MSK 3 Flashcards
what is osteogenesis imperfect
majority that are ADom
AR
brittle bone disease. defect of maturation and organisation of type 1 collagen
multiple fragility fractures of childhood, short, blue sclera, loss of hearing
fatal in perinatal period or associated with spinal deformity
osteogenesis imperfetta what are the bones like
mild type
treatment
thin (gracile) with thin cortices and osteopenia
relatively normal XR with history of low energy fractures
splint age, traction or surgical stabilisation
progressive deformity - multiple osteomies and intramedullary for correction (sofield procedure)
what is skeletal dysplasia genetics what is it how many are there which is the commonest and assoc features
short <2nd gentile
genetic heriditaty/sporadic
abnormal development of bone and connective tissue. proportionate or disproportionate length of spine and limbs
>300 types. commonest type is achondroplasia
disproportionately short limbs with predominant forehead and wide nose. joints are lax and mental development normal
other skeletal dysplasia are assoc with what
ix
treatment
LD, spine deformity, limb deformity, internal organ dysfunction, craniofacial abnormality, skin abnormality, tumour formation, joint hyper mobility, atlanta axial sublux, myelopathy, IUD
genetic testing of child and family
deformity correction including scoliosis, limb lengthening, GH therapy may be appropriate
gen familial ligamentous laxity pop %
genetic
symp
50% have hypermobility
Dom inheritance
more prone to sofft tisue injuries (ankle sprain) and recurrent dislocation of joints
Marfans genetic
symptoms/signs
what causes early death
treatment
ADom or sporadic mutation of fibrin gene
tall with disproportionately long limbs, ligamentous laxity, high arched palate, scoliosis, archonacdylt, pevux excavated, lens dislocation, retinal detachment, glaucoma, sponeqtous pneumothorax, aortic aneurysm, AR, MVP, MR
cardiac abnormalities
scoliosis correction, bony procedures for joint laxity example fusion
Ehlos dances syndrome genetics and what is it types symptoms trearment complication if surgery done
Adom
abnormal elastin and collagen formation
>10 types
profound joint hyper mobility, vascular fragility with ease of bruising, joint instability and scoliosis
boney surgery for dislocating joints
bleeding and skin healing is poor
downs msk symptoms
short
joint laxity
atlanto-axial instability in C spine
cerebral palsy causes spastic ataxic athetoid monoplegic diplegis hemiplegic total body involevemtn other features treatment
genetic, brain malfunction, intrauterine infection, prematurity, intracranial haemorrhage, hypoxia, meningitis
weakness and spascitiy cerebellum extrapyramidal motor system, pyramidal tract, BG 1 leg both one leg and one arm all 4 limbs joint contractions, scoliosis, hip dislocation
non surgical: physio and splint age, baclofen injected intrathecally into subarachnoid space, botox, orthotics
surgical: hip replacement, surgical release of contractions
spina bifocal oculta
cytica and types
treatment
pes cavus and clawing of toes
dimple/tuft of hair in skin over defect
meningocele
myelomeningocele motor and sensory
hydrocephalus - shunt
scoliosis correction, reduction and containment procedures for hip
polio is what where does the virus enter and initial symptoms resolvement of virus other symp treatment
viral infection that affects motor anterior horn cells in spinal cord or basal ganglia - LMN
GI tract with flu like illness and variable degree of paralysis in one limb group of msuciesl within 2-3 days
some recover with weakness however some have residual paralysis
joint deformities, growth defects, shortening of limb, sensation preserved
treatment of residual paralysis - splint age, shoe raise for shortened limb,
tendon transfers, fusion
syndactyl
polydactyl
tarsal coalition
failure of separation of skin/soft tissue. surgical separation at age 3-4
extra digit. remove
congenital fusion of C+W or T+C. painful flat foot in later childhood. surgical divide
fibular hemimelia is what
symptoms
treatment for mild/severe
partial or complete absence of fibula
shortened limb, bowing of tibia, ankle deformity
mild: limb lengthening with a circular frame external fixation
severe: ankle amputation at 10m-2y with use of prosthetic blow knee limb
absence/hypoplasia of radium
underdevelopment of hand - absent thumb
marked deformity - radical club hand
surgical reconstruction
erBs palsy
klumpkes
c5 and c - waitress tip
c8 and t1
DDH in who RF: what happens if left untreated ix mild treatment: severe: persitant
girls in left hip l>r
FH, breech, first born, downs, presence of congenital disorders
shortened lower limb, arthritis
ortalani/barlow
USS. femoral head ossifies at 4-6m so no XR
observe
pelvic harness 6w full time 6w part time. can be used until 4-6m until hip stable
> 18m open reduction
transient synovitis in who commonest what symp ix rx
self limiitng inflammation. 2-10 year old boys
hip pain in childhood
after URTI, limp/reluctance to bear to weight. decreased ROM
imaging to exclude perthes disease
CRP exclude septic arthritis
MRI for osteomyelitis
NSAIDs for pain
perthes cause
what is it in who
symptoms
idiopathic. skeletal dsplasia/thrombophilia if bilateral
transient blood loss. active short boys 4-9yo
unilateral pain and limp, loss of internal rotation and then of abduction and later pos tredelenburg
slipped upper femoral epiphyses symptoms and in who
ix
rx
overweight prepubertal boys. acute sudden onset pain and limp. pain in hip or in knee. loss of internal rotation of hip
lateral XR best
urgent surgery to pin femoral head
extensor mechanism problem in who
tibial tubercle
inferior pole of patella
treatment
adolescents. pattelar tendonitis
osgood chatters
sinding larseon johansonn
self limiting physio rest
meniscal problems some children have what
symp
rx
weird shaped
circular rather than C shaped
pain, popping
arthroscopic partial meniscectomy
scoliosis is what
causes
if painful
lateral curvature of psine
idiopathic. NM disease, tumour, skeletal dysplasia, infection
MRI
spondolythoesis is what where who cause symp treatment
slippage of one vertebra over another. L4/L5 or L5/S1
adolescent. development deficit or recurrent stress fractures
low back pain, may have radiculopathy, may have flat back
minor degree of slippage - rest and physio
severe - stabilise and possible reduction
