A multidisciplinary approach to the functional and esthetic rehabilitation of amelogenesis imperfecta and open bite deformity: a case report

The treatment of amelogenesis imperfecta (AI) with an anterior open bite (AOB) is a challenge for the clinician and often requires a multidisciplinary team of specialists. Most often, patients suffering from these conditions are young and a good functional and esthetic long-term result must be aspired. This clinical report illustrates the orthodontic, maxillofacial, restorative, and prosthodontic rehabilitation of a 20-year-old woman with a hypoplastic form of AI and an AOB malocclusion, having received treatment for the last 6 years. It included adhesive resin composite restorations, orthodontical and maxillofacial surgery with a one-piece Le Fort I osteotomy, and a genioplasty. Subsequent prosthodontic therapy consisted of 28 all-ceramic crowns whereby a solid interdigitation, a canine guidance, and consistent and regular contacts between tooth crowns could be achieved to assure a good functional and esthetic oral situation. The tooth preparation techniques guaranteed minimally invasive treatment. The patient was affected very positively. CLINICAL SIGNIFICANCE: This article describes an interdisciplinary approach to the successful treatment of a patient with a hypoplastic form of amelogenesis imperfecta over a period of 6 years. It starts with a discussion of the conservative steps taken during adolescence and concludes with the final prosthetic rehabilitation with all-ceramic crowns after reaching adulthood.

Coronal plane segmental flexibility in thoracic adolescent idiopathic scoliosis assessed by fulcrum-bending radiographs

Knowledge about segmental flexibility in adolescent idiopathic scoliosis is crucial for a better biomechanical understanding, particularly for the development of fusionless, growth-guiding techniques. Currently, there is lack of data in this field. The objective of this study was, therefore, to compute segmental flexibility indices (standing angle minus corrected angle/standing angle). We compared segmental disc angles in 76 preoperative sets of standing and fulcrum-bending radiographs of thoracic curves (paired, two-tailed t tests, p < 0.05). The mean standing Cobb angle was 59.7 degrees (range 41.3 degrees -95 degrees ) and the flexibility index of the curve was 48.6\% (range 16.6-78.8\%). The disc angles showed symmetric periapical distribution with significant decrease (all p values <0.0001) for every cephalad (+) and caudad (-) level change. The periapical levels +1 and -1 wedged at 8.3 degrees and 8.7 degrees (range 3.5 degrees -14.8 degrees ), respectively. All angles were significantly smaller on the-bending views (p values <0.0001). We noted mean periapical flexibility indices of 46\% (+1), 49\% (-1), 57\% (+2) and 81\% (-2), which were significantly less (p < 0.001) than for the group of remote levels 105\% (+3), 149\% (-3), 231\% (+4) and 300\% (-4). The discal and bony wedging was 60 and 40\%, respectively, and mean values 35 degrees and 24 degrees (p < 0.0001). Their relationship with the Cobb angle showed a moderate correlation (r = 0.56 and 0.45). Functional, radiographic analysis of idiopathic thoracic scoliosis revealed significant, homogenous segmental tethering confined to four periapical levels. Future research will aim at in vivo segmental measurements in three planes under defined load to provide in-depth data for novel therapeutic strategies.

Prevalence of cam-type deformity on hip magnetic resonance imaging in young males: a cross-sectional study

Objective To determine the prevalence of cam-type deformities on hip magnetic resonance imaging (MRI) in young males. Methods This was a population-based cross-sectional study in young asymptomatic male individuals who underwent clinical examination and completed a self-report questionnaire. A random sample of participants was invited for MRI of the hip. We graded the maximal offset at the femoral head–neck junction on radial sequences using grades from 0 to 3, where 0 = normal, 1 = possible, 2 = definite, and 3 = severe deformity. The prespecified main analyses were based on definite cam-type deformity grades 2 or 3. We estimated the prevalence of the cam-type deformity adjusted for the sampling process overall and according to the extent of internal rotation. Then we determined the location of the deformity on radial MRI sequences. Results A total of 1,080 subjects were included in the study and 244 asymptomatic males with a mean age of 19.9 years attended MRI. Sixty-seven definite cam-type deformities were detected. The adjusted overall prevalence was 24% (95% confidence interval [95% CI] 19–30%). The prevalence increased with decreasing internal rotation (P < 0.001 for trend). Among those with a clinically decreased internal rotation of <30°, the estimated prevalence was 48% (95% CI 37–59%). Sixty-one of 67 cam-type deformities were located in an anterosuperior position. Conclusion Cam-type deformities can be found on MRI in every fourth young asymptomatic male individual and in every second male with decreased internal rotation. The majority of deformities are located in an anterosuperior position.

The cam-type deformity of the proximal femur arises in childhood in response to vigorous sporting activity

The prevalence of a cam-type deformity in athletes and its association with vigorous sports activities during and after the growth period is unknown.

Botulinum Toxin Type A for the Treatment of Equinus Deformity in to Patients With Mucopolysaccharidosis Type II

Mucopolysaccharidoses are lysosomal storage disorders that are caused by a deficiency in the enzymes that degrade glycosaminoglycans. The accumulation of glycosaminoglycans affects multiple systems, resulting in coarse facial features, short stature, organomegaly, and variable neurological changes from normal intelligence to severe mental retardation and spasticity. Effects on the musculoskeletal system include dysostosis multiplex, joint stiffness, and muscle shortening. This article reports 2 patients with mucopolysaccharidosis type II (Hunter syndrome) who showed progressive equinus deformity of the feet. Both patients were treated with intramuscular botulinum toxin type A injections in the gastrocnemius and the soleus muscles, followed by serial casting. In both patients, passive range of motion, muscle tone, and gait performance were significantly improved. Botulinum toxin type A injections followed by serial casting are a therapeutic option for contractures in patients with mucopolysaccharidosis. However, the long-term effects and the effect of application in other muscles remain unknown.

The cam-type deformity - what is it: SCFE, osteophyte, or a new disease?

Cam-type deformity of the proximal femur is a risk factor for the development of cam-type femoroacetabular impingement and a prearthrotic condition of the hip. The etiology of cam-type deformity remains unclear. There are a number of causes of cam-type deformity including sequellae of slipped capital femoral epiphysis, Legg-Calvé-Perthes disease or Perthes-like deformities, postinfectious, and traumatic. However, the majority of cam-type deformities arise without any apparent preexisting hip disease. These "idiopathic" cam-type deformities likely represent a majority of cases, and show clear racial and sex differences, as well as developmental and genetic influences. Idiopathic cam-type deformity also seems to be a distinct entity from residual or silent slipped capital femoral epiphysis, as well as osteoarthritis-induced osteophytes. In this paper we examine the different pathogenetic aspects of the proximal femur that contribute to cam-type deformity and/or symptomatic cam-type femoroacetabular impingement.