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






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