Cargando…
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...
Autores principales: | , , , , |
---|---|
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 |
_version_ | 1783597698033647616 |
---|---|
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. |
format | Online Article Text |
id | pubmed-7574809 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Sultan Qaboos University Medical Journal, College of Medicine & Health Sciences |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT alhasanikhamism modifiedlefortiosteotomyandgenioplastyformanagementofseveredentofacialdeformityinbthalassaemiamajorcasereportandreviewoftheliterature AT bakathirabdulaziza modifiedlefortiosteotomyandgenioplastyformanagementofseveredentofacialdeformityinbthalassaemiamajorcasereportandreviewoftheliterature AT alhashmiahmedk modifiedlefortiosteotomyandgenioplastyformanagementofseveredentofacialdeformityinbthalassaemiamajorcasereportandreviewoftheliterature AT alrawahibadar modifiedlefortiosteotomyandgenioplastyformanagementofseveredentofacialdeformityinbthalassaemiamajorcasereportandreviewoftheliterature AT albakriabdullah modifiedlefortiosteotomyandgenioplastyformanagementofseveredentofacialdeformityinbthalassaemiamajorcasereportandreviewoftheliterature |