Treatment of Other Skeletal Dysplasias

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Extensive limb lengthening is also possible for other types of dysplasias:

  • pseudoachondroplasia
  • spondyloepiphyseal dysplasia
  • chondroectodermal dysplasia
  • diastrophic dwarfism

These are characterized as fragile-joint dysplasias. In these patients, the pressure of lengthening can seriously harm their joints. To avoid this, Dr. Paley will extend the fixators across the joints to protect them and minimize pressure. Soft tissue releases can also be performed if they become tight.

Ipsilateral Lengthening

Dr. Paley’s treatment strategy for more complex dysplasias is an ipsilaterl lengthening: lengthening of all one side (one femur, one tibia) followed by lengthening of the other side. This strategy is very different from that for achondroplasia, which lengthens all four lower extremity bones simultaneously (two femurs, two tibias).

Pseudoachondroplasia often has very serious associated deformities and the primary goal of treatment is deformity correction; lengthening is secondary. Some examples include flexion deformity of the femur, recurvatum of the tibia, valgus in the femur (knock knee), varus in the tibia (bowing), and windswept deformity. Dr. Paley can correct all these during surgery. There is a large variety of rare and complex dysplasias, and Dr. Paley will address each patient individually, establishing a treatment plan that will comprehensively treat the deformities.

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