT -abnormal positioning is due to altered position of navicular ,os calcis and talus
  • 21.
  • 22. First MTP Joint Metatarsal I is more in Plantar flexion than the rest of the Metatarsals.
  • 23. Resisting Talocalcaneal joint realignment Posterior talocalcaneal Peroneal tendon seath Calcaneofibular Superior peroneal retinaculum
  • 24. Resisting realignment of TN joint - posterior tibial tendon -deltoid ligament (tibial navicular) -calcaneonavicular ligament (spring ligament) -Talonavicular capsule - dorsal talonavicular ligament -bifurcated (Y) ligament -inferior extensor retinaculum, - cubonavicular oblique ligament. Bifurcate ligament
  • 25. IR of the CalcaneoCuboid joint • causes contracture of -bifurcated (Y) ligament - long plantar ligament -plantar calcaneocuboid ligament -navicular cuboid ligament -inferior extensor retinaculum -dorsal calcaneocuboid ligament -cubonavicular ligament Bifurcate ligament Due to contarcture
  • 26. Kinematic coupling • Subtalar joint axis is mobile oblique axis • Movements of one tarsal bone affects movements of other • Calcaneal adduction,inversion and flexon occur together • Calcaneal abduction ,eversion and extension occur together • Fore foot abduction causes calcaneal abduction around talus
  • 27. Calcaneo-Pedis Block The entire forefoot moves with the calcaneus around talus  Abduction pressure on forefoot, causes movement of calcaneum into abduction  As abduction is coupled with eversion and extension of calcanaeum :hind foot deformity correct
  • 28.
  • 30.
  • 31. Types of club foot 1.Idiopathic club foot isolated , unilateral or bilateral deformity no underlying neuromuscular disorder 2.Newborn neuromuscular club foot congenital myelopathy ,myelo-meningocele 3.Club foot with arthrogryposis at least one other joint contracture should be present 4.Syndromal club foot Mutliple connective tissue abnormalities eg diastrophic dysplasia
  • 32. Carroll et al . 2012 based on etiology classified • Postural bening ,resolves with streching ,full pasive ROM • Idiopathic congenital club foot with variable severity • Neurogenic –actually neuromuscular associated with underlying neurologic or muscular disorder • Syndromal Skeletal dysplasia and connective tissue disorder tend to be very rigid
  • 33. Classification of clubfoot based on management (ponsetti) • Typical clubfoot: 1-Positional clubfoot 2-Delayed treated clubfoot 3-Recurrent typical clubfoot 4-Alternatively treated typical clubfoot
  • 34. • Atypical clubfoot 1-Rigid or resistant atypical clubfoot: stiff, short, chubby, with a deep crease in the sole of the foot and behind the ankle, and have shortening of the first metatarsal with hyperextension of the metatarsal phalangeal joint 2-Syndromic clubfoot 3-Neurogenic club foot 4-Acquired clubfoot 5-Teratologic clubfoot -congenital tarsal synchondrosis
  • 35.
  • 37. Clinical index of severity Harrold and walker (1983) Grade I foot could held at or beyond neutral position Garde II foot couldn’t be manually reduced to neutral but fixed equinous or angle of varus was 20 or less Garde III fixed deformity more than 20 equinous or varus
  • 38. Pirani score • Consists of - Midfoot contracture score (MFCS)-max 3 - Hindfoot contracture score (HFCS)-max 3 • Documentation on - initial visit - changing cast - seperately for each foot • Prognostic value , severity and monitor progress of treatment
  • 39. Mid foot contracture score(MFCS)
  • 40. Hind foot contracture score(HFCS)
  • 41.
  • 42.
  • 43.
  • 44. Conclusions: Higher Pirani scores were associated with late relapses, but HFCS is a stronger predictor of potential late relapse. Close follow-up is advised for patients at risk.
  • 47. Ponseti and Smoley Classification Ankle Dorsiflexion (degrees) Heel Varus(Degrees) Adduction of fore part of foot (degrees) Tibial Torsion(degrees) Result >10 0 0-10 0 Good 0-10 0-10 10-20 Moderate Acceptable 0 >10 >20 Severe Poor
  • 49. Imaging • X ray : atypical, global neurologic or genetic, resistant to non operative treatments • In non ambulatory : simulated weight bearing AP and stress dorsiflexion lateral both feet • For older standing AP and Lateral • Talocalcaneal angle decreases on both views with increase in severity, on lateral parallel
  • 50. • - Fig: talocalcaneal angle on AP Normal-30-55°Club foot Fig talocalcaneal angle in stress dorsiflexon lateral Club foot Normal-20-50°
  • 51. • Tibiocalcaneal angle : negative Normal:10-40° Talus–first metatarsal angle : negative Normal-5-15
  • 52. Ultrasound Prenatal assesment -upto 86 % between 20-23 weeks Post natal assesment -assessing medial malleolar navicular dsiatance and calcaneo cuboid relationship - used for post tenotomy tendon healing
  • 54.
  • 55. Summary • Club foot has equinus,varus,cavus and adduction deformity • Mutiple etiology ,retraction fibrosis (crimp) latest accepetd theory • Rotatory subluxation of TCN joint complex with talus in plantar flexon and at subtalar complex in medial rotataion and inversion • Calnaneal abduction is kinameticaly coupled with eversion • Abduction of forefoot is associated with abduction and eversion of hind foot around talus (calacneo pedal block) • Typical – classic club foot can be unilateral or b/l otherwise normal infant • Atypical club foot –associated with other problem. • Rigid club foot have stiff, short ,chubby ,deep posterior and medial crease with extenion of 1st MTP joint and small 1st MT in otherwise normal infant • Pirani score consists of HFCS and MFCS ,higher score associated with increase no.cast and high tenotomy rate
  • 56. Referances • Pediatric orthopedic deformities vol 2 springer • Cambell 13 e • Tachdjian pediatric orthopaedics 4e • Ponseti method manual 3e • Related articles ,internet