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1.
J Craniofac Surg ; 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38949493

RESUMEN

BACKGROUND/OBJECTIVE: Alveolar bone graft (ABG) or repair has become a routine part of treatment protocols for individuals with cleft lip and/or palate. However, the necessity and potential benefits of presurgical orthodontic treatment in influencing the outcomes of secondary alveolar bone grafting remain inconclusive. This systematic review aimed to assess the impact of presurgical orthodontics on preparing patients for secondary alveolar bone grafts. STUDY DESIGN: The authors systematically searched for relevant articles in PubMed, Web of Science, and Embase databases spanning the period from January 1, 2000, to December 31, 2023, using keywords related to alveolar bone grafting and orthodontic treatment for patients with cleft lip and/or palate. The review encompassed various study designs, including prospective and retrospective studies, observational studies, cross-sectional studies, randomized and nonrandomized clinical trials, cohort studies, and case-control studies. The review adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and the risk of bias was evaluated in studies selected for full-text review. RESULTS: The search strategy identified 809 publications. After initial screening and application of exclusion criteria, 11 studies were included for final review. Three were prospective studies, 8 were retrospective studies, and 3 were cross-sectional cohort studies. On the basis of ROBINS-I and RoB 2 risk assessment, 9 studies were found to be of moderate risk of bias, one study was categorized as of high risk of bias, and 1 study was categorized as low risk of bias. CONCLUSIONS: Drawing from the latest and most reliable studies, 7 out of 11 included studies provided compelling evidence that presurgical orthodontics preceding alveolar bone grafting (ABG) leads to significantly improved outcomes compared with cases without presurgical orthodontic intervention. Notably, individuals with specific conditions, such as severely collapsed upper arch and mispositioned upper incisors, appear to derive the greatest benefits from presurgical orthodontic treatment. However, a call for additional studies characterized by high methodological quality and with longer follow-up periods is emphasized to enhance the safety considerations for both practitioners and patients concerning the utilization of presurgical orthodontics in the treatment of individuals with cleft lip and palate.

2.
Cleft Palate Craniofac J ; 61(5): 791-800, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-36748327

RESUMEN

OBJECTIVE: The purpose is to evaluate outcomes of alveolar bone grafting based on the pre-grafting orthodontic preparation methods. DESIGN: Retrospective analysis of individuals with unilateral cleft lip and palate. SUBJECTS AND SETTINGS: 28 individuals with non-syndromic UCLP from two craniofacial centers, 14 individuals each from XXXX and XXXX. INTERVENTIONS: The alignment group underwent maxillary expansion with incisors alignment while the non-alignment group underwent only maxillary expansion for presurgical orthodontic preparation. METHODS: Initial and post-surgical CBCT scans were compared to observe changes in angulation of the incisor adjacent to the cleft site, alveolar bony root coverage, and bone graft outcomes. RESULTS: In the alignment group, the buccolingual rotation decreased by 32.35 degrees (p = .0002), the anteroposterior inclination increased by 14.01 degrees (p = .0004), and the mesiodistal angulation decreased by 17.88 degrees (p = .0001). Alveolar bony coverage did not change after bone graft in both groups, and no difference was observed between the groups. Chelsea scale showed satisfactory bone graft outcome (category A, C) in 12 cases (85.71%) in the alignment group and 11 cases (78.51%) in the non-alignment group. The volumetric measurement showed the alignment group had better bone fill of 69.85% versus 51.45% in the non-alignment group (p = .0495). CONCLUSIONS: Alveolar bony coverage on the tooth adjacent to cleft sites did not change with alveolar bone grafting surgery in either of the alignment and non-alignment group. Presurgical orthodontic alignment does not induce root exposure nor poorer bone grafting outcome.


Asunto(s)
Injerto de Hueso Alveolar , Labio Leporino , Fisura del Paladar , Tomografía Computarizada de Haz Cónico Espiral , Humanos , Injerto de Hueso Alveolar/métodos , Labio Leporino/diagnóstico por imagen , Labio Leporino/cirugía , Fisura del Paladar/diagnóstico por imagen , Fisura del Paladar/cirugía , Estudios Retrospectivos
3.
Cleft Palate Craniofac J ; 60(6): 780-783, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-35354333

RESUMEN

BACKGROUND: California Senate Bill 630 (SB630) enacted statutorily mandated health plan coverage for orthodontic care of patients with cleft palate and craniofacial anomalies in 2009, which was effective from July 1, 2010. In this qualitative analysis, third-party compliance with SB630 in a university-based cleft and craniofacial orthodontic program is evaluated. METHODS: Privately insured patients that experienced a coverage delay or denial of orthodontic treatment for cleft lip and palate in the University of California, San Francisco Cleft and Craniofacial Orthodontic Program between July 1, 2010 and October 28, 2020 were identified. A thematic analysis of reasons for delay or denial was conducted. RESULTS: Nearly three quarters of patients experienced coverage delay and/or denials. The most common reason given was that services were not covered. CONCLUSIONS: Despite state-mandated coverage, inappropriate denials of orthodontic care for patients with cleft lip and palate by private insurers persist in California.


Asunto(s)
Labio Leporino , Fisura del Paladar , Seguro , Humanos , Fisura del Paladar/cirugía , Labio Leporino/cirugía , California
4.
Am J Orthod Dentofacial Orthop ; 161(2): e136-e146, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34565628

RESUMEN

INTRODUCTION: The study aimed to characterize the impact of the coronavirus disease 2019 (COVID-19) pandemic on predoctoral and postdoctoral orthodontic education. METHODS: Electronic surveys were distributed via e-mail to predoctoral orthodontic directors at accredited dental schools (n = 66), postgraduate orthodontic program directors at orthodontic programs (n = 73), and craniofacial orthodontic fellowship directors (n = 5) in the United States and Canada. RESULTS: The predoctoral orthodontic survey received a response rate of 38%, of the which 62% indicated significant curriculum modifications. However, orthodontic curriculum hours were largely unchanged because of the COVID-19 pandemic. Of the responding predoctoral orthodontic programs with graduate orthodontic programs in which dental students can gain clinical experience (n = 14), all but 2 reported not allowing or limiting observation or assisting opportunities. The postdoctoral orthodontic survey received a response rate of 39% and was affected by clinic modifications, including financial limitations, severe acute respiratory syndrome coronavirus 2 testing, decreased clinical hours, among other modifications. CONCLUSIONS: COVID-19 pandemic has had a measurable impact on orthodontic clinical education for the predoctoral and postdoctoral orthodontic programs evaluated. Overall, less patient care was delivered, potentially resulting in delayed treatment for patients and fewer training opportunities for learners, particularly for predoctoral dental students, whose clinical observation and assisting hours were limited.


Asunto(s)
COVID-19 , Ortodoncia , Prueba de COVID-19 , Curriculum , Humanos , Pandemias , SARS-CoV-2 , Facultades de Odontología , Encuestas y Cuestionarios , Estados Unidos/epidemiología
5.
Am J Orthod Dentofacial Orthop ; 162(5): 753-762, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37830535

RESUMEN

INTRODUCTION: It is important to achieve proper root position during orthodontic treatment involving future dental implant placement. However, current methods to evaluate root position are either inaccurate or expose patients to relatively high radiation levels. A new approach using an expected root position (ERP) setup has previously demonstrated the potential to accurately monitor root position with minimal radiation. This study aimed to evaluate whether the ERP setup is an accurate and reliable method to determine if the roots adjacent to an edentulous site are appropriate for the anticipated dental implant. METHODS: In this retrospective study, the ERP setup was generated for 22 edentulous sites selected from the University of California San Francisco Division of Orthodontics patient database. The mesiodistal angulation of all teeth adjacent to the edentulous sites and the mesiodistal space between the teeth were measured in the ERP setup and compared with the posttreatment cone-beam computed tomography (CBCT) scan, which served as the control. The intraoperator and interoperator reliability and agreement between the ERP setup and the posttreatment CBCT scan were assessed using Bland-Altman analysis. The correlation between measurements was further evaluated by the Pearson correlation coefficient. RESULTS: The Bland-Altman plots and the Pearson correlation coefficient displayed strong agreement between the ERP setup and the posttreatment CBCT scan, with only 11.4% mesiodistal angulation measurements beyond the clinically acceptable range of ± 2.5°. All mesiodistal angulations and distances were strongly correlated with high intraoperator and interoperator reliabilities. CONCLUSION: The method to generate an ERP set up to evaluate the mesiodistal angulation and space of an edentulous site prepared for a future dental implant has been demonstrated to be accurate and reliable.


Asunto(s)
Implantes Dentales , Raíz del Diente , Humanos , Raíz del Diente/diagnóstico por imagen , Imagenología Tridimensional/métodos , Estudios Retrospectivos , Reproducibilidad de los Resultados , Tomografía Computarizada de Haz Cónico/métodos
6.
Cleft Palate Craniofac J ; 57(1): 132-136, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31248274

RESUMEN

Floating-Harbor syndrome (FHS) is a rare genetic disorder caused by heterozygous mutations in the Snf2-related CREBBP activator protein (SRCAP) gene. The syndrome is characterized by proportional short stature, delayed bone maturation, delayed speech development, and facial dysmorphism. Submucous cleft palate and cleft lip have been reported in FHS, but to our knowledge orofacial clefting in this condition has not been assessed in detail. Here, we report on a case of bilateral cleft lip in a patient with FHS confirmed by exome sequencing.


Asunto(s)
Anomalías Múltiples , Labio Leporino , Fisura del Paladar , Anomalías Craneofaciales , Adenosina Trifosfatasas , Trastornos del Crecimiento , Defectos del Tabique Interventricular , Humanos
7.
Orthod Craniofac Res ; 22 Suppl 1: 149-153, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-31074131

RESUMEN

OBJECTIVES: To conduct a prospective pilot trial to test the clinical efficacy and accuracy of a newly developed Bluetooth-enabled retainer, which was synchronized with an iOS mobile application, cloud database and provider webpage. SETTING AND SAMPLE POPULATION: Five orthodontic residents in a university setting. MATERIAL AND METHODS: At the delivery of the retainers (T0), each participant was given an Bluetooth-enabled retainer, logbook and iPod Touch installed with the mobile application. Participants were instructed to wear the retainer for 12 hours per day and record in the logbook each time the retainer was inserted or removed and trained to synchronize the device daily to the mobile application. After the 5-day study period (T1), statistical analysis was performed comparing the device-reported data to the logbook data using two calculation methods. RESULTS: From T0 - T1, the participants wore their retainers for a median of 11.55 hours per day and the median difference between the self-reported (logbook) data and the device data was 35 minutes or 5.1% over the 5-day study period. Using an adjusted method to calculate the device-reported wear time, the median error was 13 minutes or 1.9%. CONCLUSION: Subjects were able to successfully wear the retainer and upload the data to the mobile application and cloud database. Patient compliance and technical issues could be monitored daily via the provider webpage, and early intervention was possible with reminder messaging. The Bluetooth-enabled retainer showed a clinically acceptable level of accuracy and usability that validates it for future clinical testing.


Asunto(s)
Retenedores Ortodóncicos , Cooperación del Paciente , Humanos , Diseño de Aparato Ortodóncico , Proyectos Piloto , Estudios Prospectivos , Resultado del Tratamiento
8.
J Craniofac Surg ; 30(7): 2094-2098, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31503128

RESUMEN

PURPOSE: The aim of this study was to measure the cortical bone thickness of the infrazygomatic crest area in individuals with unilateral cleft lip and palate using cone beam computed tomography for placement of miniplates used for bone anchored maxillary protraction. MATERIALS AND METHODS: CBCT scans were obtained from 31 non-syndromic UCLP children diagnosed with maxillary hypoplasia (17 males, 14 females, mean age: 11.9 years). 5 horizontal and 5 vertical reference planes were drawn at the infrazygomatic crest area. The cortical bone thickness at 25 intersection points on the cleft side and the non-cleft side was measured. RESULTS: The mean cortical bone thickness of the 25 measured points was 1.19 mm on the cleft side and 1.17 mm on the non-cleft side with no significant difference. The greatest cortical bone thickness was found to be at the most superior, posterior point (H+6, V+0), which was 1.49 mm on the cleft side and 1.47 mm on the non-cleft side. The thinnest mean cortical bone thickness was measured at the most inferior, anterior point (H-2, V-8), which was 0.94 mm on the cleft side and 0.95 mm on the non-cleft side. There was no significant difference between males and females.


Asunto(s)
Labio Leporino/diagnóstico por imagen , Fisura del Paladar/diagnóstico por imagen , Adolescente , Niño , Tomografía Computarizada de Haz Cónico/métodos , Hueso Cortical/diagnóstico por imagen , Femenino , Humanos , Imagenología Tridimensional , Masculino
9.
Am J Orthod Dentofacial Orthop ; 156(4): 566-573, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31582128

RESUMEN

INTRODUCTION: Accurate root position is imperative for successful orthodontic treatment that is stable and functional. Current methods to monitor root position are either inaccurate or use relatively high levels of radiation. A method to generate an expected root position (ERP) setup has been reported to have the potential to accurately evaluate root position with minimal radiation. The purpose of this study was to determine the accuracy and reliability of the clinical decisions made on root position using the ERP setup. METHODS: This retrospective study included 10 subjects who had pretreatment and midtreatment cone-beam computed tomography (CBCT) scans and study models. An ERP setup was generated for all patients at midtreatment. Four examiners assessed both the CBCT scan and ERP setup and made clinical decisions regarding the root position with each method. Cohen's kappa was determined to assess intraoperator and intermethod reliability. Sensitivity, specificity, positive predictive value, and negative predictive value were calculated to determine the accuracy of the ERP setup. RESULTS: The kappa values for intraoperator reliability for both the CBCT scan and ERP setup fell within the 0.61-0.80 range. The kappa values for intermethod reliability between the CBCT scan and ERP setup fell within the 0.61-0.80 range for all tooth groups. The sensitivity of the ERP setup ranged from 0.72 to 0.90, specificity ranged from 0.89 to 0.97, positive predictive value ranged from 0.57 to 0.85, and negative predictive value ranged from 0.93 to 0.99. CONCLUSIONS: This study demonstrated that the ERP setup, when compared with the gold standard CBCT scan, was accurate and reliable in making clinical decisions regarding root position at midtreatment.


Asunto(s)
Toma de Decisiones Clínicas , Tomografía Computarizada de Haz Cónico/métodos , Imagenología Tridimensional/métodos , Técnicas de Movimiento Dental , Raíz del Diente/anatomía & histología , Raíz del Diente/diagnóstico por imagen , Humanos , Modelos Dentales , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad
10.
Am J Orthod Dentofacial Orthop ; 154(4): 583-595, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30268268

RESUMEN

INTRODUCTION: Current methods to evaluate root position either are inaccurate (panoramic radiograph) or expose patients to relatively large amounts of radiation (cone-beam computed tomography [CBCT]). A method to evaluate root position by generating an expected root position (ERP) setup was recently reported but has not been validated. The purpose of this study was to quantitatively assess the accuracy and reliability of the ERP setup with adequate statistical power. METHODS: This retrospective study included 15 subjects who had completed phase 2 orthodontic treatment. An ERP setup was generated for all patients after treatment. The ERP setup was compared with the posttreatment CBCT scan, which served as the control. The mesiodistal angulation and buccolingual inclination of all teeth in both the ERP setup and the posttreatment CBCT scan were measured and compared. Bland-Altman analysis was used to assess interoperator reliability, intraoperator reliability, and agreement between the ERP setup and the posttreatment CBCT scan. RESULTS: Bland-Altman plots showed high interoperator and intraoperator reliabilities. These plots also showed strong agreement between the ERP setup and the posttreatment CBCT scan; 11.8% of teeth measured for mesiodistal angulation and 9.6% of teeth measured for buccolingual inclination were outside the ±2.5° range of clinical acceptability. CONCLUSIONS: We validated that the method to generate an ERP setup to evaluate root position for posttreatment orthodontic assessment is accurate and reliable.


Asunto(s)
Tomografía Computarizada de Haz Cónico/métodos , Imagenología Tridimensional/métodos , Ortodoncia Correctiva , Raíz del Diente/anatomía & histología , Raíz del Diente/diagnóstico por imagen , Diente/anatomía & histología , Diente/diagnóstico por imagen , Arco Dental/diagnóstico por imagen , Humanos , Procesamiento de Imagen Asistido por Computador/métodos , Modelos Lineales , Modelos Dentales , Proyectos Piloto , Radiografía Panorámica/métodos , Reproducibilidad de los Resultados , Estudios Retrospectivos , San Francisco , Programas Informáticos
11.
Am J Orthod Dentofacial Orthop ; 149(3): 416-28, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26926030

RESUMEN

INTRODUCTION: The purpose of this study was to present and validate a novel semiautomated method for 3-dimensional evaluation of the temporomandibular joint (TMJ) space and condylar and articular shapes using cone-beam computed tomographic data. METHODS: The protocol for 3-dimensional analysis with the Checkpoint software (Stratovan, Davis, Calif) was established by analyzing cone-beam computed tomographic images of 14 TMJs representing a range of TMJ shape variations. Upon establishment of the novel method, analysis of 5 TMJs was further repeated by several investigators to assess the reliability of the analysis. RESULTS: Principal components analysis identified 3 key components that characterized how the condylar head shape varied among the 14 TMJs. Principal component analysis allowed determination of the minimum number of landmarks or patch density to define the shape variability in this sample. Average errors of landmark placement ranged from 1.15% to 3.65%, and none of the 121 landmarks showed significant average errors equal to or greater than 5%. Thus, the mean intraobserver difference was small and within the clinically accepted margin of error. Interobserver error was not significantly greater than intraobserver error, indicating that this is a reliable methodology. CONCLUSIONS: This novel semiautomatic method is a reliable tool for the 3-dimensional analysis of the TMJ including both the form and the space between the articular eminence and the condylar head.


Asunto(s)
Tomografía Computarizada de Haz Cónico/estadística & datos numéricos , Procesamiento de Imagen Asistido por Computador/estadística & datos numéricos , Imagenología Tridimensional/estadística & datos numéricos , Articulación Temporomandibular/diagnóstico por imagen , Adolescente , Adulto , Puntos Anatómicos de Referencia/diagnóstico por imagen , Variación Anatómica , Niño , Femenino , Humanos , Masculino , Cóndilo Mandibular/diagnóstico por imagen , Variaciones Dependientes del Observador , Análisis de Componente Principal , Intensificación de Imagen Radiográfica/métodos , Reproducibilidad de los Resultados , Hueso Temporal/diagnóstico por imagen , Adulto Joven
12.
Am J Orthod Dentofacial Orthop ; 150(2): 238-51, 2016 08.
Artículo en Inglés | MEDLINE | ID: mdl-27476356

RESUMEN

INTRODUCTION: Disordered craniofacial development frequently results in definitive facial asymmetries that can significantly impact a person's social and functional well-being. The mandible plays a prominent role in defining facial symmetry and, as an active region of growth, commonly acquires asymmetric features. Additionally, syndromic mandibular asymmetry characterizes craniofacial microsomia (CFM), the second most prevalent congenital craniofacial anomaly (1:3000 to 1:5000 live births) after cleft lip and palate. We hypothesized that asymmetric rates of mandibular growth occur in the context of syndromic and acquired facial asymmetries. METHODS: To test this hypothesis, a spherical harmonic-based shape correspondence algorithm was applied to quantify and characterize asymmetries in mandibular growth and remodeling in 3 groups during adolescence. Longitudinal time points were automatically registered, and regions of the condyle and posterior ramus were selected for growth quantification. The first group (n = 9) had a diagnosis of CFM, limited to Pruzansky-Kaban type I or IIA mandibular deformities. The second group (n = 10) consisted of subjects with asymmetric, nonsyndromic dentofacial asymmetry requiring surgical intervention. A control group (n = 10) of symmetric patients was selected for comparison. A linear mixed model was used for the statistical comparison of growth asymmetry between the groups. RESULTS: Initial mandibular shape and symmetry displayed distinct signatures in the 3 groups (P <0.001), with the greatest asymmetries in the condyle and ramus. Similarly, mandibular growth had unique patterns in the groups. The dentofacial asymmetry group was characterized by significant asymmetry in condylar and posterior ramal remodeling with growth (P <0.001). The CFM group was characterized by asymmetric growth of the posterior ramus (P <0.001) but relatively symmetric growth of the condyles (P = 0.47). CONCLUSIONS: Forms of CFM are characterized by active and variable growth of the dysplastic side, which has a distinct pattern from other disorders of mandibular growth.


Asunto(s)
Asimetría Facial/etiología , Síndrome de Goldenhar/etiología , Mandíbula/anomalías , Adolescente , Algoritmos , Remodelación Ósea/fisiología , Estudios de Casos y Controles , Tomografía Computarizada de Haz Cónico , Asimetría Facial/diagnóstico por imagen , Asimetría Facial/fisiopatología , Asimetría Facial/cirugía , Femenino , Síndrome de Goldenhar/diagnóstico por imagen , Síndrome de Goldenhar/fisiopatología , Síndrome de Goldenhar/cirugía , Humanos , Imagenología Tridimensional , Masculino , Mandíbula/diagnóstico por imagen , Mandíbula/crecimiento & desarrollo , Estudios Retrospectivos
13.
J Craniofac Surg ; 26(6): 1954-6, 2015 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-26335328

RESUMEN

Hecht Syndrome is an autosomal dominant distal arthrogryposis caused by mutation in the MYH8 locus characterized by trismus and pseudocamptodactyly. Hecht-associated trismus is thought to result from bilateral hyperplasia of the mandibular coronoid processes. Although several interventions to address trismus have been pursued, no consensus exists regarding optimal management. In this report, the authors present a 7-month-old male with Hecht Syndrome referred for management of trismus. By age 2, interincisal opening had progressively decreased from 12 to 5 mm despite physical therapy. Nutrition was limited to liquids, oral hygiene was compromised, and aspiration risk was present. Computed tomography examination revealed enlarged coronoid processes extending medially and superiorly to the zygomatic arches. To release bony impaction of the coronoid processes against the zygoma and to prevent reossification of the temporalis tendon insertion, resection of the enlarged coronoids and distal temporalis muscles as well as placement of Alloderm spacers were performed via an open craniofacial transzygomatic approach. Jaw motion rehabilitation was used following surgery. Two years postoperatively, the patient had no signs of recurrence and good functional stability of jaw excursion. He was able to chew and swallow solid foods, protrude his tongue, use utensils, and perform regular oral hygiene, none of which were possible before surgery. This case demonstrates that open bilateral coronoidectomy can be a successful and durable management option for trismus in patients with Hecht Syndrome. The open transzygomatic approach is safe, has low morbidity, and provides direct access and adequate exposure for coronoid resection, spacer placement, and prevention of temporalis reinsertion.


Asunto(s)
Anomalías Múltiples/cirugía , Artrogriposis/cirugía , Mandíbula/cirugía , Trismo/cirugía , Dermis Acelular , Colágeno/uso terapéutico , Terapia por Ejercicio , Estudios de Seguimiento , Humanos , Hiperplasia , Lactante , Masculino , Osteotomía/métodos , Rango del Movimiento Articular/fisiología , Músculo Temporal/cirugía , Articulación Temporomandibular/fisiopatología , Cigoma/cirugía
15.
Am J Med Genet A ; 164A(6): 1425-30, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24668879

RESUMEN

Costello syndrome (CS) is a RASopathy characterized by a wide range of cardiac, musculoskeletal, dermatological, and developmental abnormalities. The RASopathies are defined as a group of syndromes caused by activated Ras/mitogen-activated protein kinase (MAPK) signaling. Specifically, CS is caused by activating mutations in HRAS. Although receptor tyrosine kinase (RTK) signaling, which is upstream of Ras/MAPK, is known to play a critical role in craniofacial and dental development, the craniofacial and dental features of CS have not been systematically defined in a large group of individuals. In order to address this gap in our understanding and fully characterize the CS phenotype, we evaluated the craniofacial and dental phenotype in a large cohort (n = 41) of CS individuals. We confirmed that the craniofacial features common in CS include macrocephaly, bitemporal narrowing, convex facial profile, full cheeks, and large mouth. Additionally, CS patients have a characteristic dental phenotype that includes malocclusion with anterior open bite and posterior crossbite, enamel hypo-mineralization, delayed tooth development and eruption, gingival hyperplasia, thickening of the alveolar ridge, and high palate. Comparison of the craniofacial and dental phenotype in CS with other RASopathies, such as cardio-facio-cutaneous syndrome (CFC), provides insight into the complexities of Ras/MAPK signaling in human craniofacial and dental development.


Asunto(s)
Síndrome de Costello/genética , Anomalías Craneofaciales/embriología , Anomalías Craneofaciales/genética , Sistema de Señalización de MAP Quinasas/genética , Proteínas Proto-Oncogénicas p21(ras)/genética , Anomalías Múltiples/embriología , Anomalías Múltiples/genética , Adolescente , Adulto , Niño , Hipoplasia del Esmalte Dental/embriología , Hipoplasia del Esmalte Dental/genética , Displasia Ectodérmica/embriología , Displasia Ectodérmica/genética , Facies , Insuficiencia de Crecimiento/embriología , Insuficiencia de Crecimiento/genética , Femenino , Hiperplasia Gingival/embriología , Hiperplasia Gingival/genética , Cardiopatías Congénitas/embriología , Cardiopatías Congénitas/genética , Humanos , Masculino , Maloclusión/embriología , Maloclusión/genética , Proteínas Quinasas Activadas por Mitógenos/genética , Mutación , Fosfatidilinositol 3-Quinasas/genética , Diente/embriología , Anomalías Dentarias/embriología , Anomalías Dentarias/genética , Adulto Joven
16.
J Calif Dent Assoc ; 42(3): 173-7, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25080723

RESUMEN

Evaluate the use of cone beam computed tomography among orthodontists in two areas of the Pacific Coast region via an electronic survey sent to the chairs of the orthodontic programs at the University of California, San Francisco, and A.T. Still University in Mesa, Ariz. The survey link was subsequently forwarded to each program's alumni. Overall, 85.7 percent of the orthodontists reported using CBCT scans. The scans were primarily used for impacted/supernumerary teeth and temporomandibular joint disorders analysis.


Asunto(s)
Tomografía Computarizada de Haz Cónico/estadística & datos numéricos , Ortodoncia/estadística & datos numéricos , Arizona , Tomografía Computarizada de Haz Cónico/economía , Tomografía Computarizada de Haz Cónico/instrumentación , Humanos , Imagenología Tridimensional/estadística & datos numéricos , Planificación de Atención al Paciente , Proyectos Piloto , Pautas de la Práctica en Odontología/estadística & datos numéricos , Práctica Privada , Dosis de Radiación , San Francisco , Trastornos de la Articulación Temporomandibular/diagnóstico por imagen , Diente Impactado/diagnóstico por imagen , Diente Supernumerario/diagnóstico por imagen
17.
Am J Med Genet C Semin Med Genet ; 163C(4): 318-32, 2013 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-24124058

RESUMEN

Dental anomalies are common congenital malformations that can occur either as isolated findings or as part of a syndrome. This review focuses on genetic causes of abnormal tooth development and the implications of these abnormalities for clinical care. As an introduction, we describe general insights into the genetics of tooth development obtained from mouse and zebrafish models. This is followed by a discussion of isolated as well as syndromic tooth agenesis, including Van der Woude syndrome (VWS), ectodermal dysplasias (EDs), oral-facial-digital (OFD) syndrome type I, Rieger syndrome, holoprosencephaly, and tooth anomalies associated with cleft lip and palate. Next, we review delayed formation and eruption of teeth, as well as abnormalities in tooth size, shape, and form. Finally, isolated and syndromic causes of supernumerary teeth are considered, including cleidocranial dysplasia and Gardner syndrome.


Asunto(s)
Dentición , Discapacidades del Desarrollo/fisiopatología , Diente/crecimiento & desarrollo , Diente/patología , Anomalías Múltiples/fisiopatología , Animales , Segmento Anterior del Ojo/anomalías , Segmento Anterior del Ojo/fisiopatología , Labio Leporino/complicaciones , Labio Leporino/fisiopatología , Fisura del Paladar/complicaciones , Fisura del Paladar/fisiopatología , Quistes/complicaciones , Quistes/fisiopatología , Discapacidades del Desarrollo/complicaciones , Discapacidades del Desarrollo/genética , Displasia Ectodérmica/complicaciones , Displasia Ectodérmica/fisiopatología , Anomalías del Ojo/complicaciones , Anomalías del Ojo/fisiopatología , Enfermedades Hereditarias del Ojo , Holoprosencefalia/complicaciones , Holoprosencefalia/fisiopatología , Humanos , Labio/anomalías , Labio/fisiopatología , Ratones , Síndromes Orofaciodigitales/complicaciones , Síndromes Orofaciodigitales/fisiopatología
18.
J Craniofac Surg ; 23(6): 1665-7, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23147311

RESUMEN

OBJECTIVE: The aim of this study was to determine the frequency of surgical correction of maxillary hypoplasia in individuals with nonsyndromic cleft lip and/or palate (CL/P) treated at the Center for Craniofacial Anomalies at University of California, San Francisco (UCSF). SUBJECTS: This is a retrospective cohort study of individuals with cleft lip and/or palate born between 1970 and 1990 who were treated at the UCSF Center for Craniofacial Anomalies. Data were gathered from the UCSF Craniofacial Anomalies Filemaker Pro database. METHODS: From the database, we collected the following information: age, gender, cleft type, date of orthognathic surgery, and type of osteotomy. The subjects were further subcategorized by cleft type and gender. RESULTS: A total of 973 individuals with a diagnosis of cleft lip and/or palate were reviewed: 325 subjects had an associated syndrome and 648 were nonsyndromic. A total of 59 of these 648 nonsyndromic cleft individuals (9.1%) required surgical intervention for correction of maxillary hypoplasia: 2/105 (1.9%) for cleft lip, 4/122 (3.3%) for cleft palate, 35/286 (12.2%) for unilateral cleft lip and palate, and 18/135 (13.3%) for bilateral cleft lip and palate. CONCLUSIONS: The frequency of surgical correction for maxillary hypoplasia in cleft individuals at UCSF Center for Craniofacial Anomalies at 9% was lower than the reported average of 25%.


Asunto(s)
Labio Leporino/cirugía , Fisura del Paladar/cirugía , Maxilar/anomalías , Maxilar/cirugía , Micrognatismo/cirugía , Femenino , Humanos , Lactante , Masculino , Osteotomía , Estudios Retrospectivos , Resultado del Tratamiento
19.
Cleft Palate Craniofac J ; 49(5): 553-60, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-21121765

RESUMEN

OBJECTIVE: The purpose was to compare the jaw size, jaw relationship, and facial proportions of children with nonsyndromic Pierre Robin sequence with children with nonsyndromic or isolated cleft palate. DESIGN: This is a retrospective cohort study comparing radiographic findings in children with Pierre Robin sequence or isolated cleft palate at two time intervals: ages 4 to 7 years (T1) and ages 10 to 13 years (T2). Linear and angular measurements were obtained using cephalograms; the cephalometric values were compared with unpaired t tests, assuming unequal variances. SETTING: The study was conducted at the Center for Craniofacial Anomalies at the University of California, San Francisco. PATIENTS: The sample included 13 children with Pierre Robin sequence and 14 children with isolated cleft palate who were followed at University of California, San Francisco, craniofacial anomalies clinic during the period from 1971 to 2007 and met the inclusion criteria. MAIN OUTCOME MEASURES: The outcome measures were mandibular length, maxillary length, and sagittal jaw relationship in Pierre Robin sequence and isolated cleft palate children at T1 and T2. RESULTS: During early childhood (T1: 4 to 7 years), mandibular length (Co-Gn) was similar in both Pierre Robin sequence and isolated cleft palate groups, as was the maxillary length (Co-A). In older children (T2: 10 to 13 years) mandibular length was significantly shorter in the Pierre Robin sequence group compared with the isolated cleft palate group (difference  =  10.9 mm, p  =  .009). Maxillary length in Pierre Robin sequence and isolated cleft palate groups was similar at T2 but significantly shorter in comparison to age-matched norms with a difference of 14.5 mm (p  =  .037) for Pierre Robin sequence and 12.4 mm (p  =  .045) for isolated cleft palate. Children with Pierre Robin sequence did not show a sagittal jaw discrepancy due to a proportionate deficiency in maxillary and mandibular length; whereas, children with isolated cleft palate showed a greater sagittal jaw discrepancy due to normal mandibular length and deficiency in maxillary length. CONCLUSION: Our study confirms the findings of some previous studies that suggest that children with nonsyndromic Pierre Robin sequence have a proportionate retrusion of the maxilla and mandible, resulting in a convex facial profile by early adolescence.


Asunto(s)
Desarrollo Maxilofacial , Síndrome de Pierre Robin/fisiopatología , Cráneo/anomalías , Adolescente , Cefalometría , Niño , Preescolar , Fisura del Paladar/diagnóstico por imagen , Fisura del Paladar/fisiopatología , Femenino , Humanos , Registro de la Relación Maxilomandibular , Masculino , Síndrome de Pierre Robin/diagnóstico por imagen , Radiografía , Estudios Retrospectivos , Cráneo/diagnóstico por imagen
20.
Am J Orthod Dentofacial Orthop ; 141(4 Suppl): S74-81, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22449602

RESUMEN

INTRODUCTION: Hemifacial microsomia is the most common congenital craniofacial anomaly after cleft lip and palate. The purpose of this study was to evaluate the final outcome of team care for patients with hemifacial microsomia after a protocol of growth management, orthodontic treatment, orthognathic surgery, and soft-tissue augmentation. METHODS: This was a retrospective study with chart reviews, radiographs, and photographs to document the treatment interventions and outcomes. Six patients, 2 in each of 3 mandibular types, who had recently completed treatment and had complete records available and were not previously reported, were included. Facial midlines and maxillary and mandibular deviations from the midline were measured. RESULTS: The maxillary deviation changed by 10.0 ± 4.6 mm to a deviation from the midline of 1.1 ± 0.6 mm. The chin deviation improved by 8.1 ± 2.7 mm to a mean distance of 0.6 ± 0.5 mm from the midline. The occlusal plane can't changed from 7.0° ± 4.2° to 2.3° ± 1.4°. All changes were significant. CONCLUSIONS: Facial asymmetry in patients with hemifacial microsomia can be significantly improved by stepwise orthodontic treatment, orthognathic and facial surgery, and soft-tissue augmentation. As for most craniofacial conditions, team care is essential.


Asunto(s)
Asimetría Facial/cirugía , Asimetría Facial/terapia , Ortodoncia Correctiva , Procedimientos Quirúrgicos Ortognáticos , Grupo de Atención al Paciente , Adolescente , Cefalometría/estadística & datos numéricos , Niño , Preescolar , Mentón/cirugía , Protocolos Clínicos , Tomografía Computarizada de Haz Cónico , Asimetría Facial/complicaciones , Asimetría Facial/diagnóstico por imagen , Femenino , Humanos , Lactante , Masculino , Maloclusión/etiología , Maloclusión/cirugía , Maloclusión/terapia , Mandíbula/anomalías , Mandíbula/cirugía , Maxilar/anomalías , Maxilar/cirugía , Ortodoncia Correctiva/métodos , Osteotomía Le Fort , Osteotomía Sagital de Rama Mandibular , Fotografía Dental , Procedimientos de Cirugía Plástica , Retrognatismo/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
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