Journal of Clinical and Diagnostic Research (Apr 2019)

Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves

  • Pankaj Kumar Mishra,
  • Amol Dubepuria,
  • Sanjiv Gaur

DOI
https://doi.org/10.7860/JCDR/2019/40367.12767
Journal volume & issue
Vol. 13, no. 4
pp. RC01 – RC05

Abstract

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Introduction: Hereditary Multiple Exostoses (HME) is an inherited genetic skeletal disorder of enchondral bone. It is an autosomal dominant disorder affecting juxtaepiphyseal region of the long bones and includes multiple exostoses. The treatment of HME is mainly surgical, while the medical approach is still in evolving phase. Surgery is needed only in symptomatic cases to avoid eventual intricacy. Limb length equalization, deformity correction by osteotomy and epiphysiodesis are frequently entertained procedures. Aim: To carry out the descriptive study over HME patients with clinical profile and surgical execution. Materials and Methods: It was a prospective study involving 17 cases. The present institute provides tertiary care as well; caters to the major number of cases, even from long distance. So it is assumed that most of the HME patients would have been referred to or visited the hospital only after moderate to severe functional deficit in their lifestyle. All the patients were dealt with according to their complaint and the Short Assessment of Patient Satisfaction (SAPS) score was obtained. Results: Three patients (17.6%) were very satisfied, 11 patients were satisfied (64.7%), 3 (17.64%) patients were dissatisfied and one patient was very dissatisfied with the treatment executed. Conclusion: The orthopaedic surgeon is the first treating doctor who faces diagnosis and treats HME patients. Orthopaedists should be cognizant to clinical display of it, along with its differential and aftermath. Surgical mediation often needed to excise the clinically obliged exostoses only.

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