Peds Flashcards
1
Q
Obstetrical Clavicle Fracture - Risk factors
A
• Large birth weight (>4kg) • Shoulder dystocia • Prolonged gestation • Forceps delivery
2
Q
Medial Epicondyle Fracture – Surgical Indications
A
• 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)
3
Q
Infantile Scoliosis - Risk of progression
A
• RVAD > 20 degrees • Phase 2 rib • Cobb angle > 30 degrees
4
Q
Describe the Kocher Criteria
A
Septic hip? • Temp > 38.5 • CRP • ESR • Refusal to WB • WBC count
5
Q
Septic Arthritis Poor prognostic factors
A
• Age < 6 months • Delay to treatment > 4 days • Hip • Joint effusion with underlying osteomyelitis
6
Q
Neonatal Infections: Risk factors in NICU
A
• Phlebotomy sites • Indwelling catheters • Invasive monitoring • Peripheral alimentation • IV drug administration
7
Q
Lyme Disease Features
A
NIVEA • Neuropathies • Intermittent reactive arthritis • Cardiac arrhythmias (“V-Tach”) • Erythema migrans (“bulls eye” rash) • Acute arthritis
8
Q
ACL “Rules” to avoid physeal arrest
A
- 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)
9
Q
NF dystrophic scoliosis features
A
- 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)
10
Q
Proteus Syndrome - 3 characteristic findings
A
- hemihypertrophy
- macrodactyly
- partial gigantism of hands/feet/both
11
Q
Hemihypertrophy - associated with (4)
A
- Klippel-Trenaunay-Weber syndrome
- Proteus
- NF-1
- Beckwith-Wiedemann syndrome
12
Q
Syndactyly - associated with (4)
A
PACU
- Poland syndrome
- Apert syndrome
- Congentical constriction band syndrome (Streeters syndrome)
- Ulnar longitudinal deficiency
13
Q
Order of frequency of syndactyly webbing
A
- 3rd webspace
- 4th webspace
- 2nd webspace
- 1st webspace
14
Q
Poland Syndrome
A
- 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
15
Q
Apert Syndrome
A
- FGFR2***
- Associated with (TAMP)
- tarsal coalition
- acrosyndactyly (spadelike hand)
- mental retardation
- premature fusion of cranial sutures
16
Q
Trigger thumb
A
- 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
17
Q
Clinodactyly
A
- radioulnar deformity of 5th digit
- autosomal dominant
- Associated with:
- Down syndrome (80%)
- Russel-Silver syndrome
- Feingold syndrome
18
Q
Risk factors for brachial plexus birth palsy
A
- macrosomia
- multiparous pregnancy
- prolonged labour
- difficult delivery
- shoulder dystocia
- difficult arm/head extraction in breech
- previous BPBP
19
Q
Natural history of brachial plexus birth palsy
A
- 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)
20
Q
Brachial plexus birth palsy - signs of preganglionic lesion (4)
A
- horner syndrome
- elevated hemidiaphragm
- winged scapula
- absence of rhomboid/rotator cuff/lat dorsi function
21
Q
Brachial plexus birth palsy - Indications for microsurgery
A
- no biceps function at 3-6 mths
- flail extremity + horners at 3 mths
- others say - flail or horners @ 1 mth
22
Q
Erb’s Palsy treatment
A
- “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)
23
Q
What is Sprengel’s deformity
A
- small, undescended scapula
- associated with
- scapular winging
- hypoplasia
- omovertebral connections (bony or fibrous connection from scapula to vertebral column)
24
Q
Disorders/diseases associated with Sprengel’s
A
- Congenital scoliosis
- Klippel-Feil
- Torticollis
- Poland Syndrome
- Facial asymmetry
- UE abnormalities
- Diastematomyelia
- Pulmnary or renal dx
PPT FUCKeD
25
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
26
Teratologic DDH associations
* arthrogryposis
* myelomeningocele
* SMA
* CMT
* CP
* Larsen's
* Ehlers-Danlos
* Down Syndrome
* Mucopolysaccharoidosis (later in life)
27
DDH associated with packaging problems
* prematurity
* oligohydramnios
* multiparous birth
* congenital knee dislocation
* congenital torticollis (20%)
* metatarsus adductus (10%)
28
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
29
DDH Ultrasound Parameters
* Alpha angle = bony acetabular roof + ileum
* Normal \> 60
* Beta angle = cartilaginous acetabular roof + ileum
* Normal \< 55
* \*BIG BETA BAD
30
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
31
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
32
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)
33
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
34
DDH Indications for Open Reduction
* failed closed treatment
* non-concentric reduction (likely block present)
* reduction requiring extreme, dangerous positioning
* gross instability
35
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
36
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
37
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
38
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
39
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\*\*\*
40
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
41
Perthes - Waldenstrom's Radiographic Stages
1. Initial (xrays normal 3-6 mths)
2. Fragmentation (lasts 1 yr) - use all classifications during this stage\*
3. Re-ossification (3-5 yrs)
4. Remodeling
42
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
43
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
44
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\*
45
Perthes - Salter-Thompson Classification
* Group A = subchondral fracture/crescent affected \<50% of femoral head
* Group B = \>50%
46
Salter Pelvic Osteotomy - Amount of Coverage Achieved
up to 15 deg lateral coverage
and 25 deg anterior coverage
47
Risk Factors for SCFE
* african descent/pacific islanders
* male gender
* renal failure
* endocrine abnormalities
* radiation/chemo
* obesity
* down syndrome
REAM ROD
48
Indications for Endocrinology workup in SCFE
* Age \< 10
* Weight \< 50th percentile
Labs: TSH, GH, BUN/Cr (renal osteodystrophy)
49
Conditions that weaken physis (associated with SCFE)
* hypothyroidism
* panhypopituitarism
* GH abnormalities
* hypogonadism
* hyper- or hypoPTH
* renal osteodystrophy
* previous radiation/chemo
50
Physeal zone affected in SCFE
HYPERTROPHIC
| (think fat kid)
51
Deformity of distal fragment in SCFE
* Varus in coronal plane
* Extension in sagital plane
* External rotation in axial plane
* (VAR-EX-EX)
52
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
53
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)
54
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
55
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
56
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
57
Coxa Vara - Types
* Developmental (genetics - AD)
* Congenital (eg with PFFD)
* Dysplastic (skeletal dysplasias, OI, rickets)
* Acquired/traumatic
C-DAD
58
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
59
Hemihypertrophy - important association\*\*\*
Get renal/abdominal US to rule out visceral malignancy
Beckwith-Wiedemann = Wilm's tumor
60
LLD - Prediction Methods
* Arithmetic method
* Anderson & Green tables
* Moseley straight line graph
* Paley multiplier method\* (most accurate)
An AMP
61
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)
62
Physeal Bar Excision - Indications
\<50% of the physis
At least 2 yrs growth remaining
63
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
64
Max Shortening Parameters
* 10-20% of length
* Femur 5cm
* Tibia 3cm
* Humerus 4cm
65
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
66
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
67
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
68
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)
69
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
70
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
71
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)
72
Skeletal Dysplasias causing Genu Valgum
PEMMS
* Pseudoachondroplasia
* Ellis-Van Creveld (EVC)
* MED
* Morquio
* SED
ME PMS
73
Genu Valgum - Indications for Surgery
* TF angle \> 15
* Intermalleolar distance \> 10cm
* Mechanical axis at lateral plateau in children age \> 10
74
Causes of Intoeing by Age
* Infant = metatarsus adductus
* Toddler = internal tibial torsion
* \>3 yrs = femoral anteversion
75
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
76
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
77
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
78
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
79
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
80
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)
81
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
82
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
83
Congenital Knee Dislocation - Associated Orthoapedic Issues
* metatarsus adductus
* equinovarus foot
* CVT
* clubfoot
* ipsilateral hip dislocation (70-100%)
84
Congenital Knee Dislocation - Classification
* Type 1
* recurvatum, can passively flex to 90
* Type 2
* subluxation, can passively flex to 45
* Type 3
* dislocation
85
Congenital Patellar Dislocation - Associations
DAN LED
* Down's
* Arthrogryposis
* Nail-Patella syndrome
* Larsens
* Ellis-Van Creveld syndrome
* Diastrophic dysplasia
DAN LED
86
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
87
Clubfoot - Associated Conditions
* tibial hemimelia
* hand abnormalities
* arthrogryposis
* diastrophic dysplasia
* myelomeningocele
* amniotic band syndrome
* larsen's
HAD MALT
88
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
89
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)
90
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
91
CVT - Classification
* Coleman classification
* Type 1 = no cuboid subluxation/dislocation
* Type 2 = cuboid subluxation/dislocation
* harder to treat
92
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
* 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
93
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
94
Cavovarus - muscle imbalance
* peroneus longus \> tib ant (PF)
* tib post \> peroneus brevis (inv)
* weak instrinsics (DF MTP, flex IP)
95
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
96
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
97
Accessory Navicular - Types
* Type 1 = sesamoid in tib post tendon
* Type 2 = separate ossicle attached via synchondrosis
* Type 3 = bony enlargement
98
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
* Type 3 = dysplasia (diastrophic)
99
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
100
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
101
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
102
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
103
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
104
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
105
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
106
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
107
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)
108
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
109
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
110
Dural ectasia associations
* Marfan's
* Ehler's Danlos
* Achondroplasia
* NF
MEAN
111
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
112
Ca/Phos/Vit D homeostasis
113
OI - Classification
* Type 1 (dominant, blue sclera)
* AD
* Type II (lethal perinatal)
* AD
* Type III (progressive deforming)
* AR
* Type IV (dominant, white sclera)
* AD
114
VACTERL
Vertebral anomalies
Anal anomalies
Cardiac anomalies
Tracheo-esophageal fistula
Renal anomalies
Limb anomalies (radial deficiency)
115
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\*
116
Risk factors for DVT in paeds MRSA osteomyelitis
CRP \> 6
Age \> 8
MRSA
PVL (Panton-Valentine leukocidin)
Surgical treatment
CAMPS\*
117
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
