Peds Flashcards
Obstetrical Clavicle Fracture - Risk factors
• Large birth weight (>4kg) • Shoulder dystocia • Prolonged gestation • Forceps delivery
Medial Epicondyle Fracture – Surgical Indications
• Absolute o Entrapment of medial epicondyle • Relative o > 5mm displacement (usually goes distal and lateral) o Associated with elbow dislocation o Dominant arm in throwing athlete o Weight-bearing extremity in athlete who weight bears through arm (gymnast)
Infantile Scoliosis - Risk of progression
• RVAD > 20 degrees • Phase 2 rib • Cobb angle > 30 degrees
Describe the Kocher Criteria
Septic hip? • Temp > 38.5 • CRP • ESR • Refusal to WB • WBC count
Septic Arthritis Poor prognostic factors
• Age < 6 months • Delay to treatment > 4 days • Hip • Joint effusion with underlying osteomyelitis
Neonatal Infections: Risk factors in NICU
• Phlebotomy sites • Indwelling catheters • Invasive monitoring • Peripheral alimentation • IV drug administration
Lyme Disease Features
NIVEA • Neuropathies • Intermittent reactive arthritis • Cardiac arrhythmias (“V-Tach”) • Erythema migrans (“bulls eye” rash) • Acute arthritis
ACL “Rules” to avoid physeal arrest
- Avoid over-tensioning
- Tunnels filled with soft tissue graft only (less likely to form physeal bars)
- Avoid bone blocks and hardware at level of physis
- Tunnels small (6-7mm) - <5% of physis (8mm tunnel in 12yr old = 3-4%)
- Tunnels perpendicular to physis (not oblique)
NF dystrophic scoliosis features
- short (4-6 levels), sharp curves, <6 yrs
- Xray findings
- scalloping end plate (posterior)
- foraminal enlargement
- pencilling of ribs
- vertebral wedging
- dysplastic pedicles
- dural ectasia (*MRI pre-op)
- dumbbell lesion (canal neurofibroma)
Proteus Syndrome - 3 characteristic findings
- hemihypertrophy
- macrodactyly
- partial gigantism of hands/feet/both
Hemihypertrophy - associated with (4)
- Klippel-Trenaunay-Weber syndrome
- Proteus
- NF-1
- Beckwith-Wiedemann syndrome
Syndactyly - associated with (4)
PACU
- Poland syndrome
- Apert syndrome
- Congentical constriction band syndrome (Streeters syndrome)
- Ulnar longitudinal deficiency
Order of frequency of syndactyly webbing
- 3rd webspace
- 4th webspace
- 2nd webspace
- 1st webspace
Poland Syndrome
- subclavian artery hypoplasia
- absence of sternocostal head of pec major
- associated with
- limb hypoplasia (synbrachydactyly - short digits)
- carpal coalition
- RU synostosis
- sprengel’s deformity
- scoliosis
- dextrocardia
- nail agenesis
Apert Syndrome
- FGFR2***
- Associated with (TAMP)
- tarsal coalition
- acrosyndactyly (spadelike hand)
- mental retardation
- premature fusion of cranial sutures
Trigger thumb
- constriction of FPL at A1 pulley
- 60% bilateral
- 30% spontaneous resolution if <1 yr
- <10% spontaneous resolution if >1 yr
- Surgery –> at 2 years if not resolved
- A1 pulley release
Clinodactyly
- radioulnar deformity of 5th digit
- autosomal dominant
- Associated with:
- Down syndrome (80%)
- Russel-Silver syndrome
- Feingold syndrome
Risk factors for brachial plexus birth palsy
- macrosomia
- multiparous pregnancy
- prolonged labour
- difficult delivery
- shoulder dystocia
- difficult arm/head extraction in breech
- previous BPBP
Natural history of brachial plexus birth palsy
- 80-90% spontaneous recovery
- antigravity biceps by 2 mths = full recovery anticipated
- biceps recovery at/after 5 mths= incomplete recovery anticipated
- poor prognostic signs
- horner syndrome
- phrenic nerve palsy
- total plexopathy (flail extremity)
- most common problem = IR contracture of arm = glenoid dysplasia (posterior subluxation, humeral head flattening, increased glenoid retroversion)
Brachial plexus birth palsy - signs of preganglionic lesion (4)
- horner syndrome
- elevated hemidiaphragm
- winged scapula
- absence of rhomboid/rotator cuff/lat dorsi function
Brachial plexus birth palsy - Indications for microsurgery
- no biceps function at 3-6 mths
- flail extremity + horners at 3 mths
- others say - flail or horners @ 1 mth
Erb’s Palsy treatment
- “waiter’s tip” - arm adducted, IR, pronated, extended at elbow
- can trial splinting (sup, ER)
- sx options
- pec major release
- subscap release
- anterior capsule release
- lat dorsi & teres major transfer (in young patients to try and prevent progression of dysplasia)
- external rotation osteotomy of humerus (in significant dysplasia)
What is Sprengel’s deformity
- small, undescended scapula
- associated with
- scapular winging
- hypoplasia
- omovertebral connections (bony or fibrous connection from scapula to vertebral column)
Disorders/diseases associated with Sprengel’s
- Congenital scoliosis
- Klippel-Feil
- Torticollis
- Poland Syndrome
- Facial asymmetry
- UE abnormalities
- Diastematomyelia
- Pulmnary or renal dx
PPT FUCKeD
Components of Madelung’s Deformity
VARA
- Vicker’s ligament (lunate to radius*)
- increased radial inclination
- volar subluxation of the carpus
- apparent dorsal subluxation of the ulna
Teratologic DDH associations
- arthrogryposis
- myelomeningocele
- SMA
- CMT
- CP
- Larsen’s
- Ehlers-Danlos
- Down Syndrome
- Mucopolysaccharoidosis (later in life)
DDH associated with packaging problems
- prematurity
- oligohydramnios
- multiparous birth
- congenital knee dislocation
- congenital torticollis (20%)
- metatarsus adductus (10%)
Indications for screening US in DDH
- positive family hx (parent or sibling)
- breech position in utero
- congenital muscular torticollis
- congenital knee dislocation
- positive physical exam
- equivocal exam in patient at risk
DDH Ultrasound Parameters
- Alpha angle = bony acetabular roof + ileum
- Normal > 60
- Beta angle = cartilaginous acetabular roof + ileum
- Normal < 55
- *BIG BETA BAD
DDH Radiographic Parameters
- Hilgenreiner’s line - horizontal across superior aspect of both triradiate cartilage
- Perkin’s line - perpendicular to H at lateral edge of acetabulum
- *ossific nucleus in infero-medial quadrant
- *if no ossificationuse medial aspect of femoral neck
- Shenton’s line
- Acetabular Index (AI)
- >30 abnormal in infant (1 yr)
- >20 abnormal >2 yrs
- Center-edge angle of Wiberg
- <20 abnormal
DDH Blocks to Reduction
*OUTSIDE –> IN
- iliopsoas and adductor longus contracture
- hip capsule
- inverted labrum
- inverted limbus (hypertrophied labrum)
- TAL
- pulvinar (fatty tissue filling space)
- ligamentum teres
Pavlik Harness - Position & Complications
- 90 degrees flexion (anterior strap)
- Risk: Femoral nerve palsy
- <60 degrees abduction (posterior strap)
- Risk: AVN (impingement of posterosuperior branch of MFCA)
- *Length of Pavlik tx = age + 3 mths (1/2 full time, 1/2 part time)
DDH Closed Reduction Technique and Principles
- traction, flexion, abduction
- confirm with medial dye pool < 5-7mm
- “safe zone” of Ramsey
- maximum passive ABD to ADD where hip is stable
- adductor tenotomy - as required to increase safe zone
- spica cast - “in 90-100 degrees flexion within the safe zone avoiding > 60 degrees abduction and neutral rotation”
- post-op CT or MRI
- cast x 3-4 mths, change at 6 weeks
DDH Indications for Open Reduction
- failed closed treatment
- non-concentric reduction (likely block present)
- reduction requiring extreme, dangerous positioning
- gross instability
Spica Position after Open Reduction/Pelvic Osteotomy
- 30 degrees flexion
- 30 degrees abduction
- minor IR
- *cast x 6 weeks, abduction brace x 6 weeks
- *if medial approach = spica x 3 months
Bony Changes in DDH (femur & acetabulum)
- Femur
- small head
- short anteverted neck
- valgus
- posterior GT
- tight isthmus (esp M/L)
- Acetabulum
- shallow, hypoplastic
- anteverted
- anterolateral & superior deficiency
DDH Anterior Approach - Advantages & Disadvantages
- Advantages
- Capsulorrhaphy possible*
- Lower risk of osteonecrosis (MFCA)*
- Direct access to acetabulum/blocks to reduction
- Able to do pelvic osteotomy through same incision
- Shorter duration of spica (6 wks)
- Familiar surgical approach
- For high-riding dislocations, older patients (>1 yr)
- Disadvantages
- Post-op stiffness
- Potential blood loss
- Risk to LFCN
DDH Medial Approach - Advantages & Disadvantages
- Advantages
- Direct access to medial structures (lig teres)
- Avoid damage to abductors, splitting iliac crest apophysis
- Less stiffness
- Less invasive, minimal dissection, quicker
- Less blood loss*
- Better scar
- Disadvantages
- Capsulorrhaphy not possible*
- Pelvic osteotomy not possible (ie use in <18 mths)
- Poor visualization of acetabulum
- Labrum not accessible
- Higher risk osteonecrosis*
- Longer duration of cast (3 mths)*
- dont have capsulorrhaphy to help maintain reduction as in anterior approach
Perthes - Poor Prognostic Factors
- onset of symptoms > age 8*
- female
- premature physeal closure
- lateral hip subluxation
- reduced hip ROM (abd)
- >50% femoral head involvement/collapse*
- aspherical head, incongruent joint (Stulberg)
- Herring B or C
- Catterall III or IV
- Salter-Thompson B
- 2+ Catterall at risk signs
- ***head involvement (shape, congruency) + age at onset of disease most important risk factors***
Perthes - Catterall Radiographic Head-At-Risk Signs
- Need 2 or more
- Gage sign (“V” in lateral epiphysis)
- Calcification lateral to epiphysis
- Lateral subluxation of femoral head
- Horizontal physis
- Metaphyseal cysts
*G-MLCH
Perthes - Waldenstrom’s Radiographic Stages
- Initial (xrays normal 3-6 mths)
- Fragmentation (lasts 1 yr) - use all classifications during this stage*
- Re-ossification (3-5 yrs)
- Remodeling
Perthes - Catterall Classification
- Group I = anterior head involvement only
- Group II = anterolateral/central head
- Group III = 75% head involvement
- Group IV = total head involvement
*fragmentation stage
Perthes - Stulberg Classification
- *At maturity - correlates shape of head and development of radiographic OA
- Type 1 = normal hip joint
- Type 2 = spherical head
- Type 3 = non-spherical head (60% develop OA within 40 yrs)
- Type 4 = flat head
- Type 5 = flat head with incongruent hip joint
Perthes - Herring Classification
- Group A = no involvement of lateral pillar
- Group B = >50% lateral pillar maintained
- Group C = <50% latearl pillar maintained
- Group B/C*
- lateral pillar = lateral 15-30% of epiphysis
- use during fragmentation stage
- BEST PREDICTOR OF LONG TERM OUTCOME*
Perthes - Salter-Thompson Classification
- Group A = subchondral fracture/crescent affected <50% of femoral head
- Group B = >50%
Salter Pelvic Osteotomy - Amount of Coverage Achieved
up to 15 deg lateral coverage
and 25 deg anterior coverage
Risk Factors for SCFE
- african descent/pacific islanders
- male gender
- renal failure
- endocrine abnormalities
- radiation/chemo
- obesity
- down syndrome
REAM ROD
Indications for Endocrinology workup in SCFE
- Age < 10
- Weight < 50th percentile
Labs: TSH, GH, BUN/Cr (renal osteodystrophy)
Conditions that weaken physis (associated with SCFE)
- hypothyroidism
- panhypopituitarism
- GH abnormalities
- hypogonadism
- hyper- or hypoPTH
- renal osteodystrophy
- previous radiation/chemo
Physeal zone affected in SCFE
HYPERTROPHIC
(think fat kid)
Deformity of distal fragment in SCFE
- Varus in coronal plane
- Extension in sagital plane
- External rotation in axial plane
- (VAR-EX-EX)
SCFE - Imaging Findings
- Retroversion deformity
- Metaphyseal blanch sign of Steel (double density)
- Klein’s line (AP)
- Trethowan’s sign (asymmetry in Klein’s line)
- Scham’s sign
- Southwick angle (AP&frog) - normal 12
SCFE Classification Systems
- Temporal (acute <3 weeks, chronic >3 weeks, acute on chronic)
- Percent slippage (mild 0-33%, mod 33-66%, severe >66%)
- Loder Classification (stable vs unstable)
- Southwick angle difference (mild <30 deg, mod 30-60, severe >60)
Indications for prophylactic fixation of opposite hip in SCFE
- Age <10
- Associated condition (endocrinopathy)
- Mental delay
- Poor compliance/follow-up
- Symptoms on other side***
- Posterior sloping angle >12
PAM SPA
SCFE In Situ Fixation - Key Points
- Fracture table, gentle* traction
- Guide pin starts anterolateral (screw heads posterior)
- Avoid start point at/distal to LT
- Keep start point lateral to intertrochanteric line (impingement)
- 7.3 mm cannulated screw, 4-5 threads minimum crossing
- Approach-withdraw method to confirm no screw penetration
- Single vs multiple screws (unstable SCFE)
- Stable = PWB, unstable = NWB
SCFE - Late Treatment Osteotomy
- Left with retroversion deformity + anterior FAI
- Flexion intertrochanteric osteotomy = Imhauser osteotomy
- lateral approach to femur
- osteotomy just proximal to LT
- FLEXION primary goal (anterior wedge of bone removed from osteotomy)
- IR distal shaft (anteverts head)
- mild valgus
- ttwb x 3 mths
Coxa Vara - Types
- Developmental (genetics - AD)
- Congenital (eg with PFFD)
- Dysplastic (skeletal dysplasias, OI, rickets)
- Acquired/traumatic
C-DAD
Coxa Vara - Op vs Non-Op
- Non-Op
- Asymptomatic, HE angle < 45
- Asymptomatic, HE angle 45-60 (follow with serial radiographs)
- Operative
- Symptomatic, HE angle 45-60
- HE angle >60 likely to progress
- Progressive decrease NSA <100
- GOAL = HE < 38
Hemihypertrophy - important association***
Get renal/abdominal US to rule out visceral malignancy
Beckwith-Wiedemann = Wilm’s tumor
LLD - Prediction Methods
- Arithmetic method
- Anderson & Green tables
- Moseley straight line graph
- Paley multiplier method* (most accurate)
An AMP
Limb growth / year
Leg grows 23 mm/year, with most of that coming from the knee (15 mm/yr)
- proximal femur - 3 mm / yr (1/8 in)
- distal femur - 9 mm / yr (3/8 in)
- proximal tibia - 6 mm / yr (1/4 in)
- distal tibia - 5 mm / yr (3/16 in)
Physeal Bar Excision - Indications
<50% of the physis
At least 2 yrs growth remaining
LLD General Treatment Principles (based on length)
- 0-2 cm = shoe lift
- 2-5cm = contralateral epiphysiodesis, shortening (if after skeletal maturity)
- 5-20cm = lengthening
- 15-20cm = lengthening + epiphysiodesis/shortening, amputation
Max Shortening Parameters
- 10-20% of length
- Femur 5cm
- Tibia 3cm
- Humerus 4cm
Hueter Volkmann Law
- theory suggest that mechanical forces influence longitudinal growth
- growth accelerated by tension
- compressive forces inhibit growth
- implicated in scoliosis*
- also idea behind 8-plates
Limb Lengthening Important Points
- lengthen at metaphyseal level
- delay lengthening after corticotomy by 5-7 days
- lengthen at rate of 1 mm/day (0.25mm 4x/day)
- limit lengthening to <20% bone length
- femur 8cm
- tibia 5cm
Lower Limb Alignment Milestones Through Growth
- Birth: 10-15 varus
- 6 mths: 20 varus (max)
- 18 mths: neutral
- 3 yrs: 12 valgus (up to 20)
- 6 yrs: standard 7 valgus
*Salenius and Vankka graph
Angular Measurements for Tibia Vara
- Metaphyseal-diaphyseal angle
- MD < 10 physiologic
- MD > 16 Blounts
- Tibiofemoral angle: >15 abnormal
- Epiphyseal-metaphyseal angle: >20-30 abnormal
- Ratio of femoral MDA and tibial MDA
- >1 = physiologic
- <1 = more likely Blounts (more angle from tibia than femur)
Blounts Classification
Langenskiold Classification (Infantile Blounts)
- I = beak
- II = saucer
- III = step
- IV = bent
- V = double epiphysis
- VI = bony bar
= increasing medial metaphyseal sloping with bony bridge @ V/VI
BullShit SlotBack Defensive Back
Infantile Blounts - Indications for Surgery
- Age 3-4
- Langenskiold stage III or above
- MD angle > 16
- Progressive deformity
*risk of recurrence slighly less if performed before age 4
Infantile Blounts - Surgical Principles
Proximal tibial valgus & rotational osteotomy
- Multiplanar*
- valgus, lateral translation, ER
- correct increased posterior slope
- Lateral & anterior compartment fasciotomy
- Fibular osteotomy (resect 1 cm)
- Osteotomy below tibial tubercle, at CORA
- Dome osteotomy, convex proximal
- Over-correct into 10-15 valgus
- May need bony bar resection, medial tibial plateau elevation if any joint depression (V, VI)
Skeletal Dysplasias causing Genu Valgum
PEMMS
- Pseudoachondroplasia
- Ellis-Van Creveld (EVC)
- MED
- Morquio
- SED
ME PMS
Genu Valgum - Indications for Surgery
- TF angle > 15
- Intermalleolar distance > 10cm
- Mechanical axis at lateral plateau in children age > 10
Causes of Intoeing by Age
- Infant = metatarsus adductus
- Toddler = internal tibial torsion
- >3 yrs = femoral anteversion
Lower Extremity Rotational Profile - Normals
- Femoral anteversion: 15 degrees
- Hip IR: 30-60
- Hip ER: 30-60
- Thigh-foot angle: 10 ER (-5 to +20)
- Transmalleolar axis: 20 ER
- Heel-bissector line: 2nd webspace
*GT prominence test - IR until GT most prominent = femoral anteversion
Indications for Derotation Osteotomy in Femoral Anteversion
- age > 8 with pain, <10 ER
- anteversion > 50 and IR > 80
*amount of rotation needed to correct excessive anteversion = prone IR-ER/2
*WAIT UNTIL AGE 8 to do any surgery
Tibial Bowing
- Anterolateral = NF
- pseudarthrosis
- bracing to prevent #
- if get # - IM nail & bone graft
- amp option = symes
- Posteromedial = physiologic, calcaneovalgus
- spontaneous improvement
- LLD - avg 4cm, deal with later (epiphysiodesis)
- Anteromedial = fibular hemimelia
- osteotomy with lengthening vs. amp
Optimal age for amputation in limb deficiency
10 mths - 2 yrs
- Syme - simple, tapered prosthesis
- Boyd - prevents heel pad migration, better end bearing - but longer length
PFFD - Classification
- spectrum of problems @ acetabulum, prox femur, knee, lower leg (mild/mod/severe)
- Aitken classification - Class A-D based on acetabulum and femoral head severity
- Gillespie/Torode Classification - best
- Group A = congenital short femur - long enough to allow WB
- Group B = PFFD, need prosthesis to WB, however have anterior displacement of tibia
- Group C = little to no femur, but without anterior displacement of tibia = easier prosthesis fit
Fibular Hemimelia - Associated Abnormalities
- shortening of femur (PFFD) and tibia
- hip dysplasia, coxa vara
- LLD
- genu valgum - lateral femoral condyle hypoplasia
- cruciate ligament deficiency, absent menisci
- anteriomedial tibial bowing
- patella alta
- equinovalgus
- tarsal coalition (talocalcaneal - complete)
- = ball and socket joint
- absent lateral ray(s)
Fibular Hemimelia - Classification
- Birch Classification
- depends on foot function - if preservable or requires amp
- Type 1 = foot preservable
- % inequality/LLD determines tx
- Type 2 = foot nonpreservable
- function of upper extremity determines early amp vs salvage
Tibial Deficiency - Classification
- Jones Classification
- Type 1 = tibia not seen at birth
- 1A = no tibia (no functional knee)
- 1B = upper tibia late to ossify (functional knee*)
- Type 2 = prox tibia w absent distal tibia
- functional knee*
- Type 3 = absent prox tibia w distal tibia
- more rare, no knee
- Type 4 = diastases of tib/fib
- most common
- no ankle mortise, foot in rigid equinus
- Type 1 = tibia not seen at birth
Congenital Knee Dislocation - Associated Orthoapedic Issues
- metatarsus adductus
- equinovarus foot
- CVT
- clubfoot
- ipsilateral hip dislocation (70-100%)
Congenital Knee Dislocation - Classification
- Type 1
- recurvatum, can passively flex to 90
- Type 2
- subluxation, can passively flex to 45
- Type 3
- dislocation
Congenital Patellar Dislocation - Associations
DAN LED
- Down’s
- Arthrogryposis
- Nail-Patella syndrome
- Larsens
- Ellis-Van Creveld syndrome
- Diastrophic dysplasia
DAN LED
Clubfoot Perani Grading System
- Posterior crease
- Empty heel
- Rigid equinus
- Medial crease
- Curved lateral border
- Lateral head of talus
REP MCL
- Each graded 0, 0.5, or 1
- 3 or greater = likely to need at least 4 casts with tenotomy
Clubfoot - Associated Conditions
- tibial hemimelia
- hand abnormalities
- arthrogryposis
- diastrophic dysplasia
- myelomeningocele
- amniotic band syndrome
- larsen’s
HAD MALT
Clubfoot - Posteromedial Release General Concepts
- prone, cincinnati incision
- if <18mths - TA tenotomy/lengthening
- release ankle capsule
- release subtalar joint
- FHL lengthening
- Tib post lengthening
- FDL, abductor hallucis lengthening
- *do not release plantar fascia (planovalgus)
- release TN joint
- release spring ligament
- release CC joint
- Realign foot & pin
- straight lateral border of foot
- 1st MT lateral to talar axis (abducted)
- hindfoot valgus
- Cast x 3 mths, pins out at 4-6 wks
Congenital Vertical Talus - Associated Systemic Conditions
- SNAIL
- syndromic (trisomy 13/15/18)
- neural tube defects (myelomeningocele*, sacral agenesis, diastematomelia)
- arthrogryposis
- idiopathic
- larsens
(think of snail shaped like a vertical talus - AK)
CVT - Deformity
- severe flatfoot
- fixed hindfoot equinus & valgus
- tight achilles/peroneals
- midfoot dorsiflexion
- dorsolateral dislocation of navicular on talus
- forefoot abduction and dorsiflexion
- tight dorsolateral muscles (TA/EHL/EDL/PB/PL)
- hypoplastic talar neck
- hypoplastic, wedge-shaped navicular
- talar plantar flexion
- calcaneus plantar flexion - can see cuboid d/l
- attenuation of spring ligament
CVT - Classification
- Coleman classification
- Type 1 = no cuboid subluxation/dislocation
- Type 2 = cuboid subluxation/dislocation
- harder to treat
CVT - Radiographic Findings
- Lateral radiograph
- Talar axis metatarsal base angle (TAMBA)
- >35 = pathognomonic for CVT
- navicular ossifies at age 3 - 1st MT used as proxy
- Talar axis metatarsal base angle (TAMBA)
- Forced PF lateral - Eyre Brook view
- persistent dorsal dislocation of TN joint
- *oblique talus reduces on this view
- Forced DF lateral
- persistent plantarflexion of talus and calc
- AP
- talocalcaneal angle >40 (N 20-40)
- valgus midfoot
CVT - Treatment
- serial casting to stretch dorsolateral soft tissues
- 5-6 weekly casts
- opposite correction to clubfoot - equinus last
- bring into equinus, hindfoot varus and forefoot adduction
- last cast = maximal PF and inversion
- K-wire pinning of TN joint
- retrograde from navicular into talus - want TAMBA <30 - bury pins
- small 1 cm open incision over TN joint - do not cut capsule
- unless not reduced and need to open subtalar joint with a 2 cm medial incision and transfer for tib ant to dorsal aspect of talar neck to help correct deformity*
- fractional lengthening of dorsolat tendons
- if PF limited to <25 - lengthen EDC/tib ant
- if forefoot adduction <10 - lengthen PB
- perc tenotomy of achilles (*med to lat)
- long leg cast, 5-10 DF, pins out at 6 weeks
- boots & bars full-time 2 mths, nighttime 2 yrs
Cavovarus - muscle imbalance
- peroneus longus > tib ant (PF)
- tib post > peroneus brevis (inv)
- weak instrinsics (DF MTP, flex IP)
Cavovarus - treatment options
- Depends on deformity, flexible vs fixed
- Toe deformities
- Girdlestone-Taylor (flex to ext)
- Jones (IP fusion + EHL to 1st MT neck)
- Soft tissue procedures
- plantar fascia release* (ALL)
- TAL (equinus)
- Osteotomies
- Forefoot = cotton (1st MT DF osteotomy)
- Midfoot = midfoot closing wedge osteotomy
- Calcaneus = lateral closing wedge/slide
- Tendon transfer
- tib post - to lateral cuneiform (for DF)
- PL to PB
- Arthrodesis - triple = salvage
Tarsal Coalition - Important Points
- calcaneonavicular > talocalcaneal (middle facet*)
- 50% bilateral
- associations
- fibular hemimelia
- Apert syndrome
- clubfoot
- onset of symptoms
- age 8-12 = CN
- age 12-15 = TC
Accessory Navicular - Types
- Type 1 = sesamoid in tib post tendon
- Type 2 = separate ossicle attached via synchondrosis
- Type 3 = bony enlargement
Hallux Varus - classification (congenital)
- Congenital - classification
- Type 1 = normal toe, tight AbdH
- Type 2 = polydactyly or longitudinal epiphyseal bracket*
- Delta phalanx*
- associated with tarsal coalitions, apert syndrome
- Delta phalanx*
- Type 3 = dysplasia (diastrophic)
Physis & Associated Conditions
- Reserve zone
- Gaucher disease
- Diastrophic dysplasia
- Pseudoachondroplasia
- Proliferative zone (*ECM)
- Achondroplasia
- Gigantism
- MHE
- SED
- Hypertrophic zone (Maturation zone, Degeneration zone, Zone of provisional calcification )
- SCFE
- Rickets
- Mucopolysacharide dx
- Fractures (zone of prov calc)
- Primary Spongiosa
- Scurvy
Achondroplasia
- most common skeletal dysplasia
- autosomal dominant - but 90% new mutations
- FGFR-3
- gain in function - inhibits chondrocytes = reduced endochondral ossification, reduced growth
- Growth arrest at PROLIFERATIVE zone
- Features
- Rhizomelic shortening (prox > distal limb)
- Foramen magnum stenosis*
- TL kyphosis, TL stenosis, hyperlordosis
- *narrow interpedicular distance (L1-L5)
- NOT cervical instability
- Champagne glass pelvis
- Genu varum
Pseudoachondroplasia
- autosomal dominant
- COMP mutation - chromosome 19
- epiphysis are delayed/abdnormal
- rhizomelic shortening
- normal facies
- cervical instability*
- lower extremity bowing
- joint hyperlaxity, DDH, early OA
Diatrophic Dysplasia
- autosomal recessive
- mutation in sulphate transport protein
- rhizomelic shortening
- A-A instability, cervical kyphosis
- cleft palae, cauliflower ears
Think of a messed up wrestler… like Neufeld
RACCC
Cleidocranial Dysostosis
- autosomal dominant
- proportionate dwarfism
- defect in CBFA-1 - transcription factor for osteocalcin
- affects IM ossification - skull, clavicle, pelvis
- mild short stature
- frontal bossing
- delayed teeth
- absent clavicles
- coxa vara
CAPS C FAD
Common features of mucopolysaccharidoses
- Lysosomal storage diseases
- Autosomal recessive
- Hunter’s = X-linked recessive
- Urine test - mucopolysaccharide breakdown products
- Features
- short stature
- corneal clouding
- bullet-shaped phalanges*
- mental retardation (except Morquio’s*)
- cervical instability*
- DDH
- carpal tunnel syndrome
MBCS, CDC
Mental Bowl College Champions, Centers for Disease Control
Mucopolysaccharidoses Subtypes
- I - Hurler Syndrome (most severe)
- alpha-L-iduronidase deficiency
- accumulation of dermatan sulfate
- treat with bone marrow transplant
- death in first year of life
- II - Hunter Syndrome
- X-linked recessive*
- accumulation of dermatan/heparan sulfate
- III - Sanfilippo Syndrome (most common)
- accumulation of heparan sulfate
- IV - Morquio Syndrome (most common)
- Type A (galactosamine-6-sulphatase)
- Type B (beta-galactosidase deficiency)
- accumulation of keratan sulfate
- *normal intelligence
Multiple Epiphyseal Dysplasia
- autosomal dominant
- multiple genes (COMP1, COL9A2, COL9A3)
- proportionate dwarfism
- multiple abnormal epiphyses
- shortened metacarpals/metatarsals
- valgus knees*
- double layer patella*
- DDH
- OA/joint contractures
- NO spinal involvement*
AMP MS VODD
Spondyloepiphyseal Dysplasia
- proportionate dwarfism
- cervical instability***
- platyspondyly
- SED congenita
- COL2A1 gene
- proliferative zone affected
- coxa vara, genu valgum
- retinal detachment, myopia
- SED tarda
- X-linked recessive, SEDL gene
- milder, later onset
SED - S = Second (two types)
Spondylo = spine (cervical spine/platyspondyly)/skeleton (coxa vara/genu valgum)
Epiphyseal = eye (retinal detachment/myopia)
Radial Head Dislocation - Associations
SMASHED LUCK NK
- Silver’s syndrome
- Marfan’s
- Achondroplasia
- Steel syndrome
- Hereditary multiple exostosis
- Ehler’s Danlos
- Down syndrome
- Larsen’s syndrome
- Ulnar longitudinal deficiency
- Cornelia de Lange
- Klippel-Feil syndrome
- Nail-Patella syndrome
- Klinefelter’s
Marfan’s Syndrome
- Autosomal dominant
- Fibrillin-1 gene (FBN1) - chromosome 15q21
- MSK - tall, thin etc
- 60-70% have scoliosis
- narrow pedicles, wide TPs, vertebral scalloping
- dural ectasia*
- acetabular protrusio
- pes planovalgus
- superior lens dislocation
- aortic root dilatation
- Ghent classification
- 1 major criterion from 2 different organ systems + 3rd system
Dural ectasia associations
- Marfan’s
- Ehler’s Danlos
- Achondroplasia
- NF
MEAN
Ehlers-Danlos Syndrome
- Classification
- Classic (Type 1, gravis)
- Autosomal dominant
- COL5A1, COL5A2 = type V collagen
- Type III - hypermobility
- Autosomal dominant
- Type VI - kyphoscoliosis
- Autosomal recessive
- Mutation in lysyl hydroxylase
- Severe kyphoscoliosis
- Type VII - arthrochalasis
- autosomal dominant
- COL1A1, COL1A2
- congenital bilateral hip dislocation, hypermobility
- Classic (Type 1, gravis)
Ca/Phos/Vit D homeostasis
OI - Classification
- Type 1 (dominant, blue sclera)
- AD
- Type II (lethal perinatal)
- AD
- Type III (progressive deforming)
- AR
- Type IV (dominant, white sclera)
- AD
VACTERL
Vertebral anomalies
Anal anomalies
Cardiac anomalies
Tracheo-esophageal fistula
Renal anomalies
Limb anomalies (radial deficiency)
Syndromes associated with cervical instability
Skeletal dysplasias:
- Pseudoachondroplasia
- MPS - Morquio’s (type IV)
- SED
- Diatrophic dysplasia
Connective tissue:
- Down’s
- Marfans
- Larsens
- Ehlers-Danlos
NOT achondroplasia*
Risk factors for DVT in paeds MRSA osteomyelitis
CRP > 6
Age > 8
MRSA
PVL (Panton-Valentine leukocidin)
Surgical treatment
CAMPS*
Panton-Valentine leukocidin (PVL) associated with
Some strains of CA-MRSA harbor genes encoding for Panton-Valentine leukocidin (PVL)
- Complex infections
- Multifocal infections
- Prolonged fever
- Myositis
- Pyomyositis
- Intra-osseus or subperiosteal abscess
- Chronic osteomyelitis
- DVT
DIS C2M2P2
