TY - JOUR
T1 - Modified le fort i osteotomy and genioplasty for management of severe dentofacial deformity in β-thalassaemia major case report and review of the literature
AU - Al Hasani, Khamis M.
AU - Bakathir, Abdulaziz A.
AU - Al-Hashmi, Ahmed K.
AU - Al Rawahi, Badar
AU - Albakri, Abdullah
N1 - Publisher Copyright:
© 2020, Sultan Qaboos University. All rights reserved.
PY - 2020/8
Y1 - 2020/8
N2 - 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.
AB - 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.
KW - Beta-Thalassaemia
KW - Case Report
KW - Cooley's Anemia
KW - Dentofacial Deformities
KW - Genioplasty
KW - Le Fort Osteotomy
KW - Oman
KW - Thalassaemia Major
UR - http://www.scopus.com/inward/record.url?scp=85092050050&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85092050050&partnerID=8YFLogxK
U2 - 10.18295/squmj.2020.20.03.018
DO - 10.18295/squmj.2020.20.03.018
M3 - Article
C2 - 33110654
AN - SCOPUS:85092050050
SN - 2075-051X
VL - 20
SP - e362-e367
JO - Sultan Qaboos University Medical Journal
JF - Sultan Qaboos University Medical Journal
IS - 3
ER -