msk cortex - paediatric orthopaedics Flashcards
What type of collagen disorder is osteogenesis imperfect a
Type 1
How does osteogenesis imperfecta present
Blue sclera
Fragility fractures
Short stature with multiple deformities
Loss of hearing
*aka brittle bone disease
Is autosomal dominant
What is the most recognised type of skeletal dysplasia
Achondroplasia - disproportionately short limbs with a prominent forehead and widened nose
What type of collage results in joint hypermobility
Type 1 - mainly affects bone tendon and ligaments
Eye problems associated with Marfan’s
Lens dislocation and retinal detachment
Heart problems associated with Marfans
Aortic aneurysm
Cardiac valve abnormalities
What type of inheritance pattern are muscular dystrophies
Usually x-linked recessive (only affects boys)
What is Gower’s sign?
+ duchenne muscular dystrophy
Prognosis of patients with duchenne muscular dystrophy
Poor :(
By age 20 there is progressive cardiac and respiratory failure —> death in the early 20s usually
Diagnosis of duchenne muscular dystrophy
Raised serum creatinine phosphokinase and abnormalities on muscle biopsy
Management of duchenne muscular dystrophy
Physiotherapy
Splintage
Deformity correction
Severe scoliosis can be corrected by spinal surgery
What is the most common type of Cerebral palsy
Spastic - 80%
Non-pharmacological treatment of cerebral palsy
Physiotherapy
Splintage
Management of Cerebral palsy
Baclofenac - given in the subarachnoid space
Botox - reduce spasticity
Surgery - hip excision or replacement to treat a painful hip dislocation
What is spina bifida
Two halves of the posterior vertebral arch fail to fuse
There are two types - oculta and cytsica
Oculta = pes cavus
Cystica = herniating of vertebral canal - type of myelomeningoceal
What is Erb’s palsy
Type of obstetric brachial plexus palsy
With injury to the upper C5-C6 nerve roots resulting in loss of motor innervation of deltoid , supraspinatus , infraspinatus , biceps and brachialis muscles
*waiter’s tip posture
Erb’s palsy
Due to internal rotation of the humerus
Treatment of Erb’s palsy
Physiotherapy —> good prognosis
Surgical release of contracture and tendons also may be required if there is no recovery
What is Klumpke’s palsy ?
Another brachial plexus palsy - and is due to injury or lower brachial plexus roots (C8-T1)
Leads to paralysis of intrinsic hand muscles