Paeds Non-Hip Flashcards
What are risk factors for the development of clubfoot?
[CORR (2009) 467:1146–1153]
- Family history
- Boys
- Race (highest in Hawaiians and Maoris)
- Early amniocentesis (<13 weeks)
- Oligohydramnios
- Exposure to cigarette smoke inutero
What is the Pirani scoring system of clubfoot and what can it predict?
- Six signs are scored from 0 (no abnormality), 0.5 (moderate abnormality), 1 (severe abnormality)
- 3 signs related to the hindfoot
- Severity of the posterior crease
- Emptiness of the heel
- Rigidity of the equinus
- 3 signs related to the midfoot
- Curvature of the lateral border of the foot
- Severity of the medial crease
- Position of the lateral part of the head of the talus
- Predicts need for tenotomy
* 85% of feet with a score above 5 required tenotomy
What is the main radiographic feature in clubfoot?
[Orthobullets]
Hindfoot parallelism
- Talus and calcaneus are parallel/less divergent on AP and lateral
What are the 4 components of the clubfoot deformity?
[CORR (2009) 467:1146–1153]
CAVE
- Midfoot cavus
- Forefoot adductus
- Hindfoot varus
- Hindfoot equinus
Describe the pathoanatomy resulting in the deformity in clubfoot
[J Am Acad Orthop Surg 2003;11:392-402]
- Navicular displaces medially (articulates with the medial head of talus)
- Cuboid is adducted infront of the calcaneus
- Metatarsals are adducted on the midfoot
- Calcaneus is adducted and inverted around the talus medially
- Forefoot is pronated relative to the hindfoot (causing cavus)
- Tight muscles (gastroc/soleus, tib post, FHL, FDL)
- Tight posteromedial capsule and ligaments
Describe the Ponsetti method for clubfoot correction.
[J Am Acad Orthop Surg 2003;11:392-402] [J Am Acad Orthop Surg 2010;18:486-493]
- Serial foot manipulation followed by casting to maintain the correction with foot abduction orthosis as the final stage
- Manipulations are held for 1-3 minutes followed by above knee plaster cast with knee at 90° flexion
- Weekly cast change and manipulation
- ~6 cast changes required to correct most clubfeet
- Ponsetti method started ideally within the first month of life
- The order of foot deformity correction is cavus, adductus, varus, equinus (CAVE)
- Cavus correction
- Usually achieved with the first cast
- Technique – pressure under first metatarsal head to elevate it in line with other metatarsals
- Forefoot adduction and hindfoot varus corrected simultaneously
- With foot in slight supination and equinus the forefoot is abducted while stabilizing counterpressure is applied to the lateral head of the talus
- This will simultaneous correct adduction and hindfoot varus as the calcaneus abducts freely under the talus (important to avoid max dorsiflexion)
- Equinus correction
- Perform when hindfoot is neutral or slight valgus and forefoot is abducted 70° relative to the leg
- Technique – progressive dorsiflexion applied with broad pressure over sole of foot
- Heel cord tenotomy
- Required in ~75% of cases
- Performed after 4-6 weeks of casting
- Percutaneous tenotomy performed in clinic or OR followed by cast immobilization for 3-4 weeks
- Foot abduction orthoses (‘boots and bars’, Denis-Browne bar)
- Required to prevent relapse
- 15° dorsiflexion needed for proper fit
- Clubfoot placed in 70° abduction, unaffected foot in 40° abduction with feet shoulder width apart
- Worn full time (23 hours/day) for 3 months followed by bed and naptime use until age 4 (range 2-5)
- Patient should be followed every 3 months after bracing starts until 2 years of age
What is the most common factor related to clubfoot relapse?
[J Am Acad Orthop Surg 2010;18:486-493]
Failure to comply with foot abduction orthoses
By what age is clubfoot relapse most likely to occur?
[Am Acad Orthop Surg 2017;25:195-203]
Most frequently by age 5
- Rare after age 5 and extremely rare after 7
What are the signs of clubfoot relapse?
[Am Acad Orthop Surg 2017;25:195-203]
- Loss of dorsiflexion is the earliest sign
- Older infants – mild forefoot adductus, cavus, heel varus and limited abduction
- Walking child – increased lateral contact during stance phase, heel varus, inward deviation of toes, and dynamic supination during swing phase
How do you manage clubfoot relapse?
[Am Acad Orthop Surg 2017;25:195-203]
- Mild dorsiflexion loss
* If early, home exercise program and increased foot abduction orthosis use - If <10° dorsiflexion
* Repeat manipulation and casting as per Ponsetti (2-3 casts usually required changed weekly)
* Repeat tenotomy if 15° dorsiflexion not achieved
* Resume foot abduction orthosis - If >2.5 years of age:
* Consider anterior tibial tendon transfer to 3rd cuneiform (now sufficiently ossified)
* First requires obtaining original correction with manipulation and casting (2-3 casts) and heel cord tenotomy if <10° dorsiflexion
* Anterior tendon should never be split (split weakens eversion power)
* Technique for anterior tibial tendon transfer:- 3-4cm incision starting just distal to the navicular and extending proximal inline with tibialis anterior tendon is made
- The tibialis anterior tendon is released from the base of the 1st MT and a whip stitch is placed in the tendon
- A 2nd incision is made over the lateral cuneiform and localized with fluoro guidance, once confirmed a drill hole is made dorsal to plantar
- The tendon is tunneled subcutaneously from the medial to lateral incision
- Keith needles are threaded on to the sutures and passed through the drill hole and out through the plantar aspect of the foot
- The ankle is dorsiflexed and everted and the sutures are tied over a button
- The patient is casted for 6 weeks
- If age 4-9 with well-formed medial cuneiform ossific nucleus:
* Consider closing wedge osteotomy through the cuboid and medial opening wedge osteotomy through the cuneiforms (flip-flop technique) - Patients whose parents are unwilling to allow repeated cast and brace treatment and patients with feet that are otherwise refractory to the Ponseti method:
* Consider posteromedial release (required in less than 5%)- Highly associated with development of pain, stiffness and weakness in late adolescence and early adulthood
* Technique for posteromedial release [Lovell and Winter]: - Cincinnati incision
- Extends medially from navicular, posteriorly just below medial malleolus and 1cm proximal to the posterior heel crease, laterally just below lateral malleolus ending at the sinus tarsi
- Releases include:
- Heel cord lengthening
- Posterior release of ankle and subtalar joint
- Plantar fascia
- Abductor hallucis
- +/- Tib post tendon lengthening
- +/- Talonavicular joint capsule release
- +/- FHL and FDL release
- Highly associated with development of pain, stiffness and weakness in late adolescence and early adulthood
What are the associated conditions with congenital knee dislocation?
[JAAOS 2009;17:112-122]
- Ipsilateral DDH (70-100% of cases)
- Clubfoot
- Arthrogryposis
- Myelodysplasia
- Larsen syndrome
What is the classification of congenital knee dislocation?
[JAAOS 2009;17:112-122]
- Grade 1 = recurvatum
- Grade 2 = subluxation
- Grade 3 = complete dislocation
What is the clinical presentation of congenital knee dislocation?
[JAAOS 2009;17:112-122]
- Knee hyperextension
- Inability to flex knee in complete dislocation
What is the management of congenital knee dislocation?
[JAAOS 2009;17:112-122]
- Nonoperative
- Closed reduction and serial casting
- Closed reduction achieved by traction followed by knee flexion
- Serial casting in progressive knee flexion
- Operative
- Failure of nonoperative
- <30° of flexion after 3 months of casting
- Performed at ~6 months of age
- Involves open reduction and quadriceps lengthening
- Percutaneous quadriceps release
- Open V-Y quadriceps advancement
- Possible femoral shortening osteotomy (relative lengthening of extensor mechanism) [POSNA]
What is the management of ipsilateral congenital knee dislocation and DDH?
[JAAOS 2009;17:112-122]
- Treat congenital knee dislocation first
- Once adequate knee flexion achieved patient can be placed in Pavlik harness
* Pavlik harness helps to hold knee in flexion and maintain hip reduced
What is the definition of congenital dislocation of the patella?
Congenital, irreducible lateral patellar dislocation present at birth
What are the findings in patients with congenital dislocation of the patella?
- Anatomical
- Tight lateral structures (capsule, ITB, etc)
- Quadriceps contracture
- Lateralized patellar tendon insertion on tibia
- Hypoplastic patella
- Shallow trochlear groove
- Clinical
- Knee flexion contracture
- Genu valgum
- External tibial torsion
- Prominent femoral condyles
What is the management of congenital patellar dislocation of the patella?
Operative
- Principles:
- Extensive lateral release
- ITB, capsule, biceps femoris
- VY lengthening of quadriceps
- Medial capsule imbrication OR MPFL reconstruction
- Lateral patellar tendon insertion addressed via:
- Roux-Goldthwait procedure:
- Lateral half of patellar tendon detached from tibial tubercle, passed deep to remaining patellar tendon and attached medial to the medial half of the patellar tendon
- Patellar tendon periosteal sleeve medialization (complete medialization of patellar tendon)
- Roux-Goldthwait procedure:
- Extensive lateral release
What is developmental coxa vara?
[Lovell and Winter]
Decreased femoral neck shaft angle believed to be a result of a primary defect in endochondral ossification of the medial part of the femoral neck
What is the presentation of developmental coxa vara?
[Lovell and Winter]
Painless limp (unilateral) or waddling gait (bilateral)
- Due to abductor weakness and minor LLD in unilateral cases
What are the radiographic features of developmental coxa vara?
[Lovell and Winter]
- Decreased femoral neck-shaft angle (<120o)
- Vertical position of physeal plate
- Triangular metaphyseal fragment in inferior femoral neck with associated inverted Y appearance
- Shortened femoral neck
- Decrease in normal anteversion
How is the amount of varus deformity in developmental coxa vara quantified on plain films?
[Lovell and Winter]
Hilgenreiner epiphyseal angle (H-E)
- Angle between the physeal plate and Hilgenreiner line
- Normal = <25° (average 16°)
- Coxa vara = 40-70°
What is the management of developmental coxa vara?
[Lovell and Winter]
- Nonoperative
- H-E angle <45
- H-E angle 45-59 and asymptomatic
2.Operative
- H-E angle >60
- H-E angle 45-59 and symptomatic
- Symptomatic limp, Trendelenburg gait, or progressive deformity
- Neck shaft angle <100
- Technique:
- Valgus-producing proximal femoral osteotomy
What is the goal of correction in developmental coxa vara?
- <38° H-E angle [Orthobullets]
- 16° H-E angle [Lovell and Winter]
What are the associated conditions with PFFD?
[Lovell and Winter]
- PFFD is associated with fibular deficiency in 70% to 80% of cases
- Associated conditions are similar to that of fibular hemimelia
What is the most widely used classification for PFFD?
[Lovell and Winter]
- Aitken
- Class A
- Femoral head ossification delayed
- Acetabulum well formed
- Femur is short
- Proximal femur is at or above level of acetabulum
- Class B
- Femoral head ossification delayed
- Mild acetabular dysplasia
- Proximal femur is above level of acetabulum
- Subtrochanter region will not ossify and forms pseudoarthrosis
- Class C
- Femoral head does not form
- Severe acetabular dysplasia
- Femur is shorter than B
- Entire proximal femur does not form
- Class D
- Femoral head does not form
- Acetabulum does not form
- Distal femoral condyles are at level of acetabulum
What is the management of PFFD?
[Lovell and Winter]
- Nonoperative
- ‘bridge treatment’ from time patient begins to walk to surgical treatment (~3 years of age)
- Bilateral PFFD
- Operative
- 3 main procedures (stable hip)
- Knee fusion with foot ablation
- Indications:
- >20cm of LLD at maturity
- Foot above level of contralateral knee
- Ankle has <60° arc of motion
- Stable hip
- Foot ablation (amputation) through Boyd or Syme amputation
- Indications:
- Van Nes rotationplasty
- Indications:
- >20cm of LLD at maturity
- Foot at level of contralateral knee
- Ankle has >60° arc of motion
- Stable hip
- Technique:
- Leg is rotated 180° through the knee arthrodesis
- Indications:
- Limb lengthening
- Indications:
- <20cm of LLD at maturity
- Stable hip
- Good knee, ankle, foot function
- Indications:
- Knee fusion with foot ablation
- Unstable hip (Aitken C/D)
- Possible iliofemoral fusion (knee then functions as hip and ankle as knee)
What is the difference between true, apparent and functional LLD?
[Lovell and Winter]
- True (structural) LLD = anatomic difference in length of one of the segments of the lower extremity (femur, tibia, foot)
- Apparent (postural) LLD = discrepancies that are not true differences in anatomic segment lengths (eg. knee flexion contracture)
- Functional LLD = the sum of the true and apparent leg-length discrepancy (most important in treatment decisions)
What are the causes of LLD?
[Lovell and Winter]
- Congenital
* PFFD, fibular hemimelia, tibia hemimelia, posteromedial tibial bowing, clubfoot, hemihypertrophy - Acquired
* Trauma (growth arrest, overgrowth, malunion), radiation, tumor, infection
What are clinical methods to assess LLD?
[Lovell and Winter]
- True leg length
* Measure from ASIS to medial malleolus - Apparent leg length
* Measure from umbilicus to medial malleolus (affected by pelvic obliquity) - Galeazzi sign
* Difference in knee heights suggests difference in femoral lengths - Heel pad height
* With patient prone assess difference in heights of the heel pads, suggests difference in tibia/fibula length - Block method
* Place blocks under foot of short leg until pelvis level, height of block indicates LLD
What are the radiographic methods used to assess LLD?
[Lovell and Winter]
- Teleoroentgenogram (C)
- Standing alignment film (35cm × 90cm) taken with a single exposure at a distance of 2 m centered on the knee joint
- Advantages – angular deformity assessment
- Disadvantage – magnification error
- Orthoroentgenogram (A)
- Three separate exposures at the hip, knee, and ankle all placed on the same longstanding film with ruler centered over each joint
- Advantages – no magnification error
- Disadvantages – cannot assess angular deformity
- Scanogram (B)
- All three joints are placed on one smaller film; obtained having the film and x-ray source move
- Advantages – no magnification error
- Disadvantages – cannot assess angular deformity
- CT scanogram
- CT scan through hip, knee, and ankle to assess length
- Advantages – accurate length measurements in setting of joint contractures
- Disadvantages – cannot assess angular deformity
What is a method to assess skeletal age (rather than chronological age)?
[Lovell and Winter]
Greulich and Pyle
- Bone age x-ray (left hand)
- The clinician or radiologist can compare this child’s x-ray with those in the atlas and develop a bone age with a given standard deviation
What are methods used to predict the final LLD and timing of surgical intervention?
- Moseley Straight line graph [www.pedipod.com]
- Multiplier method
- Estimation method
What is the relative growth contribution of the growth plates of the lower extremity?
[Lovell and Winter]
- Proximal femur = 3mm/year
- Distal femur = 9mm/year
- Proximal tibia = 6mm/year
- Distal tibia = 5mm/year
What are the treatment options based on predicted LLD?
[Lovell and Winter]
- <2cm
- Observation
- Shoe lift
- 2-5cm
- Shoe lift
- Contralateral epiphysiodesis
- Contralateral skeletal shortening (usually for patients with inadequate growth remaining for epiphysiodesis)
3. >5-20cm - Limb lengthening +/- contralateral epiphysiodesis
4. >20cm - Amputation and prosthetic fitting
What are some principles for limb lengthening?
[Orthobullets]
- Initiation
- Ensure stable joint above and below prior to lengthening
- If joint subluxes during lengthening extend the frame across the joint
- Perform corticotomy and place fixator
- Metaphyseal corticotomy is preferred due to good blood supply
- Percutaneous corticotomy that minimizes trauma to the periosteum and preserves the blood supply of the marrow and periosteum
- Distraction
- Wait 5-7 days then begin distraction (allows for neovascularization of corticotomy site)
- Distract ~ 1 mm/day in four 0.25mm increments daily
- Do not distract more than 20% of the bones original length
- Following distraction keep fixator on for as many days as you lengthened
- Concurrent procedures
- May lengthen over a nail so ex-fix can be removed sooner
- Lengthening often combined with a shortening procedure (epiphysiodesis, ostectomy) on long side
- Note:
* Type of bone formation during distraction osteogenesis = intramembranous ossification
What are the causes of genu valgum?
[Orthobullets]
- Bilateral genu valgum
- Physiologic
- Renal osteodystrophy (renal rickets)
- Skeletal dysplasia (Morquio syndrome, Spondyloepiphyseal dysplasia, Chondroctodermal dysplasia)
- Unilateral genu valgum
- Physeal injury from trauma, infection, or vascular insult
- Proximal metaphyseal tibia fracture (Cozen fracture)
- Benign tumors
- Fibrous dysplasia
- Osteochondromas
- Ollier’s disease
During development when does maximum genu valgum occur?
[Lovell and Winter]
3-4 years of age (8-10 degrees)
- Corrects to stable adult valgus by age 6-7 (5-7 degrees)
What is the management of genu valgum?
[Lovell and Winter]
- Nonoperative
* Observation (bracing not indicated) - Operative
- Indications:
- Mechanical axis passes through zone 3 (beyond the lateral tibial plateau)
- .Mechanical axis passes through zone 2 (outer half of the lateral plateau) in presence of pain
- Timing :
- Usually deferred to 10-11 years of age
- Options
- Hemiepiphysiodesis
- Requires 1-2 years of growth remaining
- Eight-plate (guided growth plate) or staple placed extraperiosteally, parallel to physis, central on lateral view
- Placed medially in femur +/- tibia depending on location of deformity
- Normally the mLDFA and MPTA = 87°
- Monitor every 3 months with radiographs, remove once mechanical axis passes through central 1/3 knee joint
- Distal femur varus osteotomy
- Performed when inadequate growth remaining, severe deformity or immediate correction desired
- Hemiepiphysiodesis
What is the age of onset of infantile Blounts?
2-5
What percentage of patients with infantile Blount’s have bilateral involvement?
~50% (may not be symmetric)
What are the risk factors for infantile Blounts?
- Obesity
- Hispanic and black children
- ?early walkers
- ?Vit D deficiency
- ?zinc deficiency
What is the pathology and resulting tibial deformity in infantile Blount’s?
Spontaneous deceleration of growth occurs at the posteromedial proximal tibial physis resulting in:
- Varus/flexion/internal rotational deformity
- Medial and posterior “sloping” of the proximal tibial epiphysis
- In unilateral cases, variable relative tibial shortening
What is the histological change that occurs in the proximal tibial physis in infantile Blount’s?
[JAAOS 2013;21:408-418]
Disruption of the normal columnar architecture of the physis, replacement of physeal cartilage by fibrous tissue and, in the most severe form, osseous bridging (physeal arrest) between the epiphysis and metaphysis
What are the clinical features of infantile blounts?
[JAAOS 2013;21:408-418]
- Deformity:
- Proximal tibia varus
- Increased internal tibial torsion
- In unilateral cases, leg length inequality
- Palpable prominence or “beaking” of the proximal medial tibial epiphysis and metaphysis
- No tenderness, knee effusion, or restriction of joint motion
- Dynamic test:
- Lateral thrust may be noted in the child’s gait
- Single limb stance, the varus deformity acutely accentuates as if the knee were unstable
What are the classic radiographic features of infantile blount?
[JAAOS 2013;21:408-418] )
- Sharp varus angulation of the tibia metaphysis
- Widening and irregularity of the medial aspect of the growth plate
- Medial sloping and irregular ossification of the epiphysis
- Beaking of the medial part of the epiphysis
- The distal femur is usually normal (if abnormal it is a valgus deformity
What is the radiographic classification of infantile blounts?
[JAAOS 2013;21:408-418]
Langenskiold’s Classification
- Stage I: medio-distal beaking of the upper proximal tibial metaphysis.
- Stage II: wedging of the medial part of the upper tibial epiphyseal secondary ossification center plus a saucer shaped defect of the upper surface of the metaphyseal beak due to its dissolution, fragmentation & collapse.
- Stage III: stepping of the infero-medial border of the secondary ossification center but without extending distal to the physeal plate level plus deeping of the metaphyseal saucer into a step in the medial metaphysis.
- Stage IV: the epiphyseal secondary ossification center passes more distally and cross distal to the physeal level to fill the metaphyseal step.
- Stage V: separation of the most medial part of the ossification center from the bulk of the secondary ossification center and resides now in the depth of the metaphysiseal step below the physis.
- This is radiologically expressed as either a horizontal cleft (double epiphysis) or complete absence of the medial secondary ossification center as it will be overshadowed by the upper medial tibial metaphysis.
- Stage VI: medial epiphyseal plate closure with a bony bridge
What is the radiographic measurement of proximal tibia vara?
Metaphyseal-Diaphyseal Angle (Drennan)
- ≤9° suggest physiologic varus
- 95% chance of natural resolution of bowing
- ≥16° likely indicate infantile Blount disease
- 95% chance of progression
- >°9 and <16° are considered indeterminant and merit careful observation
What is the differential diagnosis of infantile blounts?
[JAAOS 2013;21:408-418]
- Persistent physiologic varus
- Vitamin D-deficiency rickets
- Renal osteodystrophy
- Vitamin D-resistant (hypophosphatemic) rickets
- Metaphyseal dysostosis
- SED or MED
- Thrombocytopenia-absent radius syndrome
- Focal fibrocartilaginous defect
- Proximal tibial physeal injury (eg. infection, fracture, irradiation)
What is the management of infantile blounts?
[JAAOS 2013;21:408-418]
Nonsurgical
- Anti-varus long leg bracing during ambulation
- Indications:
- Patients aged ≤3 years with unilateral involvement at stage 2, clear radiographic evidence of infantile Blount disease, or lateral thrust with ambulation
Surgical
- Indications:
- Patient is age ≥4 years
- Langenskiöld stage III or greater
- Demonstrates progressive radiographic deformity
- Options:
- High tibial and fibula osteotomy*
- Treatment of choice for progressive varus or brace failure
- Goal = Full to overcorrection of varus, flexion, and internal rotational deformities of the tibia
- Tibial osteotomy is performed below the tibial tubercle
- Closing wedge, opening wedge, dome, serrated, and inclined acceptable
- Stabilization with 1-2 pins plus long leg cast
- Lateral translation of the distal fragment to lateralize the mechanical axis of the limb is advisable
- Growth modulation
- Alternative to HTO
- Consider in young patients with less than Langenskiold stage IV
- Extraperiosteal tension band plate or staple across the lateral tibial physis
- Slight overcorrection such that the mechanical axis is lateral to the centre of the knee
- Note – does not correct the internal tibial torsion
- Physeal arrest resection
- Hemiplateau elevation
- Angular deformity correction and lengthening
- High tibial and fibula osteotomy*
What is the age of onset of adolescent blounts?
[JAAOS 2013;21:408-418]
>10
What percentage of adolescent blount patients have bilateral disease?
[JAAOS 2013;21:408-418]
Rare, usually unilateral
What are the clinical features of adolescent blounts?
[JAAOS 2013;21:408-418]
- Progressive varus deformity
- With or without knee pain
- Leg length discrepancy with unilateral or asymmetric bilateral
- Variable internal tibial torsion and proximal tibial flexion (procurvatum) deformities
- Limp or lateral thrust
What are the radiographic features of adolescent blounts?
[JAAOS 2013;21:408-418]
- Tibia findings
- Proximal varus deformity
- Widening or lucency of the medial tibial physis
- Proximal procurvatum
- Distal valgus
- Femur findings
- Distal femoral physeal growth disturbance
- Varus deformity
What is the management of adolescent blounts?
[JAAOS 2013;21:408-418]
- Nonsurgical management not indicated
- Surgical management
- Proximal tibial osteotomy with internal or external fixation
- Correction of the deformity, rather than overcorrection, is the goal of surgery because the proximal tibial physis typically grows symmetrically postoperatively
- Distal femoral varus deformity and distal tibia valgus deformity may need to be addressed
Compare and contrast infantile and adolescent blounts
What is the classification of tibia hemimelia?
[Lovell and Winter][Orthobullets]
Jones Classification
- Type 1a
- No proximal tibia visible on radiograph
- Extensor mechanism absent
- Hypoplastic distal femoral epiphysis
- Type 1b
- Proximal tibia eventually ossifies and extensor mechanism will often function
- Distal femoral epiphysis appears normal
- Type 2
- Proximal tibia present at birth but short tibia
- Distal tibia fails to ossify
- Type 3
- Diaphyseal and distal tibia present but proximal tibia absent
- Type 4
- Short tibia
- Fibula migrated proximal
- Diastasis of distal tib-fib joint
What are the clinical features of tibia hemimelia?
[Lovell and Winter]
- Shortened tibia
- Rigid equinovarus-supinated foot pointing toward the perineum
- Prominent fibular head
What is the treatment of tibia hemimelia?
[Lovell and Winter]
Nonoperative
- Indications:
- Bilateral tibial deficiency with active knee extension and acceptable foot position
Operative
- Type 1A and other types with no extensor mechanism
- Knee disarticulation with prosthetic fitting
- Type 1B and 2 with intact extensor mechanism
- Tibiofibular synostosis with modified Syme amputation
- Type 3
- Rare (limited data)
- Ankle disarticulation and prosthetic fitting
- Type 4
- Projected LLD <5cm = soft tissue correction of foot deformity + later contralateral epiphysiodesis
- Projected LLD >5cm = Syme amputation and prosthetic fitting*
What is the most common congenital long bone deficiency?
[JAAOS 2014;22:246-255]
Fibular hemimelia
What are the associated anomalies of fibular hemimelia?
[JAAOS 2014;22:246-255]
- Foot and ankle
- Absent lateral rays
- Tarsal coalition
- Ball and socket ankle
- Ankle instability
- Equinovalgus (equinovarus less frequently)
- Lower extremity
- Fibular hemimelia/amelia
- Anteromedial tibial bowing
- Hypoplastic lateral femoral condyle
- Genu valgum
- ACL deficiency
- PCL deficiency
- Patella alta
- Hypoplastic patella
- PFFD
- Varus and valgus femoral neck
- Femoral retroversion
- Acetabular dysplasia
3. Upper extremity - Ulnar hemimelia/amelia
- Syndactyly
- Other
- Renal anomalies
- Cardiac anomalies
What are the classification systems for fibular hemimelia?
[JAAOS 2014;22:246-255]
- Achterman and Kalamchi
- Type IA
- Fibula is present
- Proximal fibular epiphysis is distal to the level of the tibial growth plate
- The distal fibular growth plate is proximal to the dome of the talus
- Type IB
- Partial absence of the fibula
- The fibula is absent for 30% to 50% of its length proximally
- Distally, the fibula is present but does not support the ankle.
- Type II
- Complete absence of the fibula
- Birch classification
- Complete absence of the fibula
- Type 1 = foot preservable (3 or more rays present)
- Subdivided based on overall percentage limb-length inequality compared with the contralateral side
- Type 1A = <6% (correlates to a projected expected inequality at maturity of ≤5 cm)
- Type 1B = 6-10%
- Type 1C = 11 to <30%
- Type 1D = ≥30%
- Type 2 = foot is not preservable
- Subdivided based on presence or absence of upper extremity deficiency requiring the use of the foot to substitute for upper extremity prehension
- Type 2A = functional upper extremity
- Type 2B = nonfunctional upper extremity
Based on the Birch Classification what is the recommended treatment of fibular hemimelia?
[JAAOS 2014;22:246-255][JBJS 2011;15;93(12):1144-51]
Type 1A
- No treatment OR orthosis OR epiphysiodesis
Type 1B
- Epiphysiodesis ± lengthening
Type 1C
- 1 or 2 lengthenings ± epiphysiodesis or extension orthosis
Type 1D
- >2 lengthenings OR amputation OR extension orthosis
Type 2A
- Amputation
Type 2B
- Consider salvage
What are the general treatment options and indications for fibular hemimelia?
[JAAOS 2014;22:246-255]
- Primary problems are LLI, foot deformity, and ankle instability
* Goal of achieving normal WB, normal gait and equal limb length - Orthoses and/or epiphysiodesis candidates:
* Mild LLI (<6%) and functional plantigrade foot - Limb lengthening +/- epiphysiodesis candidates:
- Less severe foot deformities (eg. 3 or more present rays)
- Predicted LLI <30%
- Amputation candidates:
- Severe foot deformity (eg. 3 or more absent rays)
- Predicted LLI ≥30% at the age of skeletal maturity
- >5cm discrepancy at birth
What type of amputation is performed if indicated in fibular hemimelia?
[JAAOS 2014;22:246-255]
Syme or boyd
- Performed at the time the child attempts to walk
What conditions are associated with anterolateral tibial bowing?
[JAAOS 2010;18:346-357]
- Neurofibromatosis Type I
- Of patients with anterolateral tibial bowing – 50% have NF
- Of patient with NF – 5-10% have anterolateral tibial bowing
- Fibrous dysplasia (15% of cases)
What is the diagnostic criteria of NF-1?
[JAAOS 2010;18:346-357]
The diagnostic criteria for NF-1 are met when two or more of the following are found:
- ≥6 café-au-lait macules >5 mm in greatest diameter in prepubertal persons and >15 mm in greatest diameter in postpubertal persons
- ≥2 neurofibromas of any type or one plexiform neurofibroma
- Freckling in the axillary or inguinal region
- Optic glioma
- ≥2 Lisch nodules (dome-shaped gelatinous masses developing on the surface of the iris)
- A distinctive osseous lesion, such as sphenoid dysplasia or thinning of long bone cortex, with or without pseudarthrosis
- A first-degree relative (parent, sibling, or offspring) with NF-1 as diagnosed using the listed criteria
CAFÉ SPOT (mnemonic for diagnostic criteria)
- Café au lait spots (coast of california, smooth)
- Axillary and inguinal freckling
- neuroFibromas
- Eye (lisch nodules)
- Skeletal abnormality (bowing/thinning of long bone, pseudoarthrosis of tibia)
- Positive Family History
- Optic Tumor (optic glioma)
What is the natural history of anterolateral tibial bowing?
[JAAOS 2010;18:346-357]
- Anterolateral bowing of the tibia may be apparent at birth or may progress with weight bearing
- Spontaneous resolution is uncommon
- Fracture with resultant pseudarthrosis typically occurs in the first 4 to 5 years of life
- Fracture risk decreases at skeletal maturity
- Once established, the natural history of a pseudarthrosis is that of persistent instability and progressive deformity
What is the management of anterolateral tibial bowing?
[JAAOS 2010;18:346-357]
Nonoperative
- Indicated for anterolateral bowing in absence of fracture
- Involves bracing when weightbearing until skeletal maturity
- Goal is to prevent progressive deformity and fracture/pseudoarthrosis
Operative
- Osteotomies to correct bowing is contraindicated
- Surgery is indicated once pseudoarthrosis develops
- No surgical technique has proven superior
- Principles include:
- Resection of the pseudoarthrosis
- Stable fixation
- Correction of angular deformity
- Surgical options include:
- IM rod and bone grafting
- Circular fixator with bone transport
- Vascularized fibular graft
- Adjunctive use of bone morphogenetic protein
- Amputation
- Consider after persistent pseudoarthrosis after 2-3 failed surgeries
- Syme amputation preferred
What is the most common complication associated with treatment of congenital pseudarthrosis of the tibia (NF-1)?
Valgus deformity (57.8%)
- Others:
- Malalignment (anterior bowing)
- LLD
- Refracture
What condition is associated with posteromedial tibial bowing?
Calcaneovalgus foot
What is the natural history of posteromedial tibial bowing?
- Progressive correction of the deformity
- Posterior bow typically remodels completely
- Medial bow less likely to remodel completely – residual valgus remains
- Not associated with pathologic fracture or pseudarthrosis of the tibia
- Considerable leg length discrepancy typically develops
What is the management of posteromedial tibial bowing?
Nonoperative
- Observation (correction usually occurs over 5-7 years)
- Passive stretching of foot/ankle
- AFO
- Shoe lift
Operative
- Contralateral Epiphysiodesis (proximal tibia) +/- lengthening to address LLI
Compare and contrast anteromedial, anterolateral and posteromedial tibial bowing
What are causes of intoeing?
[Orthobullets][SPORC]
- Rotational
- Femoral anteversion
- Internal tibial rotation
- Metatarsus adductus
- Miserable malalignment syndrome – femoral anteversion, external tibial torsion, pes planovalgus
- Foot
- Clubfoot
- Skewfoot (metatarsus adductus and hindfoot valgus)
- Metatarsus primus varus
- Hallux varus
- Others
- Cerebral palsy
- Spastic hemiplegia with overactive posterior tibial tendon (only evident in swing phase)
- DDH
Describe the clinical examination for intoeing?
[Orthobullets]
- Gait
- Observation
- Condition specific testing
- Femoral anteversion
- Prone hip ROM (abnormal is >70° IR, <20° ER)
- Internal tibial torsion
- Thigh-foot angle (abnormal >10° IR)
- Bimalleolar angle
- Metatarsus adductus
- Heel bisector line lateral to 2nd webspace
What is the management of intoeing?
[POSNA]
- Femoral anteversion
- Usually nonop
- Surgery is a proximal femoral derotation osteotomy (subtroch fixed with locking plate)
- Internal tibial torsion
- Usually nonop
- Surgery is a proximal or supramalleolar derotation osteotomy
- Metatarsus adductus
- Usually nonop (spontaneous resolution, manipulation and serial casting)
- Surgery can include medial opening wedge osteotomy of the medial cuneiform +/- lateral closing wedge osteotomy or osteotomies of the bases of metatarsals two through four
What are the causes of cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
- Neurological (2/3)
- Central – CP, Friedrich’s ataxia, CVA
- Cord – SMA, myelomeningocele (L4), tethered cord, diastematomyelia
- Peripheral – CMT*, polio
- Clubfoot/recurrent clubfoot
- Traumatic
What is the most common bilateral cause of cavovarus foot?
[Orthobullets]
CMT
What is the foot deformity in cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
- Forefoot
* Pronation, adduction, first ray plantarflexion - Midfoot
* Cavus - Hindfoot
* Varus, calcaneus hyperdorsiflexion
Cavovarus foot is due to muscular imbalances; which muscles are weak and which are strong?
[Lovell and Winter][Orthobullets][SPORC]
- WEAK
- Tibialis anterior
- Peroneus brevis
- Intrinsics
- STRONG
- Peroneus longus
- Tibialis posterior
- Long toe extensors and flexors
- Result of muscle imbalances:
- Claw toes
- Weak intrinsics vs. strong long toe extensors and flexors
- First ray plantarflexion and forefoot pronation
- Weak tibialis anterior vs. strong peroneus longus
- Hindfoot varus
- Weak peroneus brevis vs. strong tibialias posterior
What else drives the hindfoot varus in cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
Tripod effect
- Pronated forefoot causes the medial forefoot to strike the ground first, as the lateral forefoot is brought to the ground the subtalar joint is forced into supination which causes the hindfoot varus
What are the important clinical tests to perform in cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
- Coleman block test
- Silfverskiold test
- Neurological exam
- Muscle strength testing (tendon transfer consideration)
What are the radiographic findings in cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC][JAAOS 2014;22:512-520]
- Calcaneal pitch >30
- Meary angle >4 (apex dorsal usually centered at the medial cuneiform)
- Fibula overlies the posterior 1/3 of the tibia on lateral view
- Vertically oriented midfoot with a “stacked” conformation of the talonavicular joint and calcaneocuboid joint (see picture with sinus tarsi see-through sign)
What are the indications for surgery in cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
- Evidence of a progressive deformity
- Painful callosities under the metatarsal heads or base of the fifth metatarsal
- And hindfoot/ankle instability despite nonoperative treatment
What are the principles of treatment of cavovarus foot and what are the components?
[Lovell and Winter][Orthobullets][SPORC]
- Correct the deformity AND balance the deforming muscle forces
- SOFT TISSUE RELEASES
- Medial plantar release which can include:
- Proximal release of the abductor hallucis
- Tibialis posterior tendon lengthening
- Talonavicular joint capsulotomy
- Plantar fasciotomy
- Possible TAL or gastroc recession
3. OSTEOTOMIES - 1st metatarsal dorsiflexion osteotomy
- Alternative – plantar medial cuneiform opening wedge osteotomy (may be more desirable as it is at the apex of deformity and will not affect the 1st MT growth plate)
- Lateral calcaneus closing wedge osteotomy (Dwyer) or lateral calcaneal slide
- TENDON TRANSFERS
- Peroneus longus to peroneus brevis transfer
- Tibialis posterior to tibialis anterior - 4 incision technique
- Jones transfer of EHL to neck of 1st MT (relieves 1st toe clawing)
What is the salvage procedure for cavovarus foot?
[Lovell and Winter][Orthobullets][SPORC]
Triple fusion
What are risk factors for development of flatfoot?
[Instr Course Lect 2015;64:429]
- Obesity
- Delayed motor development
- Connective tissue disorders
What is the foot deformity in flatfoot?
[Instr Course Lect 2015;64:429]
Pes Planovalgus
- Forefoot – supination, abduction
- Midfoot – flattening of medial longitudinal arch, prominent talar head
- Hindfoot – valgus
What are the clinical examination findings in flatfoot?
[Instr Course Lect 2015;64:429]
- Observation
* Flattened medial longitudinal arch
* Hindfoot valgus
* Too many toes sign - Toe stance
* Flexible = arch reconstitutes
* Rigid = arch does not reconstitute - Hallux extension
* Arch reconstitutes due to windlass effect - Decreased dorsiflexion
* Correct the hindfoot valgus prior to testing dorsiflexion
* Reduced dorsiflexion indicates tight heel cord
What are the radiographic features of pes planovalgus?
[Instr Course Lect 2015;64:429]
- Lateral
- Meary angle – apex plantar
- Calcaneal pitch (normal = 10-30°; <10° = pes planus)
- Talocalcaneal angle (normal = 25-55°; >55° = hindfoot valgus)
- AP
- Talonavicular uncoverage
- Talocalcaneal (Kite) angle (normal = 15-30°; >30° = hindfoot valgus)
- Oblique
* Assess for calcaneonavicular coalition
What is the treatment of flatfoot deformity?
[Instr Course Lect 2015;64:429]
- Asymptomatic
* No treatment - Symptomatic
- Nonoperative
- Orthotics
- Achilles stretching
- Operative
- Indications:
- Failure of nonoperative
- Modified Evans*
- Sinus tarsi type approach
- Inferior extensor retinaculum and EDB are elevated off calcaneus
- Pin the CC joint to prevent subluxation
- Osteotomy is made 2cm from CC joint between anterior and middle facets
- Trapezoidal bone allograft is placed in the opening wedge and pinned
- +/- gastroc recession or TAL
- +/- 1st MT plantarflexion osteotomy if forefoot supinated
- Calcaneo-cuboid-cuneiform osteotomy (‘Triple C’)
- Medial calcaneal slide osteotomy
- Cuboid lateral opening wedge osteotomy
- Medial cuneiform plantar closing wedge osteotomy (corrects supination)
- +/- gastroc recession or TAL
- +/- medial reefing of TN joint capsule
- Indications:
What is the differential diagnosis of a rigid flatfoot?
[Instr Course Lect 2015;64:429]
- Tarsal coalition
- Congenital vertical talus
- Peroneal spastic flatfoot without coalition
- Iatrogenic or posttraumatic deformity
What is the epidemiology and etiology of Congenital Vertical Talus (CVT)?
- Prevalence ~ 1 in 10,000 live births
- 50% of cases are isolated (idiopathic)
- 20% have a positive family history
- 50% of cases are non-isolated (occur in patients with neurologic disorders, genetic defects or syndromes)
- Neurologic disorders = arthrogryposis, myelomeningocele, diastematomyelia
- Genetic defects = aneuploidy of chromosomes 13, 15, and 18
- Syndromes = De Barsy, Costello, and Rasmussen syndromes and split hand and split foot limb malformation disorders
What is the pathoanatomy of CVT?
- Hindfoot equinus and valgus
* Caused by contracture of the Achilles tendon and the posterolateral ankle and subtalar joint capsules - Midfoot and forefoot dorsiflexion and abduction
* Secondary to contractures of the tibialis anterior, extensor digitorum longus, extensor hallucis brevis, peroneus tertius, and extensor hallucis longus tendons and the dorsal aspect of the talonavicular capsule - Navicular dislocated dorsolaterally on the head of the talus
- Vertical talus
- Cuboid dislocated dorsolaterally on the calcaneus
What are the physical examination findings in CVT?
- Convex plantar surface
- Deep creases on the dorsum of the foot
- Rigid deformity
- Distinct palpable gap dorsally where the navicular and talar head would articulate in a normal foot
* If the gap reduces with plantar flexion of the forefoot, then the deformity has a degree of flexibility (good prognosis) - Motor function of plantarflexion and dorsiflexion of the toes
- Elicit by stimulating the dorsum and plantar aspects of the foot
- Record as absent, slight, definitive (prognostic value)
- Assess for associated conditions
* Facial dysmorphic features, sacral dimple, etc.
What radiographic views must be ordered in CVT?
- AP foot
- 3 laterals (max dorsiflexion, max plantarflexion, neutral)
What angles should be evaluated on AP and lateral in CVT?
- AP talocalcaneal angle
- AP TAMBA (Talar axis-first metatarsal base angle)
- Lateral TAMBA
- Lateral talocalcaneal
- Lateral tibiocalcaneal
What should be evaluated on each radiographic view to diagnose CVT?
- Neutral lateral view
- Talus vertically oriented
- Calcaneus in equinus (high tibiocalcaneal angle)
- TAMBA >35° diagnostic for CVT
- Plantarflexed lateral view
* Persistent vertical talus - Dorsiflexed lateral view
* Persistent hindfoot equinus - AP view
* Talocalcaneal angle increased (no angle pathognomonic)
What is the feature of oblique talus?
Talonavicular subluxation that reduces with forced plantarflexion of the foot
What is the management of CVT?
- Traditional
- One or two-stage extensive soft tissue release
- Involves release of contracted tendons plus capsulotomy
- Complications - wound necrosis, osteonecrosis, undercorrection/overcorrection of deformities, long-term stiffness and degenerative arthritis
- Minimally invasive
- ‘Reverse Ponseti technique’
- Characterized by:
- Serial manipulations and casting
- Temporary stabilization of the talonavicular joint with K-wire
- Achilles tenotomy
- Recommended for all CVT regardless of age or associated conditions
Describe the minimally invasive technique for CVT management?
[J Bone Joint Surg Am. 2006 Jun;88(6):1192-200.
- Manipulation
- The thumb of one hand is placed on the head of the talus at the plantar-medial aspect of the midfoot for counterpressure
- The other hand plantarflexes and adducts the forefoot
- The calcaneus is not touched to allow it to move from valgus to varus
- Manipulation is held for 1-2 minutes
- Casting
- Long leg cast applied to hold the foot in position achieved by manipulation
- Short leg cast applied first with careful molding
- Extended above the knee with knee in 90° flexion
- Average of 5 casts required changed weekly
- The final cast positions the foot in maximal plantarflexion and inversion to ensure adequate stretching of tendons, capsule and skin
- K-wire fixation of the talonavicular joint
- A single K-wire is placed retrograde from the navicular into the talus with the foot held in maximum plantar flexion
- The wire is cut and buried underneath the skin for later removal in the operating room
- If the talonavicular joint cannot be reduced by closed means, then a small, 2-cm medial incision is made over the talonavicular joint
- An elevator is used to gently lift the talus to a horizontal and reduced position
- For patients who require this open procedure, transfer the tibialis anterior tendon from its insertion on the navicular to the dorsal aspect of the talar neck with use of suture fixation of the tendon directly into the talar neck
- Dynamic correction of the talonavicular joint
- Percutaneous Tenotomy of the Achilles Tendon
- Dynamic correction of the talonavicular joint
- Once the talonavicular joint is reduced and stabilized with the K-wire, a percutaneous tenotomy of the Achilles tendon is used to correct the equinus deformity
- A long leg cast is applied with the ankle and forefoot in a neutral position
- The cast is changed 2 weeks postoperatively to manipulate the ankle to 10° of dorsiflexion
- The K-wire is removed in the operating room 6 weeks after the index procedure.
5. Boots and bars - The patient then uses a shoe and bar brace system
- Worn full time for 2 months and then only at night for 2 years, to prevent relapse
- The shoes on the brace are set pointing straight ahead to stretch the peroneal tendons
- Parents are also taught foot stretching exercises that emphasize ankle plantar flexion and foot adduction
What are the 3 types of accessory naviculars?
[Orthobullets]
Geist classification
- Type 1 = “sesamoid”
- Sesamoid in the tibialis posterior tendon
- Type 2 = “synchondrosis”
- Accessory bone attached to native navicular by synchondrosis
- Type 3 = “synostosis”
- Complete bony enlargement
What is the treatment of an accessory navicular?
[Orthobullets]
- Nonoperative – first line
- Operative – failed nonop
- Excision of accessory navicular
- Medial approach from talar head to medial cuneiform
- Elevate the tibialis posterior tendon
- Excise the accessory navicular
- Repair tibialis posterior to navicular (suture/suture anchor)
- **Do not advance tibialis posterior (increases recovery time with no benefit)
What are the features of a calcaneovalgus foot?
[Lovell and Winter]
- Hyperdorsiflexion of the ankle
- Eversion of the subtalar joint
- Dorsal surface may rest on anterior tibia
- Dorsal soft tissue contracture limiting plantarflexion and inversion
What is the prognosis of calcaneovalgus foot?
[Lovell and Winter]
Excellent
- Severity of deformity does not predict a worse outcome
What is the treatment of calcaneovalgus foot?
[Lovell and Winter]
- Mild (plantar flexion beyond neutral) – observation
- Moderate (plantarflexion to neutral or less) – observation plus parental stretching
- Severe – consider serial casting (rarely needed)
What are common associated findings with a congenital hallux varus?
[Lovell and Winter]
- Short, thick first metatarsal
- Fibrous medial band
- Longitudinal epiphyseal bracket
- The condition is characterized by a shortened and angulated first metatarsal. The medial diaphysis and metaphyses of the bone are bracketed by a continuous epiphysis
- Suggested by the D-shape of the metatarsal with no cortical differentiation along the convex medial border of the diaphysis
- Accessory metatarsals/phalanges, duplication of the hallux
What is the treatment of congenital hallux varus?
[Lovell and Winter]
Operative
- Surgery is mandatory
- Technique depends on the associated findings:
- Medial soft tissue release – fibrous medial band
- With or without syndactylization (creates syndactyly between the 2nd toe and hallux)
- Farmer technique (see photo)
- With or without syndactylization (creates syndactyly between the 2nd toe and hallux)
- Resection (of central portion) and interposition grafting of a longitudinal epiphyseal bracket
- Medial soft tissue release – fibrous medial band
What is the most common congenital disorder of the shoulder girdle?
[JAAOS 2012;20:177-186]
Sprengel deformity
What are the characteristic features of Sprengel deformity?
[JAAOS 2012;20:177-186]
- Elevated scapula
- Hypoplasia of the scapula
- Hypoplasia of the periscapular muscles
- Malrotated scapula
* Downfacing glenoid, inferior angle rotated medially - +/- omovertebral bone
- Present in ~50%
- Characterized by fibrous, cartilaginous or mature bone connecting the superomedial aspect of the scapula to the cervical spine (usually C4-C7 spinous process, TVP or lamina)
What are the associated abnormalities in a person with Sprengel deformity and their associated prevalence?
[JAAOS 2012;20:177-186]
- Scoliosis 35–55%
- Klippel-Feil syndrome 16–27%
- Rib anomalies 16–48%
- Omovertebral bone 20–50%
- Spina bifida 20–28%
- Torticollis 4%
- Clavicular abnormalities 1–16%
- Humeral shortening 6–13%
- Femoral shortening 1%
- Talipes equinovarus 1–3%
- DDH 1–4%
- Pes planus 1–3%
- Other 1–3%
What is the clinical presentation of Sprengel deformity?
[JAAOS 2012;20:177-186]
- Cosmetic
- Suprascapular region fullness
- Neck fullness
- Functional impairment
- Due to decreased ROM
- Shoulder abduction mostly affected (usually limited to <90°)
What is the classification system for Sprengel deformity?
[JAAOS 2012;20:177-186]
Cavendish Classification of Sprengel Deformity
- Grade 1 (very mild)
- Shoulders are level
- Deformity is not visible when the patient is dressed
- Grade 2 (mild)
- Shoulders are almost level
- Deformity is visible as a lump in the web of the neck when the patient is dressed
- Grade 3 (moderate)
- Shoulder is elevated 2–5 cm
- Deformity is easily seen
- Grade 4 (severe)
- Shoulder is elevated
- The superior angle of the scapula lies near the occiput
What is the management of Sprengel deformity?
[JAAOS 2012;20:177-186]
Nonoperative
- Cavendish grade 1 and 2
Operative
- Cavendish grade 3 and 4
- Timing - ~ age 4-6
- Before age 8 (Increased risk of nerve impairment)
What are the general components of the surgical procedure for Sprengel’s Deformity?
[JAAOS 2012;20:177-186]
- Resection of the elevated portion of the scapula
- Removal of the omovertebral bone
- Inferior mobilization of the scapula
- Clavicular resection osteotomy (to avoid brachial plexus injury)
What are the two main surgical procedures described for Sprengel deformity?
[JAAOS 2012;20:177-186]
- Green (classical)
- Characterized by detachment of scapular muscles from medial border
- Technical points:
- Extraperiosteal release of all muscles at their scapular insertions
- Resection of the supraspinatus fossa and omovertebral bone (if present)
- Reattachment of muscles after caudad mobilization of the scapula
- Muscle lengthening as needed
- Technical points:
- Woodward (preferred)
- Characterized by detachment of scapular muscles from spinal insertion
- Technical points
- Resection of the superomedial portion of the scapula and omovertebral bone (if present)
- Osteotomy of the clavicle
- Detachment of the trapezius and rhomboid muscles
- Mobilization of the scapula caudad
- Reattachment of muscles back to vertebrae with the scapula in its new position
- Technical points
**Can improve abduction by 40-50o
What are the complications associated with surgical correction of Sprengel deformity and their reported prevalence?
[JAAOS 2012;20:177-186]
- Hypertrophic scar (26–64%)
- Brachial plexus injury (6–11%)
- Regrowth of the superior pole of the scapula (30%)
- Scapular winging (4–17%)
What is the definition of a brachial plexus birth palsy (BPBP)?
Traction or compression injury to the brachial plexus sustained during birth
What should be ruled out in cases of BPBP?
Pseudoparalysis secondary to humerus fracture
What are the risk factors for BPBP?
- Macrosomia (>4,500g)
- Difficult or prolonged labour
- Shoulder dystocia
- Multiparous pregnancy
- Vacuum or forceps delivery
- Prior BPBP
What are the classifications of BPBP?
- Character of the neurological injury
- Root avulsion (preganglionic)
- Root rupture (postganglionic)
- Neuropraxia
- Anatomic levels of involvement
- C5-6 = Erb’s palsy (‘waiter’s tip’)
- C8-T1 = Klumpke palsy (‘claw hand’)
- C5-T1 = Total plexus palsy
- Narakas classification
- Group I = C5-6 palsy (~46%)
- Group II = C5-7 palsy (~29%)
- Group III = flail extremity (total plexus) without Horner syndrome
- Group IV = flail extremity with Horner syndrome
What are the good and poor prognostic factors for BPBP?
- Good prognostic factors
- Erb’s palsy
- Antigravity biceps function by 2-3 months
- Poor prognostic factors
- Lack of antigravity biceps function by 3-6 months
- Root avulsion (preganglionic)
- Suggested by:
- Horner’s syndrome
- Elevated hemidiaphragm (phrenic n.)
- Scapular winging (dorsal scapular n.)
- Lack of trapezius function
- Suggested by:
- Klumpke’s palsy
- C7 involvement
What are the indications for microsurgical nerve reconstruction in BPBP?
- Absence of antigravity biceps function between age 3-9 months
- Flail extremity and Horner’s syndrome at age 3 months
What are the treatment options in BPBP?
- Nonoperative
* Physiotherapy/parental stretching while awaiting return of function - Operative
- Nerve grafting = root rupture (postganglionic)
- Nerve transfer = root avulsion (preganglionic)
What are the associated secondary conditions with BPBP and the management of each?
- Shoulder internal rotation contracture
- Latissimus dorsi and teres major transfer
- Pectoralis major and subscapularis lengthening
- Glenohumeral dysplasia
* Proximal humeral derotation osteotomy - Elbow flexion contracture
* Serial splinting or casting
What is the cause of the Madelung deformity?
[JAAOS 2013;21:372-382]
- Growth disturbance at the ulnar and volar aspect of the distal radial physis
- Vicker’s ligament may be a contributor to the growth disturbance due to the increased pressure on the growth plate
What is Vicker’s ligament?
[JAAOS 2013;21:372-382]
Abnormal volar ligament that tethers the lunate to the volar distal radius
What is the resulting bony deformity in Madelung’s deformity?
[JAAOS 2013;21:372-382]
- Ulnar and volar curvature (increased radial inclination and volar tilt)
- Positive ulnar variance
- Proximal subsidence of the lunate
Clinically, on observation how does the hand and wrist appear in Madelung’s Deformity?
[JAAOS 2013;21:372-382]
The hand appears to be translated volarly and ulnarly relative to the wrist, and dorsal prominence of the ulnar head is a distinguishing feature
What genetic syndromes are associated with Madelung’s deformity?
[JAAOS 2013;21:372-382]
- Leri-Weill Dyschondrosteosis
- rare genetic disorder caused by mutation in the SHOX (short-statute homeobox-containing) gene
- anatomically at the tip of the sex chromosome
- causes mesomelic dwarfism (short stature)
- Turner Syndrome
- Nail-Patella Syndrome
Surgical indications for Madelung deformity?
[JAAOS 2013;21:372-382]
- Pain
- Limited motion
- Cosmesis/deformity
Surgical options for Madelung’s deformity?
[JAAOS 2013;21:372-382]
- Physiolysis and Vicker’s ligament release
- Consider in early, mild deformity in the skeletally immature patient
- Involves resection of ulnar and volar physis with fat interposition and Vicker’s ligament excision
- Often combined with ulna shortening osteotomy or distal ulnar epiphysiodesis
- Radial dome osteotomy +/- ulnar shortening osteotomy
- Consider in more severe deformity
- Involves Henry approach, dome osteotomy of distal radius, correction achieved by radial deviation and pronation of the hand and dorsal displacement of the distal fragment, Vicker’s ligament resection
What are the two most common types of tarsal coalition?
[Lovell and Winter]
- Calcaneonavicular
* Between the anterior process of the calcaneous and the navicular - Talocalcaneal
* Between the middle facet of the talocalcaneal joint
**NOTE: these two types occur with equal frequency
- Others
* Talonavicular, calcaneocuboid, naviculocuneiform, cuneiform-metatarsal coalitions
How often are tarsal coalitions bilateral?
~50-60%
What percentage of tarsal coalitions are symptomatic?
25% (75% asymptomatic)
What is the foot deformity that occurs in tarsal coalition?
- Rigid flat foot with peroneal spasticity
- Hindfoot valgus, forefoot abduction, pes planus (arch does not reconstitute with toe standing)
What is the presenting complaint in tarsal coalition?
- Activity related pain in the sinus tarsi or medial hindfoot
- Recurrent ankle sprains
At what age do patients present?
8-12 years of age
What are the radiographic features of tarsal coalition?
- Calcaneonavicular coalition
* Anteater sign (elongated anterior process) on oblique foot view - Talocalcaneal coaltion
- C-sign (lateral)
- Dorsal talar beaking
3.Ball and socket ankle
What is the nonoperative management of symptomatic tarsal coalition?
Activity modification, NSAIDs, OTC soft shoe inserts, casting or walking boot
What are the indications for talocalcaneal coalition resection vs. subtalar fusion?
[POSNA]
- Indications for resection:
* <50% posterior facet involvement - Indications for subtalar fusion
- >50% posterior facet involvement
- Posterior facet joint degeneration
- Hindfoot valgus >16-21 degrees
What additional imaging is required prior to operative management of tarsal coalition?
CT and/or MRI
- CT – better assessment of coalition size and associated arthritis
- MRI – better assessment of associated soft tissue pathology and fibrous/cartilaginous coalitions
Describe the calcaneonavicular bar (coalition) resection?
- Incision = Ollier (dorsolateral oblique incision)
- Starts 1-2cm distal to tip of fibula and extends towards to talonavicular joint
- Can extend as far as the extensor and peroneal tendons
- EDB proximally is identified and retracted distally to identify the calcaneonavicular bar
- Visualize the sinus tarsi, calcaneocuboid joint and talonavicular joint
- Using a ¼ or ½ osteotome a trapezoidal (not triangular) piece of bone is excised
- Visualize a sufficient gap and ensure motion occurs between the navicular and calcaneus
- Interpose fat, bone wax or EDB
- Free fat graft from buttock with EDB covering
- EDB passed into defect with straight Keith needles tied over button on medial foot
Describe the talocalcaneal bar (coalition) resection?
[Foot Ankle Clin. 2015 Dec;20(4):681-91]
- Medial incision distal to the medial malleolus extending the length of the subtalar joint
- Identify and protect the NV bundle and FHL
- Tendon sheath is opened and FDL is taken inferior and tib posterior taken superior
- Bar resection of the middle facet is completed with osteotome, rongeur and burr until normal cartilage of the posterior facet is visualized
- Visualize gap and ensure adequate hindfoot motion
- Bone wax applied to bony ends and fat graft interposed
NOTE: can also be done all arthroscopic
What is Kocher’s criteria for pediatric septic arthritis?
[JBJS Am. 1999 Dec;81(12):1662-70.]
- WBC > 12,000 cells/µl of serum
- Inability to bear weight
- Fever > 101.3° F (38.5° C)
- ESR > 40 mm/h
What is the predicted probability of septic arthritis based on the number of Kocher criteria met?
[JBJS Am. 1999 Dec;81(12):1662-70.]
0 = <0.2%
1 = 3.0%
2 = 40.0%
3 = 93.1%
4 = 99.6%
What other blood marker is an independent risk factor for septic arthritis?
[AAOS Comprehensive Review 2014]
CRP >20
List the criteria to differentiate between septic arthritis and transient synovitis
[Pediatr Clin N Am 61 (2014) 1109–1118]
What is the underlying pathology of all forms of Rickets?
[Lovell and Winter]
- Rickets is failure or delay of calcification of newly formed bone at long bone physes
- Due to inadequate calcium or phosphate
- Occurs at zone of provisional calcification [Orthobullets]
What are the orthopedic manifestations of Rickets?
[Lovell and Winter]
- Decreased longitudinal bone growth
- Angular deformities
- Genu varum
- Genu valgum
- Coxa vara
- Osteomalacia
- Costochondral enlargement (rachitic rosary)
- Kyphoscoliosis
- Skull
- Delayed anterior fontanelle closure
- Parietal and frontal bossing
- Plagiocephaly
- Delayed primary dentition
What are the radiographic features of Rickets?
[Lovell and Winter]
- Wide and indistinct growth plates (HALLMARK)
- Lateral expansion of growth plates
- Cupped and splayed metaphysis
- Short long bones for age
- Angular deformity (coxa vara, genu varum/valgum)
- Looser zones
- Transverse bands of unmineralised osteoid (‘Pseudofracture’)
- Typically appear in the medial aspect of the proximal femur and at the posterior aspect of the ribs
- Acetabular protrusio
- Pathological fracture
What are the types of Rickets?
[Lovell and Winter]
- Nutritional
- Causes:
- Vit D deficiency (most common)
- Profound calcium deficiency (rare)
- Combined Vit D and calcium deficiency
- Presents at 6m - 3y
- Pathophysiology [Orthobullets]
- Low Vitamin D levels lead to decreased intestinal absorption of calcium
- Low calcium levels leads to a compensatory increase in PTH and bone resorption
- Bone resorption leads to increased alkaline phosphatase levels
- Treatment = Vit D and calcium
2. X-linked hypophosphatemic rickets - AKA Familial hypophosphatemic rickets (x-linked dominant)
- Cause = renal phosphate wasting AND low or normal kidney production of 1,25-dihydroxyvitamin D3
- Inability of renal tubules to absorb phosphate
- Presents at 1 - 2y
- Treatment = phosphate and Vit D (calcitriol)
- Renal osteodystrophy
- Causes = renal failure
- Insufficient 1,25-dihydroxyvitamin D3 activation
- Reduced phosphate excretion
- Hyperphosphatemia causes hypocalcemia (reduced renal uptake) which causes secondary hyperparathyroidism
- Treatment = dietary phosphate restriction, phosphate binding agent, Vit D3
- Hypophosphatasia
- Causes = ALP deficiency (autosomal recessive)
- Treatment = no medical treatment
- 1-Alpha-Hydroxylase deficiency (‘Vitamin D dependent’-Type 1)
- Causes = unable to convert 25-hydroxyvitamin D3 to its biologically active form of 1,25-dihydroxyvitamin D3
- Treatment = 1,25 Vit D3
- End organ insensitivity (‘Vitamin D dependent’-Type 2)
- Causes = lack receptor for 1,25 Vit D3
- Treatment = high dose 1,25 Vit D3 and Calcium
What is the etiology of osteogenesis imperfecta?
Mutation in genes coding for Type I collagen resulting in quantitative and qualitative defects
- Type I Collagen is a triple-helix molecule is composed of:
- Two alpha-1 chains – COL1A1 gene
- One alpha-2 chain – COL1A2 gene
What are the extra-skeletal manifestations of OI?
- Ocular
- Blue sclera (present in ∼50% OI types)
- Other – Glaucoma, Cataracts, Ectopia lentis, lens subluxation/dislocation, Presbyopia (farsightedness)
- Dentinogenesis imperfecta
- Discoloration of the teeth (yellowing and apparent transparency)
- Abnormal formation of the teeth such as bulbous crowns and short roots
- Teeth wear and break prematurely
- Hearing loss
- Occur in up to 50% of individuals by 50 years of age
- Hearing loss may be conductive, sensorineural or mixed
- Due to otosclerosis, fracture of the ossicles, as well as neural degeneration
- Joint hypermobility
- Easy bruisability
- Cardiac
- Mitral and aortic valve insufficiency
- Aortic root dilation
- Hypercalcuria
- Affects 1/3 of OI patients
- Increased kidney stone risk
- Dysmorphic, triangle-shaped facies
What are the skeletal manifestations of OI?
- Fracture
- Multiple childhood fractures
- Fractures tend to decrease after adolescents (can increase later in life)
- Most commonly long bones and vertebral bodies (codfish vertebrae)
- Apophyseal avulsion fractures of the olecranon are characteristic of OI
- Long bone deformity
- Short stature
- Wormian skull bones
- Scoliosis
- Pectus excavatum/carinatum
- ↓ Bone mineral density
- Basilar invagination
- Present in ∼8–25%
- May present with symptoms including sleep apnea, headache, nystagmus, cranial nerve palsies, ataxia, and quadriparesis
*
What is the classification system for OI?
Silence Classification
- TYPE I (non-deforming)
- Prevalance = ∼50% (most common)
- Severity = most mild (minimally deforming)
- Inheritance = AD
- Features:
- Blue sclera – YES
- DI – variable
- Ambulatory, normal/slightly short stature, minimal kyphoscoliosis, variable hearing loss
- Hallmark is multiple childhood fractures
- Likely underdiagnosed
- TYPE II (lethal)
- Prevalance = N/A (rare)
- Severity = lethal
- Inheritance = AR
- Features:
- Fatal in the perinatal period secondary to thoracic bony insufficiency and respiratory complications
- Type III (severe deforming)
- Prevalance = ∼20%
- Severity = most severe form compatible with life
- Inheritance = AR
- Features:
- Blue sclera = NO
- DI = YES
- Wheelchair-bound or assistive devices, short stature, severe kyphoscoliosis, frequent hearing loss, shortened and bowed limbs, triangular facies, chronic pain
- Type IV (intermediate)
- Prevalance = ∼20%
- Severity = moderate
- Inheritance = AD
- Features:
- Blue sclera = NO
- DI = variable
- Moderately short stature, moderate kyphoscoliosis, variable hearing loss
Modification to Sillence classification
- Type V
- Prevalance = ∼5-10%
- Severity = moderate
- Inheritance = AD
- Features:
- Blue sclera = NO
- DI = NO
- Normal hearing, mild to moderate short stature, variable kyphoscoliosis
- Congenital bilateral anterolateral radial head dislocation with synostosis, hyperplastic callus formation in long bones following fracture
- Type VI-XIII
- Variable
What is the nonoperative management of OI?
- General recommendations
- Optimize Vitamin D and calcium intake
- Encourage regular (low-risk) weightbearing activities
- PT and aquatherapy for moderate-severe forms to maintain function and independence
- Medical therapy
- Bisphosphonates
- Often started in childhood
- Increases the bone volume but no effect on bone quality
- Controlled trials are equivocal about reduction of long bone fractures and have not supported improved mobility or pain status with bisphosphonates
- Still standard of care for moderate to severe OI
What are the principles of fracture management in OI?
- Fracture healing is normal – no need for prolonged immobilization
- Nonsurgical treatment preferred in equivocal situations
- 20% will experience nonunion over lifetime
- Always get full length films to assess for bowing, deformity, limb
- IM devices preferred over plate constructs
- Issues with plates
- Prone to fracture at ends of plates (stress riser)
- Locking plates – promote stress shielding and bone resporption
- Nonlocking plates – prone to failure via screw pullout
- Issues with IM devices
- Risk of iatrogenic fracture
- Abnormal anatomy (supraphysiologic bowing, non-linear/imperforate canals)
- Telescoping IM Rods
- “Bailey-Dubow” “Sheffield” “Fassier-Duval”
- Lengthens with growing bone
- Consider after age 2 (prior to age 2 treat as if patient does not have OI)
What is the operative management of long bone bowing deformity in OI?
Sofield osteotomy
- “shish kebab” multiple long-bone osteotomies with IM device to correct deformity
What is the management of scoliosis in OI?
[JAAOS 2017;25:100-109]
- Bracing not recommended due to fragility of the ribs
* Continued progression and chest wall deformity secondary to the brace - Spinal fusion is considered when curve is >45°
* In severe OI consider fusion when curve >35° - Consider preoperative bisphosphonate therapy
- Strengthens cortical bone and improves pullout strength of pedicle screw
- Hold postoperatively for ~4 months to allow remodelling of the fusion mass
- Consider pedicle screws with cement augmentation
* Used at the distal and proximal foundations
What is the management of basilar invagination in OI?
[JAAOS 2017;25:100-109]
- Surgical treatment for basilar invagination reserved for patients with clinical symptoms
* Most commonly includes headaches, cranial nerve palsy, dysphagia, and symptoms of myelopathy, such as hyperreflexia, quadriparesis, and gait abnormality - Evidence lacking to support orthotic braces for asymptomatic basilar invagination or to delay independent upright posture until 18 months of age
- Hydrocephalus addressed with ventriculoperitoneal shunt first
- Craniocervical fusion with or without traction
What is arthrogryposis?
[POSNA][JAAOS 2002;10:417-424]
Describes a group of nonprogressive disorders that result in fetal akinesia (decreased movement), multiple joint contractures and varying degrees of muscle weakness
- Amyoplasia
- Refers to the most common type with multiple joint contractures (classical disease)
- Usually involves all 4 limbs
- Distal arthrogryposis
- Refers to involvement of the hands or feet
What are the goals of treatment in arthrogryposis?
[POSNA][JAAOS 2002;10:417-424]
Independent function
- Do not compromise function for cosmesis
What are the features of arthrogryposis?
[POSNA][JAAOS 2002;10:417-424]
- Midline cutaneous hemangioma (nevus flammeus) on the forehead
- Limbs appear thin, atrophic and are without normal flexion creases
- Active motion is limited (weakness and contractures)
- Characteristic contractures:
- Upper extremity
- Waiter’s tip – shoulders adducted and internally rotated, elbows extended, forearms pronated, wrist flexed and ulnarly deviated, thumb opposed
- Hip
- Hip flexion, abduction and external rotation contractures
- Hip dislocation (~30%)
- Knee
- Flexion or extension contractures
- Congenital dislocation of the knee
- Foot
- Rigid equinovarus
- Congenital vertical talus
- Scoliosis
- C-shaped neuropathic curve
How is arthrogryposis treated?
[POSNA][JAAOS 2002;10:417-424]
- In general:
- Initial treatment should start at birth and involves gentle stretching, range of motion and taping of any contractures
- Once the position of a joint is acceptable, lightweight splinting may slow recurrence of contractures
- If the joints cannot be placed into an acceptable position, serial casting or soft tissue releases followed by casting may be undertaken
- In the lower extremity proceed from distal to proximal (feet – knees – hips)
- Surgery may be avoided if deficits can be overcome with adaptive equipment
2. Hips - Hip flexion, abduction and external rotation contractures
- Mild contractures – stretching
- Soft tissue release
- Hip dislocation
- Teratologic hip dislocation – not responsive to Pavlik or closed reduction
- Unilateral hip dislocation
- Open reduction ~6-12 months of age
- Observation (consider given high risk of failure and osteonecrosis)
- Bilateral hip dislocations
- Controversial – often observation
- Dislocated supple hips are often better than reduced stiff hips
- Open reduction of both will often not result in both being supple and reduced
- Knee
- Controversial – often observation
- Unilateral hip dislocation
- Teratologic hip dislocation – not responsive to Pavlik or closed reduction
- Stretching and splinting first line
- Flexion contracture
- Quadricepsplasty
- Distal femoral extension and shortening osteotomy
- Extension contracture
- Hamstring, posterior capsule, PCL release
- Feet
* Equinovarus- Ponsetti method
- Posteromedial release – rigid recurrence, incomplete correction, older age at presentation
- Upper extremity
- Extension contracture
- Stretching/splinting
- Triceps lengthening and posterior capsular release
- Internal rotation contracture
- External humeral rotation osteotomy
- Wrist flexion contracture
- Stretching/splinting
- FCU transfer to dorsum of hand (FCU is often only functioning wrist flexor/extensor)
- Carpal wedge osteotomy
- Thumb in palm deformity
- Soft tissue release
- Scoliosis
- Bracing ineffective
- Anterior and posterior spinal fusion
What is the treatment of congenital pseudarthrosis of clavicle?
- Typically on right except in patients with dextrocardia
- Treatment if symptomatic = take down pseudoarthrosis, restore length, tricortical ICBG, plate
What are the indications for physeal bar excision and interposition?
<50% physeal involvement and >2 years or >2cm growth remaining
In patients with hemihypertrophy, what additional test/exam is required?
Perform serial abdominal ultrasounds (every 3 months) until age 7 to rule out Wilm’s tumor
What allergy is associated with myelomeningocele?
A 20-70% incidence of IgE mediated latex allergy
What level of Myelomeningocele is most associated with hip dislocation?
Occurs most commonly at L3 level due to unopposed hip flexion and adduction
What is the most common genetic disease resulting in death in childhood?
Spinal muscular atrophy
What is the deformity in infantile blounts?
Deceleration of growth of the posteromedial proximal tibial physis Varus, flexion, internal rotation deformity Medial and posterior sloping off proximal tibial epiphysis Variable relative tibial shortening (in unilateral cases)
