Spinal involvement in mucopolysaccharidosis IVA (Morquio-Brailsford or Morquio A syndrome): presentation, diagnosis and management.

Mucopolysaccharidosis IVA (MPS IVA), also known as Morquio-Brailsford or Morquio A syndrome, is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase (GALNS). MPS IVA is multisystemic but manifests primarily as a progressive skeletal dysplasia. Spinal involvement is a major cause of morbidity and mortality in MPS IVA. Early diagnosis and timely treatment of problems involving the spine are critical in preventing or arresting neurological deterioration and loss of function. This review details the spinal manifestations of MPS IVA and describes the tools used to diagnose and monitor spinal involvement. The relative utility of radiography, computed tomography (CT) and magnetic resonance imaging (MRI) for the evaluation of cervical spine instability, stenosis, and cord compression is discussed. Surgical interventions, anaesthetic considerations, and the use of neurophysiological monitoring during procedures performed under general anaesthesia are reviewed. Recommendations for regular radiological imaging and neurologic assessments are presented, and the need for a more standardized approach for evaluating and managing spinal involvement in MPS IVA is addressed

THE ROLES OF ORTHOPAEDIC PATHOLOGY AND GENETIC DETERMINANTS IN EQUINE CERVICAL STENOTIC MYELOPATHY

Cervical stenotic myelopathy (CSM) is an important musculoskeletal and neurologic disease of the horse. Clinical disease occurs due to malformations of the vertebrae in the neck causing stenosis of the cervical vertebral canal and subsequent spinal cord compression. The disease is multifactorial in nature, therefore a clearer understanding of the etiology and pathogenesis of CSM will allow for improved management and therapeutic practices. This thesis examines issues of equine CSM diagnosis, skeletal tissue pathology, and inherited genetic determinants utilizing advances in biomedical imaging technologies and equine genomics. Magnetic resonance imaging (MRI) data provided a more complete assessment of the cervical column through image acquisition in multiple planes. First, MRI was compared to standing cervical radiographs for detection of stenosis. Using canal area or the cord canal area ratio, MRI more accurately predicted sites of compression in CSM cases. Secondly, articular process skeletal pathology localized on MRI was found to be more frequent and severe in CSM horses compared to controls. In addition, lesions were generalized throughout the cervical column and not limited to the spinal cord compression sites. A subset of lesions identified on MRI was evaluated using micro-CT and histopathology. Osteochondrosis, osseous cyst-like structures, fibrous tissue replacement of bone, and osteosclerosis were observed. These lesions support likely developmental aberrations of vertebral bone and cartilage maturation with secondary biomechanical influences. Bone cyst-like structures are a novel finding in this disease. Finally, the long-standing question of the contribution of genetic determinants to CSM was investigated using a genome wide association study (GWAS). Multiple significant loci were identified supporting the influence of a complex genetic trait in clinical disease. A simple Mendelian trait controlled by one gene is unlikely given the detection of variants across multiple chromosomes. Major contributions from this research include documentation of articular process bone and cartilage pathology in horses with CSM, support for abnormal cervical vertebrae development being an important contributing factor in the etiology and/or pathogenesis of equine CSM, and evidence that multiple genetic loci contribute to the CSM disease phenotype

Artificial intelligence in dentistry, orthodontics and orthognathic surgery: A literature review

Artificial intelligence is the ability of machines to work like humans. The concept initially began with the advent of mathematical models which gave calculated outputs based on inputs fed into the system. This was later modified with the introduction of various algorithms which can either give output based on overall data analysis or by selection of information within previous data. It is steadily becoming a favoured mode of treatment due to its efficiency and ability to manage complex conditions in all specialities. In dentistry, artificial intelligence has also popularised over the past few decades. They have been found useful for diagnosis in restorative dentistry, oral pathology and oral surgery. In orthodontics, they have been utilised for diagnosis, assessment of treatment needs, cephalometrics, treatment planning and orthognathic surgeries etc. The current literature review was planned to highlight the uses of artificial intelligence in dentistry, specifically in orthodontics and orthognathic surgery

Cone Beam Computed Tomography in Orthodontics

Cone beam computed tomography (CBCT) is an important source of three‐dimensional volumetric data in clinical orthodontics. Due to the progress in the technology of CBCT, for orthodontic clinical diagnosis, treatment and follow‐up, CBCT supply much more reliable information compared to conventional radiography. The most justified indications for the use of CBCT in orthodontics are the existence of impacted and transposed teeth. For the management of the impacted teeth, CBCT enhances the ability to localize these teeth accurately and to assess root resorption of adjacent teeth. Patients with craniofacial anomalies like cleft palate cases, the abnormalities of the temporomandibular joint contributing malocclusion, evaluation of airway morphology in obstructive sleep apnea cases, patients needing maxillary expansion or planning orthognathic surgery in severe skeletal discrepancies are also listed among the indications of using CBCT in orthodontics. CBCT is useful in identifying optimal site location for temporary skeletal anchorage device. The use of CBCT for the assessment of treatment outcomes and evaluation of cervical vertebral maturation are still controversial issues. It should be kept in mind that before using CBCT, justification and evaluation of risks and benefits are needed. In order to minimize the radiation dose, the exam should include only the areas of interest

Evaluation of the Artificial Neural Network and Naive Bayes Models Trained with Vertebra Ratios for Growth and Development Determination

Objective: This study aimed to evaluate the success rates of the artificial neural network models (NNMs) and naive Bayes models (NBMs) trained with various cervical vertebra ratios in cephalometric radiographs for determining growth and development.Methods: Our retrospective study was performed on 360 individuals between the ages of 8 and 17 years, whose cephalometric radiographs were taken. According to the evaluation of cephalometric radiographs, growth and development periods were divided into 6 vertebral stages. Each stage was considered as a group, each group had 30 girls and 30 boys. Twenty-eight cervical vertebral ratios were obtained by using 10 horizontal and 13 vertical measurements. These 28 vertebral ratios were combined in 4 different combinations, leading to 4 different datasets. Each dataset was split into 2 parts as training and testing. To prevent the overfitting, a 5-cross fold validation technique was also used in the training phase. The experiments were conducted on 2 different train/test ratios as 80%-20% and 70%-30% for both NNMs and NBMs.Results: The highest determination success rate was obtained in NNM 3 (0.95) and the lowest in NBM 4 (0.50). The determination success of NBM 1 and NBM 3 was almost similar (0.60). The success of NNM 2 did not differ much from that of NNM 1 (0.94). The determination success of stage 5 was relatively lower than the others in NNM 1 and NNM 2 (0.83).Conclusion: The NNMs were more successful than the NBMs in our developed models. It is important to determine the effective ratio and/or measurements that will be useful for differentiation

Cone beam computed tomography evaluation of midpalatal suture maturation in a select Western Cape sample

Magister Scientiae Dentium - MSc(Dent)There is controversy in the literature regarding the ideal treatment timing for rapid maxillary expansion. The successful use of rapid maxillary expansion (RME) has largely been limited to young patients with chronological age being a determinant of the patency of the midpalatal suture. However, there is consensus in the literature that chronological age is not a valid indicator of skeletal age. Additionally, conventional radiology and histology has revealed that the midpalatal suture may be patent in young adults (<25 years of age), with successful RME shown in these patients

Reliability of Frontal Sinus Morphology with Cervical Vertebral Maturation for the Assessment of Skeletal Maturity

INTRODUCTION: Growth, an expository variable aids the orthodontist in precise diagnosis and treatment planning. Variations in developmental pattern led to the evolution of malocclusion and dentofacial deformities. Developmental status of a patient can be evaluated by several methods but most of them failed to give a reliable estimate of skeletal maturity. AIM OF THE STUDY: To evaluate the correlation of frontal sinus morphology with cervical vertebrae stages as a skeletal maturity indicator. MATERIALS AND METHODS: A total of 180 lateral cephalograms of subjects aged between 8 and 16 years were included in the study. Based on the cervical maturation stages, the subjects were divided into 6 groups with 1:1 male to female ratio. The frontal sinus index and the cervical stages were evaluated on the same radiograph. Frontal sinus index was compared with different cervical stages by Kruskal-Wallis test and frontal sinus index values between adjacent cervical stages were compared for each sex by post hoc Dunnett T3 test. The correlation between the cervical stages and the sinus index were assessed by Kendall tau-b values. RESULTS: There was a significant linear increase and a good statistical correlation of the frontal sinus height and width at each cervical vertebral maturation stages. The index of frontal sinus increases statistically with the cervical vertebral maturation but had a weak correlation with the maturation stage. There was no significant relationship and no significant correlation of frontal sinus height, width and index with gender. CONCLUSION: Frontal sinus morphology cannot be used as a reliable sole indicator for the appraisal of skeletal maturity in patients