Achondroplasia
Normal IQ , co-operative
Head and face
large head , frontal bossing
mid face hypoplasia
dental malocclusion
Skeletal features
disproportionate short stature
normal trunk length with rhizomelic shortening of proximal limbs with
redundant skin folds
brachydactyly and trident hands
lumbar lordosis
hyperextension knees
limited elbow extension and rotation
bowed legs
Radiologically
1. small skull base
2.progressive interpedicular narrowing L-S spine
3.short pedicles leading to spinal stenosis
4.short femoral neck and metaphyseal flaring with inverted V-shape distal physis
5. small sacrosciatic notch , flat roofed acetabulum
Orthopaedic problems
1.craniocervical junction abnormailities
cord compression
risk of contact sports , car accident and intubation
2.lumbar stenosis with neurological claudication
3. kyhposis
Management
Growth hormone treatment under evaluation
management of complications
Head and face
large head , frontal bossing
mid face hypoplasia
dental malocclusion
Skeletal features
disproportionate short stature
normal trunk length with rhizomelic shortening of proximal limbs with
redundant skin folds
brachydactyly and trident hands
lumbar lordosis
hyperextension knees
limited elbow extension and rotation
bowed legs
Radiologically
1. small skull base
2.progressive interpedicular narrowing L-S spine
3.short pedicles leading to spinal stenosis
4.short femoral neck and metaphyseal flaring with inverted V-shape distal physis
5. small sacrosciatic notch , flat roofed acetabulum
Orthopaedic problems
1.craniocervical junction abnormailities
cord compression
risk of contact sports , car accident and intubation
2.lumbar stenosis with neurological claudication
3. kyhposis
Management
Growth hormone treatment under evaluation
management of complications
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