Reinhard E Friedrich1,2, Hannah T Scheuer3,2, Jan F Kersten4, Hanna A Scheuer5,6. 1. Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany; rfriedrich@uke.de. 2. Neurofibromatosis Laboratory, Hamburg, Germany. 3. Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany. 4. Competence Centre for Epidemiology and Health Services Research for Healthcare Professionals (CVcare), University Medical Centre Hamburg-Eppendorf, Hamburg, Germany. 5. Orthodontic Practice, Hamburg-Lokstedt, Hamburg, Germany. 6. Department of Orthodontics, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany.
Abstract
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. Copyright
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. Copyright
Authors: Reinhard E Friedrich; Jonathan Rother; Georg Christ; Marten Lehmann; Christine Gräfin Zu Eulenburg; Manfred Giese; Hanna A Scheuer Journal: Anticancer Res Date: 2013-03 Impact factor: 2.480