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Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report

INTRODUCTION: Hereditary multiple exostosis (HME) is an autosomal dominant disorder affecting the skeletal system, which is characterized by multiple osteochondromas in bones arising from osteochondral ossification and leading to skeletal deformities, short stature, soft tissue, and neurovascular co...

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Autores principales: Mahajan, Neetin P, Wadia, Farokh, S, Prasanna Kumar. G, Yadav, Amit Kumar, Narvekar, Mrugank, Kondewar, Pranay
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Indian Orthopaedic Research Group 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885660/
https://www.ncbi.nlm.nih.gov/pubmed/33623755
http://dx.doi.org/10.13107/jocr.2020.v10.i04.1780
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author Mahajan, Neetin P
Wadia, Farokh
S, Prasanna Kumar. G
Yadav, Amit Kumar
Narvekar, Mrugank
Kondewar, Pranay
author_facet Mahajan, Neetin P
Wadia, Farokh
S, Prasanna Kumar. G
Yadav, Amit Kumar
Narvekar, Mrugank
Kondewar, Pranay
author_sort Mahajan, Neetin P
collection PubMed
description INTRODUCTION: Hereditary multiple exostosis (HME) is an autosomal dominant disorder affecting the skeletal system, which is characterized by multiple osteochondromas in bones arising from osteochondral ossification and leading to skeletal deformities, short stature, soft tissue, and neurovascular compressive symptoms. CASE REPORT: A 10-year-old female a case of HME presented with painless multiple swelling around knees, wrist, and painful varus deformity in the lower third of the right leg. The large exostosis of the right distal tibia was symptomatic and indenting the fibula which required excision along with the segment of the fibula of about 2.5 cm above the syndesmosis adjacent to the exostosis as the mass was adherent to the fibula. CONCLUSION: Although distal tibia osteochondromas are rare, they can lead to deformity of the ankle and loss of function if not managed early and properly. Hence, early detection, proper planning, and management of periarticular distal tibia osteochondromas are essential to prevent the development or progression of the deformity. Segmental fibulectomy is required to remove the adherent osteochondromas completely and to prevent the recurrence and secondary surgical procedures. It is very essential to follow up the patient till the skeletal maturity to identify the delayed presentation and late progression of the ankle deformities.
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spelling pubmed-78856602021-02-22 Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report Mahajan, Neetin P Wadia, Farokh S, Prasanna Kumar. G Yadav, Amit Kumar Narvekar, Mrugank Kondewar, Pranay J Orthop Case Rep Case Report INTRODUCTION: Hereditary multiple exostosis (HME) is an autosomal dominant disorder affecting the skeletal system, which is characterized by multiple osteochondromas in bones arising from osteochondral ossification and leading to skeletal deformities, short stature, soft tissue, and neurovascular compressive symptoms. CASE REPORT: A 10-year-old female a case of HME presented with painless multiple swelling around knees, wrist, and painful varus deformity in the lower third of the right leg. The large exostosis of the right distal tibia was symptomatic and indenting the fibula which required excision along with the segment of the fibula of about 2.5 cm above the syndesmosis adjacent to the exostosis as the mass was adherent to the fibula. CONCLUSION: Although distal tibia osteochondromas are rare, they can lead to deformity of the ankle and loss of function if not managed early and properly. Hence, early detection, proper planning, and management of periarticular distal tibia osteochondromas are essential to prevent the development or progression of the deformity. Segmental fibulectomy is required to remove the adherent osteochondromas completely and to prevent the recurrence and secondary surgical procedures. It is very essential to follow up the patient till the skeletal maturity to identify the delayed presentation and late progression of the ankle deformities. Indian Orthopaedic Research Group 2020-07 /pmc/articles/PMC7885660/ /pubmed/33623755 http://dx.doi.org/10.13107/jocr.2020.v10.i04.1780 Text en Copyright: © Indian Orthopaedic Research Group http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Mahajan, Neetin P
Wadia, Farokh
S, Prasanna Kumar. G
Yadav, Amit Kumar
Narvekar, Mrugank
Kondewar, Pranay
Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title_full Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title_fullStr Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title_full_unstemmed Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title_short Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis – A Rare Case Report
title_sort segmental fibulectomy to excise the adherent distal tibia osteochondroma in a case of hereditary multiple exostosis – a rare case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885660/
https://www.ncbi.nlm.nih.gov/pubmed/33623755
http://dx.doi.org/10.13107/jocr.2020.v10.i04.1780
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