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Chondrocytes form a temporary cartilaginous model by dividing and secreting a thick gel-like extracellular matrix. Once the chondrocytes undergo programmed cell death, osteoblasts enter the site of the cartilaginous model. The process of replacing the temporary cartilaginous model with bone in an ordered manner is called endochondral ossification. In endochondral ossification, not all of the cartilage is replaced by bone tissue. Some cartilage that performs a protective and supportive function...
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Many human characteristics, like height, are shaped by both nature—in other words, by our genes—and by nurture, or our environment. For example, chronic stress during childhood inhibits the production of growth hormones and consequently reduces bone growth and height. Scientists estimate that 70-90% of variation in height is due to genetic differences among individuals, and 10-30% of variation in height is due to differences in the environments that individuals experience,...
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Growth Modulation in Achondroplasia.

Philip K McClure1, Eray Kilinc, John G Birch

  • 1Texas Scottish Rite Hospital, Dallas, TX.

Journal of Pediatric Orthopedics
|July 19, 2017
PubMed
Summary

Growth modulation can correct leg deformities in achondroplasia (a form of dwarfism). Early intervention is key, though some patients may still require osteotomy for full correction.

Area of Science:

  • Orthopedics
  • Genetics
  • Pediatric Surgery

Background:

  • Achondroplasia is the most common skeletal dysplasia, affecting nearly 1 in 10,000 individuals.
  • Lower extremity deformities are common in achondroplasia, with various surgical corrections documented.
  • No prior reports exist on using growth modulation for angular deformity in achondroplasia.

Purpose of the Study:

  • To evaluate the efficacy of growth modulation in correcting angular lower extremity deformities in achondroplasia patients.
  • To analyze outcomes and identify optimal timing for growth modulation procedures in this population.

Main Methods:

  • Retrospective review of medical records from 1985-2015 for achondroplasia patients treated with growth modulation.
  • Analysis of preoperative and final radiographs to assess deformity correction.

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  • Detailed case review of four patients with varus leg deformity.
  • Main Results:

    • Four patients with varus leg deformity underwent growth modulation; three had bilateral procedures.
    • Some improvement in alignment was observed in all limbs.
    • Only one limb achieved neutral axis correction with growth modulation alone; one patient required subsequent osteotomies.

    Conclusions:

    • Growth modulation is a viable option for correcting angular deformities in achondroplasia.
    • Earlier implantation of growth modulation devices may be necessary compared to non-achondroplasia patients.
    • Osteotomy may still be required for residual deformity after growth modulation.