Flashcards in Paediatric Orthopaedics Deck (70):
brittle bone disease - autosomal dominant
how does Osteogenesis imperfecta present?
multiple fractures in childhood
loss of hearing
what causes Osteogenesis imperfecta?
defect in the maturation and organisation of type 1 collagen
what does XR show in Osteogenesis imperfecta?
bones tend to be thin cortices and oesteopenia
how is Osteogenesis imperfecta treated?
fractures tend to heal with abundant but poor quality callus - spintahe and traction
what is a possible complication of Osteogenesis imperfecta?
oesteopenia - results from prematurity and result in lower energy fractures
what is skeletal dysplasia?
medical term for short stature
how does skeletal dysplasia present?
joints are lax - mental development is normal
what causes skeletal dysplasia?
abnormal development of bone and connective tissue
what is the most common type of skeletal dysplasia?
how is skeletal dysplasia treated?
orthopaedic treatment - deformity correct and limb lengthening
how does marfin's present?
tall stature + long limbs with ligamentous laxity
high arched palette
pectus excavatum (flattening ot chest)
eye problems - lens dissolution
what causes marfin's?
mutation in fibrillin gene - autosomal dominant
what is ehler's danlos syndrome?
heterogeneous condition which abnormal elastin and collagen formation
how does ehler's danlos syndrome present?
profound joint hyper mobility, vascular fragility with easy bruising
joint instability and scoliosis
how is ehler's danlos syndrome treated?
bony surgery - dislocated joints but bleeding can be a problem so you can get stretched cards and wound healing
what is duchenne Muscluar dystrophy?
X linked hereditary disorder resulting in progressive muscle weakness and wasting
how does duchenne Muscluar dystrophy present?
muscle weakness - usually noted when boys begin to walk
what is gower's sign?
difficulty in walking + use of hands
what causes duchenne Muscluar dystrophy?
defect in dystrophin involving in Ca2+ transport results in muscle weakness
how is duchenne Muscluar dystrophy diagnosed?
raised serum creatinine phosphokinase
abnormalities in muscle biopsy
what is backer's muscular dystrophy?
similar to DMD but milder and boys able to walk in teens and suffers may survive into 30s
what is cerebral palsy?
neuromuscular disorder with onset before 2-3 years of age to insult of brain during birth
what is the most common type of CP?
what is spastic CP?
injury to corticospinal tract which results in muscle weakness and spasticity and worsens when child grows
what is ataxic CP?
affects cerebellum and so results in lack of co-ordinationa and balance
what is athetoid CP?
affects extra-pyramidal system + basal ganglia so uncontrolled withering motion, difficulty in speech
what is spina bifida?
congenital disorder where 2 halves of posterior vertebral arch fail to fuse
what are the 2 types of spina bifida?
spina bifida occulta
spina bifida cystica
what is spina bifida occult?
deep tethered of spinal cord and roots
clawed toes and high arched foot
what is the tell tale sign of spina bifida occult?
tuft of hair in skin overlying defect
what is spina bifida cystica?
contents of vertebral canal herniate through defect with either herniation and meninges alone or with spinal cord
what is spina bifida cystica associated with?
how is spina bifida treated?
defect closed within 48 hours
hydrocephalus - treated with VP shunt
what is polio/
viral infection with affects motor anterior horn cells in spinal cord result in motor neurone deficit
how does polio present?
muscle paralysis which recovers but can be left
joint deformities and growth defects can occur
how is polio managed?
residual paralysis - splint-age, shortening of limb - show raise
what is erb's palsy?
injury to C5/6
what is affected in erb's palsy?
loss of motor innervation of deltoid, suprspinatous, biceps, infraspinatos and brachialis muscle
how does erb's palsy present?
internal rotation of humerus + waiter's tip posture
what is Klumpke's palsy?
injury to C8-T1
how is Klumpke's palsy caused?
forceful adduction which results in intrinsic hand muscles - fingers are flexed
what is development dysplasia of the hip?
dislocation/subluxation of femoral head during perinatal period which affects subsequent development of the hip
how does DDH present?
asymmetric groin/thigh crease
how is DDH diagnosed?
ortolani - reducing dislocated hip wiwth abduction and anterior displacement
barlow's - dislocate able hip with flexion and posterior displacement
how is DDH treated?
Pavlick's harness - maintains reduction
what is the transient synovitis of the hip?
self limiting inflammation of the synovium of a joint, mostly hip after an upper respiratory tract infection
how does transient synovitis of the hip present?
range of motion restricted
how is transient synovitis of the hip treated?
short course of NSAIDs
what is Perthe's disease?
idiopathic oesteochonditis of femoral head which usually present between 4-9 years
what group of people is Perthe's disease most common in?
very active boys with short stature
how does Perthe's disease present?
pain and limp
loss of internal rotation
loss of abduction
positive trendellenburg's test
what causes Perthe's disease?
femoral head transiently loses blood supply resolution in necrosis with abnormal growth
how is Perthe's disease treated?
surgery if the femoral head becomes aspherical and flattened
what is SUFE?
femoral head slips inferior in relation to the femoral neck
what causes SUFE?
growth plate is not strong enough to hold body weight
how does SUFE present?
pain in groin
pain can present in the knee
loss of internal rotation
why can SUFE present in the knee?
due obturator nerve supply
what predisposes you to SUFE?
how is SUFE treated?
urgent surgery to pin femoral head down
what is oestochonritis dissecans?
osteochondritis where fragment of hyaline cartilage with variable amount of boney fragments break off surface of the joint
how does oestochonritis dissecans present?
medial femoral condyle is most commonly affected
poor localised pain
how is oestochonritis dissecans treated?
lesions which are risk of breaking off are fixed and loose fragments are removed
what are the possible complications in oestochonritis dissecans?
loose bodies within knee joint - pot hole on surface - oestoarthritis
what is scoliosis?
lateral curvature of the spin - rotational deformity
how is scoliosis diagnosed?
MRI - painful
how is scoliosis treated?
surgery - complex - vertebral fusion and long rods connecting the poster elements of the spine
what is Spondylolisthesis?
slippage of one vertebral disc over another
how does Spondylolisthesis present?
low back pain
paradoxical flat back due to muscle spasm