Submitted by PPG Odontologia (odontologia-pg@pucrs.br) on 2017-08-29T19:45:31Z No. of bitstreams: 1 FABIANE_AZEREDO_TES.pdf: 3598342 bytes, checksum: 84ac81263b072eecf7c0a9a8c7903bb2 (MD5) / Approved for entry into archive by Caroline Xavier (caroline.xavier@pucrs.br) on 2017-09-11T14:29:24Z (GMT) No. of bitstreams: 1 FABIANE_AZEREDO_TES.pdf: 3598342 bytes, checksum: 84ac81263b072eecf7c0a9a8c7903bb2 (MD5) / Made available in DSpace on 2017-09-11T14:31:55Z (GMT). No. of bitstreams: 1 FABIANE_AZEREDO_TES.pdf: 3598342 bytes, checksum: 84ac81263b072eecf7c0a9a8c7903bb2 (MD5) Previous issue date: 2017-07-11 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / The objectives of this study were to evaluate two types of rare craniofacial and dento-skeletal anomalies: hemifacial microsomia (HFM) and primary of failure eruption (PFE) of the permanent teeth. A literature review addressed to the three-dimensional evaluation methods of mandibular asymmetries in subjects with HFM, using conventional computed tomography (CT) and cone beam (CBCT) was conducted (Paper 1). CBCT data of 14 individuals with HFM and 35 subjects without HFM were selected and mandibular dimensions were evaluated and compared. Cephalometric landmarks were identified in the multiplanar images and panoramic reconstructions, and linear and angular measurements were performed for comparison between the right and left sides or affected and nonaffected sides of the mandible. InVivo Dental Software (Anatomage, San Jose, CA) was used in this evaluation. All variables showed a significant difference between the affected and non-affected side of the mandible in the HFM group, except the mandibular length, that presented similar dimensions on both sides. In the comparison between HFM and control groups, the angular measurements were significantly higher on the affected side and the mandibular ramus length on the non-affected side of HFM individuals was higher than in the control group. This type of growth pattern may be associated with bone compensations in the mandible of HFM individuals (Paper 2). In the study about rare dento-skeletal malformations, different approaches for the orthodontic treatment in adults and children with PFE were presented (Paper 3). Depending on the condition severity, the impact on the functional and aesthetic aspects in affected individuals can be significant and difficult to treat. / O objetivo deste trabalho foi estudar dois tipos de anomalias craniofacias e altera??es dento-esqueletais raras: microssomia hemifacial (MHF) e falhas de erup??o prim?ria (FEP) dos dentes permanentes. Foi realizada uma revis?o de literatura, que abordou os m?todos de avalia??o tridimensional de assimetrias mandibulares em indiv?duos com MHF, por meio de tomografia computadorizada convencional e cone beam (TCCB) (artigo 1). Exames de TCCB de 14 indiv?duos com MHF e de 35 sem MHF foram selecionadas e as dimens?es mandibulares foram avaliadas e comparadas. Pontos cefalom?tricos foram localizados nas imagens multiplanares e reconstru??es panor?micas, sobre as quais medidas lineares e angulares foram realizadas para compara??o entre os lados direito e esquerdo ou lados afetado ou n?o da mand?bula. O software InVivo Dental (Anatomage, San Jose, CA) foi utilizado para esta avalia??o. Todas as vari?ves apresentaram diferen?a significativa entre o lado afetado e n?o afetado da mand?bula no grupo MFH, exceto o comprimento mandibular, que apresentou dimens?es semelhantes em ambos os lados. Na compara??o entre os grupos MHF e controle, as medidas angulares foram significativamente maiores no lado afetado e o comprimento do ramo mandibular do lado n?o afetado pela MHF foi maior do que no grupo controle. Este tipo de padr?o de crescimento pode estar associado a compensa??es ?sseas na mand?bula de indiv?duos com MHF (artigo 2). No estudo que abordou altera??es dento-esqueletais raras, foram apresentadas diferentes abordagens para tratamento ortod?ntico das FEP em adultos e crian?as (artigo 3). Deformidades faciais e dento-esqueletais raras podem promover altera??es anat?micas complexas em diferentes estruturas craniofaciais. Dependendo da severidade da condi??o, o impacto nos aspectos funcionais e est?ticos dos indiv?duos acometidos pode ser significativo e de dif?cil tratamento.

Syftet med denna studie var att utvärdera behandling med hjälp av elektropalatografi (EPG) och portabel träningsenhet (PTU) hos en person med kvarstående artikulationsproblem. Försökspersonen var en vuxen man med medfödd hemifacial mikrosomi och mikrognati som tidigare genomfört konventionell talträning. En kvasiexperimentell, prospektiv, kontrollerad single subjekt A-B-A design användes. Försökspersonen hade vid träningsstart en tillbakadragen artikulation av /t/ och /d/ till velart/uvulart artikulationsställe med kvarstående svårighet att göra sitt tal förståeligt för sina samtalspartners.  Behandlingseffekten utvärderades genom instrumentell EPG analys av /t/ i enstaka ord före och efter behandling. Analysen visade signifikanta behandlingsresultat där en fortgående förbättring av produktionen av /t/ i medial och final position skedde även fyra år efter avslutad behandling. Perceptuell lyssnarbedömning av tränade och otränade lyssnare uppvisade signifikant förbättrad skattning av t-likhet av målljudet /t/ i medial och final position över tid. Ett mycket svagt samband sågs mellan perceptuell skattning av t-likhet och de analyserade EPG-värdena. Lyssnarskattningen av spontantalet visade en förbättring av förståelighet vid kontroll 4 år efter avslutad träning. Utvärderingen bekräftar tidigare forskning och visar att talträning med EPG är ett effektivt sätt att ge visuell återkoppling i försök att etablera ett korrekt artikulationsmönster, förbättrat tal och ökad förståelighet. Detta provades i föreliggande studie och även om resultaten inte var entydiga, antyddes att metoden kan vara långsiktigt framgångsrik vid sedan lång tid väl etablerade talavvikelser utan tecken på förändring spontant eller efter tidigare träning utan visuell återkoppling. En viktig erfarenhet är således att planera långsiktigt och invänta automatisering av nya artikulationsrörelser som har lång etableringsfas. Träning med EPG föreslås kunna erbjudas vuxna personer med kvarstående artikulationsavvikelser där konventionell träning inte har kunnat ge önskvärda resultat. Nyckelord: Elektropalatografi, behandlingseffekt, hemifacial mikrosomi, artikulationsstörning, långtidsuppföljning, förståelighet / The aim of this study was to evaluate speech therapy using electropalatography (EPG) and portable training unit (PTU) in a subject with persistent articulation errors. The participant was a male adult with congenital hemifacial microsomia and micrognathia who previously had received conventional speech therapy. The subject had a retracted, velar/ uvular articulation of dental plosives with difficulty making speech understandable to listeners. A quasi-experimental prospective, controlled single-subject ABA design was used. Treatment efficacy was assessed by instrumental EPG analysis of  /t/ in single words before and after treatment and showed significant treatment results with a continuing improvement in the production of  /t/ in medial and final position even four years after completion of treatment. Perceptual listen assessment of trained and untrained listeners showed significantly improved estimation of the target /t/ in medial and final position over time. A very weak association was found between the perceptual estimation of t-likeness and the analyzed EPG values. Listener rating of spontaneous speech showed an improvement in intelligibility 4 years after completing training. The evaluation confirms previous research showing that speech therapy with EPG seems effective for providing visual feedback in the attempt to establish a correct articulation pattern, improved speech and enhanced intelligibility. This was investigated in the present study and although the results were not unambiguous, it was indicated that the method can be successful at long persisting well established articulation errors without signs of spontaneous change or after previous training without visual feedback. An important experience is to plan long term and wait for the automation of the new articulation movements that have a long establishment phase. Training with EPG is proposed to be offered to adults with persistent articulation disorders for which conventional therapy has been unable to provide desirable results. Keywords:  Electropalatography, treatment effect, hemifacial microsomia, articulation disorder, long- term follow- up, intelligibility

Thesis deals with studying of morphology and variability of palatal shape in patients with hemifacial microsomia. It is congenital facial disorder, manifests in hypoplasia and asymmetry of facial structures. It interfere with part of splanchnocranium,especially manifests at hypoplasia of mandible and facial asymmetry, ears or orbits can be another affects part of the face. For notice morphology of palate was use virtual dental models of superior alveolar arch and palate of patients with HFM. Mean age of patients with HFM was 27,2 years (in range 20,8 - 41,5 years), mean age of control's file was 13,2 years (in range 13,0 - 13,9 years). Every subjects, also from patient's file and from control's file are mens with Czech's nationality. Models were evaluate by methods of geometrics morphometry (DCA, PCA, analysis asymmetry) and multidimensional statistic (Hotteling test, test of homogenity). Palatal shape of patients with HFM and controls is significantly different. Palate of patients with HFM was narrower and shorter in anterior part while wider in posterior lateral part, especially at the left side relative to control's file. We found out by evaluation asymmetry that palate of patients with HFM had the biggest asymmetry at back side of palate, by contrast, the biggest symmetry was at vertical middle line of...

On a group level, satisfaction with botulinum neurotoxin (BoNT) treatment in neurological indications is high. However, it is well known that a relevant amount of patients may not respond as expected. The aim of this study is to evaluate the BoNT treatment outcome on an individual level using a statistical single-case analysis as an adjunct to traditional group statistics. The course of the daily perceived severity of symptoms across a BoNT cycle was analyzed in 20 cervical dystonia (CD) and 15 hemifacial spasm (HFS) patients. A parametric single-case autoregressive integrated moving average (ARIMA) time series analysis was used to detect individual responsiveness to BoNT treatment. Overall, both CD and HFS patients significantly responded to BoNT treatment with a gradual worsening of symptom intensities towards BoNT reinjection. However, only 8/20 CD patients (40%) and 5/15 HFS patients (33.3%) displayed the expected U-shaped curve of BoNT efficacy across a single treatment cycle. CD (but not HFS) patients who followed the expected outcome course had longer BoNT injection intervals, showed a better match to objective symptom assessments, and were characterized by a stronger certainty to control their somatic symptoms (i.e., internal medical locus of control). In addition to standard evaluation procedures, patients should be identified who do not follow the mean course-of-treatment effect. Thus, the ARIMA single-case time series analysis seems to be an appropriate addition to clinical treatment studies in order to detect individual courses of subjective symptom intensities.

No description available.

La Microsomie Hémifaciale (MHF) est un spectre de maladie qui entraîne le développement hypoplasique unilatéral des structures faciales dérivées du premier et deuxième arc pharyngé. Bien que ce soit l'une des pathologies crâniofaciales congénitales des plus courantes, son algorithme de traitement idéal reste à débattre, principalement en raison de la nature complexe des patients et de leur grande variété phénotypique. Plus spécifiquement, la reconstruction de microtie représente un défi chirurgical chez les patients atteints de MHF, principalement dû à leur anatomie hypoplasique unilatérale. Malheureusement, notre revue de littérature a démontré que les études abordant cette question sont rare/de faible qualité, avec seulement 11 études fournissant 22 recommandations de grade C (n = 5) ou D (n = 17). Plus précisément, le positionnement approprié du pavillon de l’oreille reconstruite demeure une des tâches particulièrement difficiles. Étant donné que la transposition des mesures faciales du côté non affecté (comme cela est classiquement fait pour la reconstruction de la microtie) ne serait pas symétrique et que de futures chirurgies faciales correctives (qui peuvent modifier l’apparence faciale) sont généralement effectuées, la détermination de l'emplacement idéal du pavillon reconstruit n’est pas facile. Par conséquent, une nouvelle méthode de placement symétrique du pavillon reconstruit, basée sur le crâne plutôt que sur le visage, est proposée. Nous avons mené une étude rétrospective basée sur des mesures craniométriques provenant de tomodensitométries 3-D de 36 patients atteints de MHF, représentant 44 scans. Tout d'abord, l'intégrité de la voûte crânienne postérieure chez les patients atteints d'HFM a été prouvée (p > 0.001). En outre, les relations craniométriques entre le pavillon et l'endocrâne/la voûte crânienne postérieure étaient très précises chez les témoins sains (p > 0.001), et ont été validées pour tous les groupes d'âge (p > 0.001) dans notre cohorte MHF. Les "mesures L" proposées sont des mesures linéaires entre le pavillon sain et la voûte crânienne postérieure, qui peuvent être utilisées pour prédire avec précision l'emplacement idéal du pavillon reconstruit du côté malade. Celles-ci représentent un complément simple et peu coûteux dans la planification préopératoire de la reconstruction de microtie pour les patients atteints de MHF. / Hemifacial Microsomia (HFM) is a disease spectrum causing the unilateral hypoplastic development of facial structures derived from the first and second pharyngeal arches. Although it is one of the most common congenital craniofacial disorders, its optional treatment algorithm remains unclear, mainly due to the complex nature of these patients and the wide variety of phenotypes. More specifically, microtia reconstruction is a highly challenging surgery among HFM patients, given their unilateral hypoplastic anatomy. Unfortunately, our review of the literature demonstrated it is quite sparse/of low-quality regarding studies addressing such, with only 11 studies providing 22 main recommendations of either grade C (n = 5) or D (n = 17). More specifically, appropriate construct positioning is a particularly difficult task in microtia reconstruction for HFM patients. Given that transposing facial measurements from the unaffected side (as is classically done for microtia reconstruction) would not be symmetric and that future corrective skeletal surgeries (that may alter the facies) are usually performed, determining the construct’s ideal location is difficult. Therefore, a novel method for symmetric construct placement, based on the cranium rather than the face, was hypothesized. We conducted a retrospective, 3-D CT based craniometric study of 36 HFM patients accounting for 44 scans. First, the integrity of the posterior cranial vault among HFM patients was determined (p > 0.001). Further, craniometric relationships between the auricle and endocranium/the posterior cranium were highly precise in healthy controls (p > 0.001), and were proved to hold true across all age groups (p > 0.001) in our HFM cohort. The proposed “L measures” are linear measurements between the healthy auricle and the posterior cranium, that can be used to accurately predict ideal construct location on the diseased side. These represent a simple and costless adjunct in the pre-operative planning of microtia reconstruction for HFM patients.