Craniofaciossinostose sindrômica (CS) é uma desordem congênita associada às mutações dos genes FGFR1, FGFR2 e FGFR3. Ocasiona uma fusão prematura das suturas craniofaciais, levando à deficiência no crescimento do crânio e terço médio da face. A severidade dos sintomas relacionados à hipertensão intracraniana, aparelho respiratório (síndrome da hipopneia-apneia do sono) e ocular (órbitas rasas, proptose ocular, exorbitismo e falta de proteção do globo ocular), são parâmetros para indicar o procedimento cirúrgico. A tomografia-3D é uma opção atual e pouco utilizada para avaliar os volumes orbitais e a magnitude dos avanços craniofaciais e seus vetores; esse método permite melhor definição das estruturas anatômicas sem distorção das imagens, promovendo uma avaliação adequada dos resultados. Os objetivos do presente estudo foram avaliar os volumes orbitais, os avanços craniofaciais e seus vetores nas osteotomias Le Fort III e Monobloco com uso de aparelhos distratores, a correlação entre os avanços craniofaciais e a variação dos volumes orbitais e determinar a eficácia dos avanços craniofaciais por meio da comparação com índices normais de volumes orbitais. No período de janeiro de 2001 a dezembro de 2012, foram selecionados 20 pacientes submetidos ao alongamento ósseo gradual do terço médio da face, divididos em dois grupos. Grupo LF (n = 9), submetidos à osteotomia Le Fort III e Grupo MB (n = 11), submetidos à osteotomia frontofacial monobloco. A avaliação constou da revisão de prontuários e exames tomográficos, em que foram mensurados o volume orbital pela segmentação de imagens no programa InVesalius e modelagem 3D no programa Magics, e a magnitude dos avanços craniofaciais, e seus vetores, foram medidos no programa Rhinoceros. Os resultados foram submetidos à análise estatística: teste t- Student e ANOVA. No Grupo MB, houve aumento de 8,94 mm3 e 9,84 mm3 para a variação do volume orbital, nas órbitas direita e esquerda, respectivamente; no Grupo LF: 5,70 mm3 e 5,77 mm3, respectivamente, para esses parâmetros. A resultante do avanço médio, para a órbita direita e esquerda no Grupo LF foi 11,36 ± 3,80 mm e 11,11 ± 3,45 mm, respectivamente; já no Grupo MB, foi 14,22 ± 4,12 mm e 14,48 ± 4,49 mm, respectivamente. A correlação entre a resultante do avanço e a variação dos volumes orbitais foi significante no Grupo LF e para a órbita esquerda no Grupo MB. Em conclusão, o procedimento cirúrgico foi eficaz, tanto para o Grupo LF como para o Grupo MB, pois, houve aumento estatisticamente significativo do volume orbital no período pós-operatório; não houve diferença estatisticamente significativa, na avaliação da simetria, entre os volumes orbitais pós-operatórios, direito e esquerdo. Ainda, não houve diferença estatisticamente significante entre os volumes orbitais pósoperatórios quando comparados aos índices de normalidade / Syndromic craniofacial synostosis (CS) is a congenital disorder, which is most often, an autosomal dominant mutation associated to FGFR1, FGFR2 and FGFR3 genes. It causes a premature fusion of craniofacial sutures, leading to deficiency in skull growth and middle-third of the face. The severity of the intracranial hypertension related, respiratory syndrome (hypopneaapnea) and eye (shallow orbits, ocular proptosis, exorbitism and lack of protection of the eyeball) symptoms, are parameters that indicate the surgical procedure. Distraction osteogenesis of the middle-third of the face is currently the chosen technique for the treatment of patients with Craniofacial Synostosis syndrome candidates for the advancement of the middle-third of the face. The 3D-tomography is a current option and seldom used for this purpose; this method allows better definition of anatomical structures without image distortion, providing a more accurate assessment of the results. The objectives of the present study, were to assess quantitatively the craniofacial advancement and its vectors through 3D tomographic images, the osteotomy Le Fort III and Monobloc with use of distractor devices; Also, to evaluate the correlation between the craniofacial advances and the variation of orbital volumes and determine the effectiveness of craniofacial advances through the comparison with normal levels of orbital volumes. From January 2001 to December 2012, 20 patients underwent distraction osteogenesis of the middle-third of the face, divided into two groups. LF group (n = 9) underwent a Le Fort III osteotomy and MB group (n = 11) underwent a monobloc frontalfacial osteotomy. The evaluation consisted of the reviewing of the records and tomographic exams; measured is the orbital volume by image segmentation on the InVesalius software and 3D modeling on Magics software and the magnitude of facial advances, and their vectors, were measured on Rhinoceros software. The results were subject to statistical analysis: t-Student test and ANOVA. In the MB group, there was an increase of 8.94 mm3 and 9.84 mm3 for the variation of the orbital volume, the right and left orbits, respectively; in Group LF: 5.70 mm3 and 5.77 mm3 for these parameters. The resulting of average advance, for right and left orbit in Group LF was of 11.36 ± 3.80 mm, 11.11 ± 3.45mm, respectively; as for group MB it was 14.22 ± 4.12 mm and 14.48 ± 4.49 mm, respectively. Correlation between the results from the progress and the variation of orbital volume was significant in Group LF and left orbit in MB Group. In conclusion, the surgical procedure was effective for both the LF group as for the MB Group because: there was a statistically significant increase in orbital volume on the postoperative period; there was no statistically significant difference in the evaluation of symmetry between the postoperative period left and right orbital volumes. Still, there was no statistically significant difference between the postoperative orbital volumes when compared to normal ranges

Craniofaciossinostose sindrômica (CS) é uma desordem congênita associada às mutações dos genes FGFR1, FGFR2 e FGFR3. Ocasiona uma fusão prematura das suturas craniofaciais, levando à deficiência no crescimento do crânio e terço médio da face. A severidade dos sintomas relacionados à hipertensão intracraniana, aparelho respiratório (síndrome da hipopneia-apneia do sono) e ocular (órbitas rasas, proptose ocular, exorbitismo e falta de proteção do globo ocular), são parâmetros para indicar o procedimento cirúrgico. A tomografia-3D é uma opção atual e pouco utilizada para avaliar os volumes orbitais e a magnitude dos avanços craniofaciais e seus vetores; esse método permite melhor definição das estruturas anatômicas sem distorção das imagens, promovendo uma avaliação adequada dos resultados. Os objetivos do presente estudo foram avaliar os volumes orbitais, os avanços craniofaciais e seus vetores nas osteotomias Le Fort III e Monobloco com uso de aparelhos distratores, a correlação entre os avanços craniofaciais e a variação dos volumes orbitais e determinar a eficácia dos avanços craniofaciais por meio da comparação com índices normais de volumes orbitais. No período de janeiro de 2001 a dezembro de 2012, foram selecionados 20 pacientes submetidos ao alongamento ósseo gradual do terço médio da face, divididos em dois grupos. Grupo LF (n = 9), submetidos à osteotomia Le Fort III e Grupo MB (n = 11), submetidos à osteotomia frontofacial monobloco. A avaliação constou da revisão de prontuários e exames tomográficos, em que foram mensurados o volume orbital pela segmentação de imagens no programa InVesalius e modelagem 3D no programa Magics, e a magnitude dos avanços craniofaciais, e seus vetores, foram medidos no programa Rhinoceros. Os resultados foram submetidos à análise estatística: teste t- Student e ANOVA. No Grupo MB, houve aumento de 8,94 mm3 e 9,84 mm3 para a variação do volume orbital, nas órbitas direita e esquerda, respectivamente; no Grupo LF: 5,70 mm3 e 5,77 mm3, respectivamente, para esses parâmetros. A resultante do avanço médio, para a órbita direita e esquerda no Grupo LF foi 11,36 ± 3,80 mm e 11,11 ± 3,45 mm, respectivamente; já no Grupo MB, foi 14,22 ± 4,12 mm e 14,48 ± 4,49 mm, respectivamente. A correlação entre a resultante do avanço e a variação dos volumes orbitais foi significante no Grupo LF e para a órbita esquerda no Grupo MB. Em conclusão, o procedimento cirúrgico foi eficaz, tanto para o Grupo LF como para o Grupo MB, pois, houve aumento estatisticamente significativo do volume orbital no período pós-operatório; não houve diferença estatisticamente significativa, na avaliação da simetria, entre os volumes orbitais pós-operatórios, direito e esquerdo. Ainda, não houve diferença estatisticamente significante entre os volumes orbitais pósoperatórios quando comparados aos índices de normalidade / Syndromic craniofacial synostosis (CS) is a congenital disorder, which is most often, an autosomal dominant mutation associated to FGFR1, FGFR2 and FGFR3 genes. It causes a premature fusion of craniofacial sutures, leading to deficiency in skull growth and middle-third of the face. The severity of the intracranial hypertension related, respiratory syndrome (hypopneaapnea) and eye (shallow orbits, ocular proptosis, exorbitism and lack of protection of the eyeball) symptoms, are parameters that indicate the surgical procedure. Distraction osteogenesis of the middle-third of the face is currently the chosen technique for the treatment of patients with Craniofacial Synostosis syndrome candidates for the advancement of the middle-third of the face. The 3D-tomography is a current option and seldom used for this purpose; this method allows better definition of anatomical structures without image distortion, providing a more accurate assessment of the results. The objectives of the present study, were to assess quantitatively the craniofacial advancement and its vectors through 3D tomographic images, the osteotomy Le Fort III and Monobloc with use of distractor devices; Also, to evaluate the correlation between the craniofacial advances and the variation of orbital volumes and determine the effectiveness of craniofacial advances through the comparison with normal levels of orbital volumes. From January 2001 to December 2012, 20 patients underwent distraction osteogenesis of the middle-third of the face, divided into two groups. LF group (n = 9) underwent a Le Fort III osteotomy and MB group (n = 11) underwent a monobloc frontalfacial osteotomy. The evaluation consisted of the reviewing of the records and tomographic exams; measured is the orbital volume by image segmentation on the InVesalius software and 3D modeling on Magics software and the magnitude of facial advances, and their vectors, were measured on Rhinoceros software. The results were subject to statistical analysis: t-Student test and ANOVA. In the MB group, there was an increase of 8.94 mm3 and 9.84 mm3 for the variation of the orbital volume, the right and left orbits, respectively; in Group LF: 5.70 mm3 and 5.77 mm3 for these parameters. The resulting of average advance, for right and left orbit in Group LF was of 11.36 ± 3.80 mm, 11.11 ± 3.45mm, respectively; as for group MB it was 14.22 ± 4.12 mm and 14.48 ± 4.49 mm, respectively. Correlation between the results from the progress and the variation of orbital volume was significant in Group LF and left orbit in MB Group. In conclusion, the surgical procedure was effective for both the LF group as for the MB Group because: there was a statistically significant increase in orbital volume on the postoperative period; there was no statistically significant difference in the evaluation of symmetry between the postoperative period left and right orbital volumes. Still, there was no statistically significant difference between the postoperative orbital volumes when compared to normal ranges

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Historique & Objectifs: Les effets de l’avancement maxillaire (AM) sur l’anatomie velopharyngée ont déjà été étudiés en utilisant la céphalométrie. Cette modalité ne permet toutefois pas de bien caractériser les tissus mous. Le but de cette étude est de comparer la configuration de l’espace vélopharyngé en pré- et post-opératoire, telle que mesurée par tomodensitométrie (TDM). De plus, notre objectif est d’analyser et de comparer les différences dans ces mesures chez les patients avec et sans fente palatine (FP). Méthodologie: Ceci est une étude rétrospective portant sur 44 patients avec et sans FP, traités avec AM pour une hypoplasie maxillaire et une malocclusion dento-squelettique. Les TDM pré- et post-opératoires ont été comparés en se basant sur des repères préétablis. Des distances linéaires, des aires de sections transversales et des mesures volumétriques ont été mesurées en utilisant des reconstructions tridimensionnelles des TDM. Résultats: Pour les distances linéaires mesurées, une différence statistiquement significative a été notée pour les mesures linéaires du nasopharynx et du palais mou (25.1 vs 28.5 mm p=0.001 et 6.5 vs 7.6 mm p=0.026, respectivement). Les aires des sections transversales au niveau du nasopharynx et du palais mou ainsi que l'évaluation volumétrique de l'espace vélopharyngé n'ont pas démontrées une différence statistiquement significative en comparant les mesures en pré- et post-opératoire (p>0,05). En comparant les patients avec et sans FP, une différence statistiquement significative n’a été notée que pour la distance linéaire et l’aire de la section transversale du nasopharynx (p=0.045 et p=0.04, respectivement). Un antécédent de réparation de FP n’était pas prédictif de différences de mesures pré- et post-opératoire. Conclusion: Nos résultats confirment que, bien que certaines modifications structurelles de l’espace vélopharyngé soient inhérentes à l’AM chez les patients avec FP, leurs aires et volumes ne semblent pas changer de façon significative. Ces changements sont indépendants d’une histoire de FP réparée. / Background & Purpose: The effects of maxillary advancement (MA) on velopharyngeal anatomy have primarily been studied using lateral cephalometric radiographs. However, with recent advances in orthognathic surgery, there is an increased need for more detailed and precise imaging such as computerized tomographic (CT) scan reconstructions, to help in surgical planning and to measure outcomes. The purpose of this study is to compare the pre-and post-operative velopharyngeal space configuration modifications as measured on CT scans. The aim is also to assess differences in these airway measures between patients with and without history of prior repaired cleft palate (CP). Methods: This is a retrospective cohort study of 44 patients with and without CP who were treated with MA for midface hypoplasia and secondary malocclusion at skeletal maturity. The pre-and post-operative CT scans were compared with respect to pre-established landmarks. Linear distances, cross-sectional areas, and volumes were measured using 3-dimensional (3D) CT scan reconstructions. Results: For the linear distances measured, a statistically significant difference was found when comparing the pre-and post-operative measures of the narrowest part of the nasopharynx and the narrowest part of the retropalatal airway space (25.1 vs 28.5 mm p=0.001 and 6.5 vs 7.6 mm p=0.026, respectively). Retropalatal cross-sectional areas, nasopharyngeal cross-sectional areas and the volumetric assessment of the nasopharyngeal space showed no statistically significant differences when comparing pre-and post-operative scans (p>0.05). The main effect of palatal repair (CP vs. Non-CP) showed that there was only a statistically significant difference for the measures of the narrowest part of the nasopharynx and the nasopharyngeal cross-sectional area (p=0.045 and p=0.04, respectively). Mean changes in the measures did not differ over time (pre-and post-op) depending on whether there was prior history of CP repair. Conclusion: Our results support the hypothesis that although structural modifications of the pharyngeal space are inherent to MA in patients with CP, its surface area and volume do not change significantly. These changes are also independent of history of previous CP repair.