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

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

[Stability of Le Fort I impaction osteotomies]. / Stabilité osseuse des ostéotomies de Le Fort I d'impaction.

INTRODUCTION: Long-term results of Le Fort I osteotomy with vertical impaction for maxillary vertical excess may be not stable. We had for aim to analyze postoperative maxillary and dental displacement after a Le Fort I vertical impaction osteotomy, to identify causes of relapse. PATIENTS AND METHODS: A clinical and radiological evaluation was made on postoperative occlusion (early and late). The position of three bone (O: lower orbit; P: greatest palatine convexity; T: lowest part of the mandibular foramen) and of three dental landmarks (I: occlusal edge of the upper incisor; i: occlusal edge of the lower incisor; m: first molar distal vestibular cuspid) was measured in a standardized method on pre-surgical, early and late (1 to 2.5 years) postoperative cephalometric X rays. Eighteen patients were operated by the same surgeon for maxillary anterior vertical excess and underwent Le Fort I impaction osteotomy, alone or associated with a mandibular osteotomy. Stability was defined by a postoperatory displacement smaller than 1mm. RESULTS: All patients had stable bone landmarks. Three patients had unstable dental landmarks due to relapse. DISCUSSION: Impaction maxillary osteotomy provides stable bone results for maxillary facial height excess. Unsatisfactory outcome is always due to postoperative dental and alveolar displacement. These results correlate to published data.

Resection of pyriform aperture: a useful adjunct in nasal surgery.

OBJECTIVE: The aim of this paper is to describe a novel technique of improving the nasal airflow in patients in whom, despite reduction surgery to the inferior turbinates (either alone or with septal surgery), the nasal airways remain compromised because of obstruction occurring anterior to the inferior turbinate, due to a prominent nasal process of the maxilla at the pyriform aperture. METHOD: We describe a novel approach to resection of the nasal process of the maxilla, a technique performed in 40 patients with either: residual obstructive symptoms following septal surgery with or without turbinate surgery; and significant inspiratory alar collapse. The same technique was also used in two patients to improve the intranasal approach to the medial and anterior maxilla during endoscopic medial maxillectomy for recurrent inverted papilloma. RESULTS: Patients reported a subjective improvement in their nasal airways, with resolution of inspiratory alar collapse. This technique significantly improved intranasal access in the two patients with inverted papilloma. CONCLUSION: This technique confers significant subjective improvement of nasal airways patency in selected patients, and improves endoscopic surgical access to clear inverted papilloma.