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Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature

β-thalassaemia major is an autosomal recessive form of haemoglobinopathy that is characterised by complete lack of production of the β-chains resulting in multiple complications that include severe anaemia, failure to thrive and skeletal abnormalities. Facial deformities induced by β-thalassaemia ma...

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Autores principales: Al Hasani, Khamis M., Bakathir, Abdulaziz A., Al-Hashmi, Ahmed K., Al Rawahi, Badar, Albakri, Abdullah
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sultan Qaboos University Medical Journal, College of Medicine & Health Sciences 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7574809/
https://www.ncbi.nlm.nih.gov/pubmed/33110654
http://dx.doi.org/10.18295/squmj.2020.20.03.018
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author Al Hasani, Khamis M.
Bakathir, Abdulaziz A.
Al-Hashmi, Ahmed K.
Al Rawahi, Badar
Albakri, Abdullah
author_facet Al Hasani, Khamis M.
Bakathir, Abdulaziz A.
Al-Hashmi, Ahmed K.
Al Rawahi, Badar
Albakri, Abdullah
author_sort Al Hasani, Khamis M.
collection PubMed
description β-thalassaemia major is an autosomal recessive form of haemoglobinopathy that is characterised by complete lack of production of the β-chains resulting in multiple complications that include severe anaemia, failure to thrive and skeletal abnormalities. Facial deformities induced by β-thalassaemia major are rare and are very challenging to treat from a surgical point of view. We report a 33-year-old female patient with β-thalassaemia major who presented to the Dental & Maxillofacial Surgery Department, Sultan Qaboos University Hospital, Muscat, Oman, in 2017 with gross dentofacial skeletal deformity contributing to her psychosocial issues. The facial deformity was corrected surgically by excision of the enlarged maxilla, modified Le Fort I osteotomy and advancement genioplasty. This case highlights the pre-operative preparation, surgical management, encountered complications and treatment outcome within 24 months of follow-up.
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spelling pubmed-75748092020-10-26 Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature Al Hasani, Khamis M. Bakathir, Abdulaziz A. Al-Hashmi, Ahmed K. Al Rawahi, Badar Albakri, Abdullah Sultan Qaboos Univ Med J Case Report β-thalassaemia major is an autosomal recessive form of haemoglobinopathy that is characterised by complete lack of production of the β-chains resulting in multiple complications that include severe anaemia, failure to thrive and skeletal abnormalities. Facial deformities induced by β-thalassaemia major are rare and are very challenging to treat from a surgical point of view. We report a 33-year-old female patient with β-thalassaemia major who presented to the Dental & Maxillofacial Surgery Department, Sultan Qaboos University Hospital, Muscat, Oman, in 2017 with gross dentofacial skeletal deformity contributing to her psychosocial issues. The facial deformity was corrected surgically by excision of the enlarged maxilla, modified Le Fort I osteotomy and advancement genioplasty. This case highlights the pre-operative preparation, surgical management, encountered complications and treatment outcome within 24 months of follow-up. Sultan Qaboos University Medical Journal, College of Medicine & Health Sciences 2020-08 2020-10-05 /pmc/articles/PMC7574809/ /pubmed/33110654 http://dx.doi.org/10.18295/squmj.2020.20.03.018 Text en © Copyright 2020, Sultan Qaboos University Medical Journal, All Rights Reserved This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License (http://creativecommons.org/licenses/by-nd/4.0/) .
spellingShingle Case Report
Al Hasani, Khamis M.
Bakathir, Abdulaziz A.
Al-Hashmi, Ahmed K.
Al Rawahi, Badar
Albakri, Abdullah
Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title_full Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title_fullStr Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title_full_unstemmed Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title_short Modified Le Fort I Osteotomy and Genioplasty for Management of Severe Dentofacial Deformity in β-Thalassaemia Major: Case report and review of the literature
title_sort modified le fort i osteotomy and genioplasty for management of severe dentofacial deformity in β-thalassaemia major: case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7574809/
https://www.ncbi.nlm.nih.gov/pubmed/33110654
http://dx.doi.org/10.18295/squmj.2020.20.03.018
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