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BACKGROUND: Anxiety and depression are among the most common and debilitating psychiatric disorders affecting youth, with both related to increased suicide risk. While rates of youth anxiety and depression were increasing before the COVID-19 pandemic, the pandemic further negatively impacted adolescent mental health. Unfortunately, few studies have examined prevalence of these concerns among early adolescents (ages 10-13) longitudinally during the pandemic. METHOD: The current study examined self-reported anxiety and depression symptoms, and suicidal ideation amongst a general pediatrics population of 11- to 13-year-olds (n = 623) from March through September 2020 (early-pandemic) and approximately 7 months later (September 2020 through May 2021; mid-pandemic). Paired samples proportions were used to examine changes in prevalence of moderate to severe anxiety, depression, and suicidal ideation from early- to mid-pandemic. RESULTS: Results highlight high initial rates and stability in anxiety and suicidal ideation, as well as a significant increase in depression (42.9% increase; p < .05) among the full sample during the COVID-19 pandemic. Prevalance of concerns were greatest for females and Hispanic youth during the early-pandemic, and generally highest for females and Medicaid insured youth at mid-pandemic. DISCUSSION: Results extend recent research and underscore the need for continued monitoring of mental health concerns across development for youth who grew up during the COVID-19 pandemic; highlighting the need for sustainable, effective, and accessible early detection, prevention, and intervention strategies. Improving these services is critical to support youth who experienced pandemic-related stressors, and to prepare for supporting youth during future disruptive and isolating events.
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Ansiedade , COVID-19 , Depressão , Ideação Suicida , Humanos , COVID-19/psicologia , COVID-19/epidemiologia , Adolescente , Feminino , Masculino , Criança , Depressão/epidemiologia , Depressão/psicologia , Ansiedade/epidemiologia , Ansiedade/psicologia , Estados Unidos/epidemiologia , SARS-CoV-2 , Prevalência , Estudos Longitudinais , Autorrelato , PandemiasRESUMO
Background: Incorporating post-discharge data into trauma registries would allow for better research on patient outcomes, including disparities in outcomes. This pilot study tested a follow-up data collection process to be incorporated into existing trauma care systems, prioritizing low-cost automated response modalities. Methods: This investigation was part of a larger study that consisted of two protocols with two distinct cohorts of participants who experienced traumatic injury. Participants in both protocols were asked to provide phone, email, text, and mail contact information to complete follow-up surveys assessing patient-reported outcomes six months after injury. To increase follow-up response rates between protocol 1 and protocol 2, the study team modified the contact procedures for the protocol 2 cohort. Frequency distributions were utilized to report the frequency of follow-up response modalities and overall response rates in both protocols. Results: A total of 178 individuals responded to the 6-month follow-up survey: 88 in protocol 1 and 90 in protocol 2. After implementing new follow-up contact procedures in protocol 2 that relied more heavily on the use of automated modalities (e.g., email and text messages), the response rate increased by 17.9 percentage points. The primary response modality shifted from phone (72.7%) in protocol 1 to the combination of email (47.8%) and text (14.4%) in protocol 2. Conclusions: Results from this investigation suggest that follow-up data can feasibly be collected from trauma patients. Use of automated follow-up methods holds promise to expand longitudinal data in the national trauma registry and broaden the understanding of disparities in patient experiences.
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OBJECTIVE: Few investigations have comprehensively described methods for assessing and monitoring suicidal ideation in pragmatic clinical trials of mental health services interventions. This investigation's goal was to assess a collaborative care intervention's effectiveness in reducing suicidal ideation and describe suicide monitoring implementation in a nationwide protocol. METHOD: The investigation was a secondary analysis of a stepped wedge cluster randomized trial at 25-Level I trauma centers. Injury survivors (N = 635) were randomized to control (n = 370) and intervention (n = 265) conditions and assessed at baseline hospitalization and follow-up at 3-, 6- and 12-months post-injury. The Patient Health Questionnaire (PHQ-9) item-9 was used to evaluate patients for suicidal ideation. Mixed model regression was used to assess intervention versus control group changes in PHQ-9 item-9 scores over time and associations between baseline characteristics and development of suicidal ideation longitudinally. As part of the study implementation process assessment, suicide outreach call logs were also reviewed. RESULTS: Over 50% of patients endorsed suicidal ideation at ≥1 assessment. Intervention patients relative to control patients demonstrated reductions in endorsements of suicidal ideation that did not achieve statistical significance (F[3,1461] = 0.74, P = .53). The study team completed outreach phone calls, texts or voice messages to 268 patients with PHQ-9 item-9 scores ≥1 (n = 161 control, n = 107 intervention). CONCLUSIONS: Suicide assessment and monitoring can be feasibly implemented in large-scale pragmatic clinical trials. Intervention patients demonstrated less suicidal ideation over time; however, these comparisons did not achieve statistical significance. Intensive pragmatic trial monitoring may mask treatment effects by providing control patients a supportive intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT02655354.
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Serviços Médicos de Emergência , Prevenção do Suicídio , Suicídio , Humanos , Ideação Suicida , Suicídio/psicologia , SobreviventesRESUMO
INTRODUCTION: Opioid use disorder (OUD) and related comorbid conditions are highly prevalent among patients presenting to emergency department (ED) settings. Research has developed few comprehensive disease management strategies for at-risk patients presenting to the ED that both decrease illicit opioid use and improve initiation and retention in medication treatment for OUD (MOUD). METHODS: The research team conducted a pilot pragmatic clinical trial that randomized 40 patients presenting to a single ED to a collaborative care intervention (n = 20) versus usual care control (n = 20) conditions. Interviewers blinded to patient intervention and control group status followed-up with participants at 1, 3, and 6 months after presentation to the ED. The 3-month Emergency Department Longitudinal Integrated Care (ED-LINC) collaborative care intervention for patients at risk for OUD included: 1) a Brief Negotiated Interview at bedside, 2) overdose education and facilitation of MOUD, 3) longitudinal proactive care management, 4) utilization of the statewide health information exchange platform for 24/7 tracking of recurrent ED utilization, and 5) weekly caseload supervision that incorporated measurement-based care treatment assessment with stepped-up care for patients with recalcitrant symptoms. RESULTS: Overall, the ED-LINC intervention was feasibly delivered and acceptable to patients. The pilot study achieved >80% follow-up rates at 1, 3, and 6 months. In adjusted longitudinal mixed model regression analyses, no statistically significant differences existed in days of opioid use over the past 30 days for ED-LINC intervention patients when compared to patients receiving usual care (incidence-rate ratio (IRR) 1.50, 95% CI 0.54-4.16). The unadjusted mean number of days of illicit opioid use decreased at the 1-month and 3-month follow-up time points for both groups. ED-LINC intervention patients had increased rates of MOUD initiation compared to control patients (50% versus 30%); intervention versus control comparisons did not achieve statistical significance, although power to detect significant differences in the pilot was limited. CONCLUSIONS: The ED-LINC intervention for patients with OUD can be feasibly implemented and warrants testing in larger scale, adequately powered randomized pragmatic clinical trial investigations. CLINICALTRIALS: gov NCT03699085.
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Prestação Integrada de Cuidados de Saúde , Transtornos Relacionados ao Uso de Opioides , Analgésicos Opioides/uso terapêutico , Serviço Hospitalar de Emergência , Humanos , Transtornos Relacionados ao Uso de Opioides/tratamento farmacológico , Projetos PilotoRESUMO
Evidence-based parenting interventions play a crucial role in the sustained reduction of adolescent behavioral health concerns. Guiding Good Choices (GGC) is a 5-session universal anticipatory guidance curriculum for parents of early adolescents that has been shown to reduce substance use, depression symptoms, and delinquent behavior. Although prior research has demonstrated the effectiveness of evidence-based parenting interventions at achieving sustained reductions in adolescent behavioral health concerns, public health impact has been limited by low rates of uptake in community and agency settings. Pediatric primary care is an ideal setting for implementing and scaling parent-focused prevention programs as these settings have a broad reach, and prevention programs implemented within them have the potential to achieve population-level impact. The current investigation, Guiding Good Choices for Health (GGC4H), tests the feasibility and effectiveness of implementing GGC in 3 geographically and socioeconomically diverse large integrated healthcare systems. This pragmatic, cluster randomized clinical trial will compare GGC parenting intervention to usual pediatric primary care practice, and will include approximately 3750 adolescents; n = 1875 GGC intervention and n = 1875 usual care. The study team hypothesizes that adolescents whose parents are randomized into the GGC intervention arm will show reductions in substance use initiation, the study's primary outcomes, and other secondary (e.g., depression symptoms, substance use prevalence) and exploratory outcomes (e.g., health services utilization, anxiety symptoms). The investigative team anticipates that the implementation of GGC within pediatric primary care clinics will successfully fill an unmet need for effective preventive parenting interventions. Trial registration: Clinicaltrials.govNCT04040153.
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Comportamentos de Risco à Saúde , Pais , Adolescente , Ansiedade , Criança , Humanos , Poder Familiar , Pais/educação , Atenção Primária à Saúde , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is a tumor predisposition syndrome. Bone findings make a significant contribution to the clinical diagnosis of NF1. It has been suggested that there are characteristic skeletal features of the NF1 patients' skull that cause a specific 'NF1 facies'. To test this thesis, skull examinations were carried out on NF1 patients. PATIENTS AND METHODS: The posteroanterior (PA) cephalograms of 76 patients with NF1 were analyzed using defined measuring points. Patients with confirmed facial plexiform neurofibromas (PNF) were excluded from the study. A special interest of the investigation was the symmetry of the measuring points defined as the distance to the median sagittal plane. RESULTS: NF1 patients have a slightly larger distance to the Z-plane than controls at the zygomatic arch and mastoid measurement points (p=0.027 and 0.028, respectively). In contrast, the distances of the juga and antegonion measurement points from the horizontal reference plane are larger in the control group (p=0.002 and 0.480, respectively). The transverse development of the midface at the level of the zygomatic arch showed no differences from the control group (p=0.841). The transverse diameter of the skull at the mastoid and juga measurement points is smaller in the NF1 group compared to the control group (p=0.010 and 0.002, respectively). There is a statistically significant left-right (LR) asymmetry of the distances to the median sagittal plane in favor of the left side in the patient group (p=0.002 to 0.037). However, the numerical deviations from the control group are small overall. CONCLUSION: Considering the natural, biological deviations of cephalometric measurements of the individual from idealized geometric norms, the facial skeleton of NF1 patients is symmetrically developed. It is unlikely that the calculated LR asymmetry of the patients has a visible effect. In comparison to cephalometric values of a normal population, no characteristic facial skeleton of the NF1 patient in the PA projection of the skull can be derived from these findings. Clear asymmetries of the facial skeleton should give rise to further diagnosis to clarify the suspicion of facial PNF.
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Cefalometria/métodos , Lateralidade Funcional , Neurofibromatose 1/patologia , Crânio/patologia , Adolescente , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Prognóstico , Crânio/diagnóstico por imagem , Adulto JovemRESUMO
BACKGROUND: Radiological cephalometry is an important diagnostic tool for analyzing the shape and proportions of the skull. Standardized teleradiography of the skull in posterior-anterior (PA) projection provides orientation data on the symmetry and vertical relations of the skull. The comparison of individual findings with normal values places high demands on the selection of a control group. The aim of this study was to characterize a group to be used as a standard for cephalometric comparisons. PATIENTS AND METHODS: PA teleradiographs of 23 healthy young adults were analyzed. Distances from reference measuring points to the median sagittal plane and the orbital horizontal plane were made. All individuals showed ideal occlusion. None of the participants had been subjected to orthodontic therapy or craniomaxillofacial surgery. RESULTS: The measurement results showed a high degree of lateral symmetry of the skeletal reference points and planes. Comparison of the vertical reference lines confirmed the symmetrical constitution of the facial skeleton. CONCLUSION: The study group is suitable for comparison with the cephalometric evaluations of other study groups.
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Crânio , Cefalometria , Humanos , Radiografia , Valores de Referência , Crânio/diagnóstico por imagem , Adulto JovemRESUMO
BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disease that causes tumors and many developmental disorders, e.g., cranial dysplasia. The purpose of this retrospective study was to analyse the pneumatisation of the sphenoid bone in NF1. PATIENTS AND METHODS: The anonymised lateral cephalograms of 166 NF1 patients and 166 age- and sex-matched controls were examined for anterior-posterior sphenoid pneumatisation. The patient group analysis considered whether the patients had been affected by a facial plexiform neurofibroma (FPNF). RESULTS: Sphenoid pneumatisation was significantly lower in NF1 patients than in controls [odds ratio (OR)=0.184; 95%CI=0.11-0.32; p<0.001]. A FPNF statistically significantly reduced sinus formation in patients (OR=0.38; p=0.002). CONCLUSION: The condition 'NF1' has an effect on sphenoid pneumatisation. The findings are relevant for planning surgical procedures in this region and confirm current concepts to evaluate NF1 as a histogenesis control gene. The examination technique and basis of calculation presented here are easy-to-use and low-irradiation exposure instruments for screening for differences in sphenoid bone pneumatisation in defined populations.
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Neurofibroma Plexiforme , Neurofibromatose 1 , Humanos , Neurofibromatose 1/diagnóstico por imagem , Neurofibromatose 1/genética , Razão de Chances , Radiografia , Estudos Retrospectivos , Osso Esfenoide/diagnóstico por imagemRESUMO
BACKGROUND/AIM: Numerical aberrations of permanent dentition and dystopic tooth eruption are part of the phenotype of the tumor predisposition syndrome neurofibromatosis type 1 (NF1). In these cases, surplus tooth germs usually develop in the alveolar processes of the jaw. This report attests to the dystopic development of a dysplastic supernumerary tooth in NF1 arising outside the jaw. CASE REPORT: The 8-year-old male patient developed a microdont outside the bone and above the occlusal plane of the retained maxillary right second molar. The supernumerary tooth was completely embedded in oral soft tissue. Hyperplastic oral soft tissue in the molar region and microdont were excised. Specimen of the mucosa surrounding the teeth was interspersed with diffuse and plexiform neurofibroma. The retained upper right first molar emerged spontaneously within a few months after surgery. The upper right second molar did not change position. CONCLUSION: Odontogenesis can take place within tumorous oral mucosa in NF1. Surgical removal of the tumorous mucous membrane facilitates tooth eruption in some cases.
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Processo Alveolar/patologia , Neoplasias Bucais/diagnóstico , Neurofibroma Plexiforme/diagnóstico , Neurofibromatose 1/diagnóstico , Erupção Ectópica de Dente/diagnóstico , Processo Alveolar/anormalidades , Processo Alveolar/diagnóstico por imagem , Processo Alveolar/cirurgia , Criança , Humanos , Masculino , Neoplasias Bucais/complicações , Neoplasias Bucais/patologia , Neoplasias Bucais/cirurgia , Neurofibroma Plexiforme/complicações , Neurofibroma Plexiforme/patologia , Neurofibroma Plexiforme/cirurgia , Neurofibromatose 1/complicações , Neurofibromatose 1/patologia , Neurofibromatose 1/cirurgia , Anormalidades Dentárias/diagnóstico , Anormalidades Dentárias/etiologia , Anormalidades Dentárias/patologia , Anormalidades Dentárias/cirurgia , Erupção Ectópica de Dente/etiologia , Erupção Ectópica de Dente/cirurgia , Dente Decíduo/anormalidades , Dente Decíduo/diagnóstico por imagem , Dente Decíduo/patologia , Dente Decíduo/cirurgia , Dente Supranumerário/diagnóstico , Dente Supranumerário/etiologia , Dente Supranumerário/patologia , Dente Supranumerário/cirurgiaRESUMO
PURPOSE: The purpose of the study was the investigation of supernumerary teeth (ST) in the permanent dentition of patients with neurofibromatosis type 1 (NF1). MATERIAL AND METHODS: The panoramic radiographs of 200 NF1 patients were analysed for ST. The potential impact of certain neurogenic facial tumours on the measured variable was investigated separately. The results were compared to dental findings in panoramic radiographs of 200 age- and sex-matched controls. RESULTS: The total number of ST in all individuals was 23 in 12 patients (3% of total population). However, all ST had developed in the group of NF1 patients (6%), (p = 0.0004). ST were particularly common distal to the molar region. Although ST occur in the region of facial plexiform neurofibroma, the numerical aberrations of the dentition are independent of specific type of facial nerve sheath tumour. CONCLUSION: The unusual accumulation of ST in the terminal sections of molar development could be an indicator of preferred points of developmental disorder, in which the genetic predisposition of the patient is involved. Reviewing the results of other NF1 patient collectives is desirable.
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Neurofibroma Plexiforme , Neurofibromatose 1 , Dente Supranumerário , Dentição Permanente , Humanos , DenteRESUMO
OBJECTIVE: The Coronavirus disease (COVID-19) outbreak has evolved into a pandemic crisis, with King County in Washington State emerging as the early US epicenter. A literature review revealed few reports providing front-line clinical and research teams guidance related to multilevel, rapidly evolving COVID-19 directives. METHOD: The Rapid Assessment Procedure Informed Clinical Ethnography (RAPICE) method was used to develop a clinical case series and conduct participant observation during an ongoing comparative effectiveness trial of peer-integrated, patient-centered interventions after traumatic injury. Participants were patients enrolled in the intervention arm of the ongoing trial, as well as front-line clinicians, patient peer interventionists, and clinical research team members implementing the trial. All participants were exposed to the Washington State COVID-19 outbreak. RESULTS: Primary and secondary COVID-19 prevention strategies were feasibly integrated into ongoing care coordination and behavioral interventions for at-risk patients. Beyond the compilation of case studies, as an iterative method, RAPICE data collection naturalistically evolved to include observations of intervention team activity occurring within the larger pandemic epicenter context. A daily clinical research team huddle that flexibly accommodated virtual participation was also feasibly implemented. CONCLUSIONS: Primary and secondary COVID-19 prevention strategies can be feasibly integrated into ongoing clinical interventions during the pandemic. Routine, proactive clinical and research team communication that transparently addresses ethical tensions and health-sustaining activities may promote well-being for providers grappling with rapidly evolving pandemic directives. Proactive assessments of individual provider vulnerabilities for severe COVID-19 related respiratory illness may also be a crucial element of the health care system pandemic responses.
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Infecções por Coronavirus/prevenção & controle , Controle de Infecções/métodos , Pandemias/prevenção & controle , Equipe de Assistência ao Paciente , Grupo Associado , Pneumonia Viral/prevenção & controle , Ferimentos e Lesões/terapia , Acidentes de Trânsito , Adolescente , Idoso de 80 Anos ou mais , Antropologia Cultural , Betacoronavirus , COVID-19 , Serviços de Saúde Comunitária , Feminino , Fraturas do Fêmur , Fraturas Múltiplas , Humanos , Masculino , Pessoa de Meia-Idade , Atenção Primária à Saúde , Quadriplegia , Ensaios Clínicos Controlados Aleatórios como Assunto , Medição de Risco , SARS-CoV-2 , Traumatismos da Medula Espinal , Washington , Ferimentos e Lesões/psicologia , Ferimentos por Arma de FogoRESUMO
Annually approximately 2-3 million Americans are so severely injured that they require inpatient hospitalization. The study team, which includes patients, clinical researchers, front-line provider and policy maker stakeholders, has been working together for over a decade to develop interventions that target improvements for US trauma care systems nationally. This pragmatic randomized trial compares a multidisciplinary team collaborative care intervention that integrates front-line trauma center staff with peer interventionists, versus trauma team notification of patient emotional distress with mental health consultation as enhanced usual care. The peer-integrated collaborative care intervention will be supported by a novel emergency department exchange health information technology platform. A total of 424 patients will be randomized to peer-integrated collaborative care (n = 212) and surgical team notification (n = 212) conditions. The study hypothesizes that patient's randomized to peer integrated collaborative care intervention will demonstrate significant reductions in emergency department health service utilization, severity of patient concerns, post traumatic stress disorder symptoms, and physical limitations when compared to surgical team notification. These four primary outcomes will be followed-up at 1- 3-, 6-, 9- and 12-months after injury for all patients. The Rapid Assessment Procedure Informed Clinical Ethnography (RAPICE) method will be used to assess implementation processes. Data from the primary outcome analysis and implementation process assessment will be used to inform an end-of-study policy summit with the American College of Surgeons Committee on Trauma. The policy summit will facilitate acute care practice changes related to patient-centered care transitions over the course of a single 5-year funding cycle. Trial registration: (Clinicaltrials.govNCT03569878).
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Serviço Hospitalar de Emergência/organização & administração , Tecnologia da Informação , Serviços de Saúde Mental/organização & administração , Equipe de Assistência ao Paciente/organização & administração , Estresse Psicológico/terapia , Ferimentos e Lesões/terapia , Comportamento Cooperativo , Humanos , Desempenho Físico Funcional , Qualidade de Vida , Projetos de Pesquisa , Método Simples-Cego , Transtornos de Estresse Pós-Traumáticos/epidemiologia , Transtornos de Estresse Pós-Traumáticos/prevenção & controle , Estresse Psicológico/psicologia , Índices de Gravidade do Trauma , Estados Unidos , Ferimentos e Lesões/psicologiaRESUMO
BACKGROUND/AIM: The purpose of the study was to investigate whether non-odontogenic intraosseous translucent lesions of mandibular body are depicted on radiographs of patients with neurofibromatosis type 1 (NF1). MATERIALS AND METHODS: The panoramic radiographs of 179 NF1 patients were analysed for translucent lesions of the mandibular body that were of intraosseous, non-odontogenic origin. The results were compared to findings obtained in panoramic radiographs of age- and sex-matched controls. RESULTS: Only three patients showed intraosseous translucent lesions. These were always unilocular findings. There were no statistically significant differences between the groups (p=0.248). CONCLUSION: Intraosseous neurofibroma of the jaw is a very rare finding in NF1 patients compared to oral neurofibromas. Accurate and exact diagnosis should be made in the case of such findings because malignant tumours in the jaw area can arise in rare cases in NF1 patients. Plain radiology findings cannot clearly indicate the type and biology of the lesion.
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Mandíbula/diagnóstico por imagem , Neoplasias Mandibulares/diagnóstico por imagem , Neurofibromatose 1/diagnóstico por imagem , Radiografia Panorâmica , Adolescente , Adulto , Idoso , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Mandíbula/patologia , Neoplasias Mandibulares/patologia , Pessoa de Meia-Idade , Neurofibromatose 1/patologia , Valor Preditivo dos Testes , Adulto JovemRESUMO
Depression is common among adolescents, affecting greater than 12% of youth in a given year. Studies have shown aberrant amygdala connectivity in depressed adolescents, compared with controls; however, no studies have examined whether these abnormalities precede and heighten risk for depressive symptom expression. This study used resting state functional connectivity (RSFC) magnetic resonance imaging to examine neurobiological markers of escalating depression symptoms in adolescents (ages 12-16 years; free from psychopathology at baseline). Of a large sample of adolescents, 18 showed ≥ 1 S.D. increase in depression scale t-scores over time ("escalators"; time to escalation ranging from 6 to 54 months in follow up) and were matched and compared to 19 youth showing stable CDI scores over time ("controls"). Whole-brain analyses on baseline RSFC data using an amygdala seed region-of-interest (ROI) showed that controls had greater RSFC, relative to escalators, between the right amygdala and left inferior frontal and supramarginal gyrus and right mid-cingulate cortex. Additionally, relative to escalators, control youth had less RSFC between the left amygdala and cerebellum. Findings suggest a possible neurobiological marker of increasing depressive symptoms during adolescence, characterized in part by reduced fronto-limbic connectivity, suggesting a premorbid deficiency in top-down emotional regulation.
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Tonsila do Cerebelo/fisiopatologia , Córtex Cerebral/fisiopatologia , Conectoma/métodos , Transtorno Depressivo Maior/fisiopatologia , Adolescente , Tonsila do Cerebelo/diagnóstico por imagem , Córtex Cerebral/diagnóstico por imagem , Criança , Transtorno Depressivo Maior/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
The aim of this study was to measure line segments and areas of sella turcica on lateral cephalograms with respect to the clinical diagnosis of facial phenotype of patients with neurofibromatosis type 1 (NF1). Special attention was given to correlate the measured values with certain tumour types that are typical for this disease. Material and methods: Lateral cephalograms of 194 individuals were investigated. Patients with NF1 were further divided according to the detection and topography of facial plexiform neurofibromas (PNF) taking into account the distribution pattern of the trigeminal nerve. All patients with PNF showed unilateral tumour localisation. Patients without any facial PNF constituted a separate group. Healthy volunteers with ideal occlusion and no history of any intervention in the maxillofacial region served as a control group. The following items were determined on the radiographs: sella entrance, sella width, sella depths, sella diagonal, and sella area. Results: Patients with PNF of the first and second trigeminal nerve branch or affected in all branches showed highly statistically significant enlarged sella tucica measurement values. On the other hand, patients with PNF restricted to one branch only or simultaneously in the second and third branches showed measurement values that were not different to those obtained in NF1 patients devoid of facial PNF. The latter group also showed no difference of sella turcica parameters obtained in the control group. Conclusion: This study provides evidence for the association of a certain NF1 phenotype with distinct skeletal alterations of the skull base, shown here using the example of the representation of the sella turcica in the lateral radiograph. These findings are also relevant in the discussion of NF1 as a disease of bones and in the assessment of brain development in NF1. Both items are discussed in relationship to a facial plexiform neurofibroma. Furthermore, the knowledge of this association of findings provides the clinician with relevant information in the planning of skull base procedures in these patients.
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PURPOSE: Neurofibromatosis type 1 (NF1) is an autosomal dominant transmitted tumour suppressor syndrome and also a bone disease. Osseous dysplasia affecting the craniofacial region is characteristic of NF1. The aim of this study was to analyse the lateral cephalograms of NF1 patients in comparison to individuals who were not affected by this condition in order to describe the skeletal phenotype of NF1 in more detail. MATERIALS AND METHODS: The study comprises the lateral cephalograms of 172 patients with established NF1 diagnoses (female = 85, male = 87). NF1 patients were distinguished by radiological and/or histological findings of the facial region suggestive of plexiform neurofibroma (PNF) or disseminated cutaneous NF (DNF). The analysed radiographs of a collection of 29 healthy volunteers with ideal occlusion served as controls. The focus of this analysis was cephalometrically defined angles. RESULTS: Cephalometric analyses of patients with DNF did not differ from those of controls for the vast majority of parameters. However, the measurement results of patients with PNF differed significantly from those of healthy volunteers and patients with DNF. The number of trigeminal nerve branches affected in PNF patients had an effect on the measurement results. CONCLUSION: Lateral cephalograms revealed no significant alteration of the facial skeleton in NF1 patients as compared to controls. Indeed, the stigma of a so-called 'NF1 facies' cannot be derived from the cephalometric findings presented. Notably, a wide range of deviating readings were recorded for individuals with facial PNF. Clinicians who treat patients with NF1 should be aware of deviations from cephalometric standards on lateral cephalograms in NF1 patients, especially when craniofacial surgical procedures are planned. Some of these findings, particularly asymmetries of the facial skeleton, could be indicators of an associated PNF.