Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves

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...

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Main Authors: Pankaj Kumar Mishra, Amol Dubepuria, Sanjiv Gaur
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2019-04-01
Series:Journal of Clinical and Diagnostic Research
Subjects:
Online Access:https://jcdr.net/articles/PDF/12767/40367_CE[Ra1]_F(AC)_PF1(AJ_SL)_PFA(AJ_SHU)_PN(SL).pdf
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spelling doaj-26b7782d39c44b15a645f3ba86d0364c2020-11-25T02:25:12ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2019-04-01134RC01RC0510.7860/JCDR/2019/40367.12767Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management EvolvesPankaj Kumar Mishra0Amol Dubepuria1Sanjiv Gaur2Assistant Professor, Department of Orthopaedics, Gandhi Medical College, Bhopal, Madhya Pradesh, India.Third Year Resident, Department of Orthopaedics, Gandhi Medical College, Bhopal, Madhya Pradesh, India.Professor and Head, Department of Orthopaedics, Gandhi Medical College, Bhopal, Madhya Pradesh, India.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.https://jcdr.net/articles/PDF/12767/40367_CE[Ra1]_F(AC)_PF1(AJ_SL)_PFA(AJ_SHU)_PN(SL).pdfgenu valgumosteochondromapsychosocial issuesquatting
collection DOAJ
language English
format Article
sources DOAJ
author Pankaj Kumar Mishra
Amol Dubepuria
Sanjiv Gaur
spellingShingle Pankaj Kumar Mishra
Amol Dubepuria
Sanjiv Gaur
Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
Journal of Clinical and Diagnostic Research
genu valgum
osteochondroma
psychosocial issue
squatting
author_facet Pankaj Kumar Mishra
Amol Dubepuria
Sanjiv Gaur
author_sort Pankaj Kumar Mishra
title Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
title_short Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
title_full Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
title_fullStr Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
title_full_unstemmed Hereditary Multiple Exostoses and Orthopaedist- Till the Plausible Management Evolves
title_sort hereditary multiple exostoses and orthopaedist- till the plausible management evolves
publisher JCDR Research and Publications Private Limited
series Journal of Clinical and Diagnostic Research
issn 2249-782X
0973-709X
publishDate 2019-04-01
description 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.
topic genu valgum
osteochondroma
psychosocial issue
squatting
url https://jcdr.net/articles/PDF/12767/40367_CE[Ra1]_F(AC)_PF1(AJ_SL)_PFA(AJ_SHU)_PN(SL).pdf
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