A combination of skeletal deformations of the dorsal mandible and temporomandibular region detected in orthopantomograms of patients with neurofibromatosis type 1 indicates an associated ipsilateral plexiform neurofibroma.

PURPOSE: Neurofibromatosis type 1 (NF1) is a tumour suppressor syndrome and also a bone disease. In the craniofacial region, local skeletal deformities have been recorded in patients with NF1. Obvious syndrome-related alterations of the jaws are particularly conspicuous in the mandible. Here we aimed to analyse the mandibular alterations of NF1-affected individuals from orthopantomograms (OPGs). MATERIALS AND METHODS: This study analyses 358 OPGs of 358 patients (mean age, 34.63 years; range, 12.57-69.13 years). The OPGs of 179 patients of Caucasian origin with NF1 were investigated (mean age, 34.84 years; range, 12.83-68.89 years; 44.13% male, 55.86% female). The radiographic findings were compared to those obtained from OPGs of sex- and age-matched controls. RESULTS: Characteristic deformations of the bone regions of interest occur in NF1 patients, especially in patients with plexiform neurofibroma of trigeminal nerve. These findings are always one-sided. The co-occurrence of several skeletal deformations can be expected with very high probability in this patient group. CONCLUSION: The radiological sign of the unilateral deformed mandible should be included in the diagnostic criteria for NF1. In addition to the diagnostic value in this syndrome, these findings are important for avoiding surgical complications in planned interventions in this region.

Decayed, missing, and restored teeth in patients with Neurofibromatosis Type 1.

BACKGROUND: NF1 is a relatively frequently occurring autosomal dominant inherited disease. There are conflicting reports about oral health status in NF1. The aim of this study was to analyze the dental status of patients with neurofibromatosis type 1 (NF1). MATERIAL AND METHODS: Radiographs of 179 patients with NF1 were analyzed for decayed, missing, and filled teeth (DMFT) in a cross-sectional, retrospective study. The results were compared to age- and sex-matched controls of individuals not affected by NF1. The NF1 group was differentiated for facial tumor type and localization. RESULTS: Missing teeth were more frequently registered in the NF1 group. On the other hand, decayed teeth were more frequent in the reference group. However, these findings had to be interpreted with caution, because the type and localization of the facial tumor affected the measured values. CONCLUSIONS: Dental health in terms of DMFT differed between NF1 patients and the control group. The presented results indicate the need for special care in dentistry in NF1 patients in order to preserve dental health, particularly in individuals affected with certain types of facial tumors. Key words:DMFT index, neurofibromatosis type 1, plexiform neurofibroma, oral health.

Periapical Cemento-osseous Dysplasia Is Rarely Diagnosed on Orthopantomograms of Patients with Neurofibromatosis Type 1 and Is Not a Gender-specific Feature of the Disease.

Several skeletal aberrations of the skull have been described for the tumor predisposition syndrome neurofibromatosis type 1 (NF1). Recently, periapical cemental/cemento-osseous dysplasia (COD) has been described in females affected with NF1. This reactive lesion of the hard tissues in tooth-bearing areas of the jaw has been proposed to represent a gender-specific radiological feature of NF1. The aim of this study was to investigate the prevalence of COD in patients with NF1. PATIENTS AND METHODS: The orthopantomograms (OPGs) of 179 patients with a confirmed diagnosis of NF1 were analyzed for COD. The results were compared to radiographic findings obtained in OPGs of age- and sex-matched controls. The NF1 patient group was further differentiated according to the evidence of facial plexiform neurofibroma. RESULTS: COD was a very rare finding in both groups. The extension of the diagnostic criteria including radiologically-healthy teeth and a widened periodontal gap in the periapical area only marginally increased the number of considered cases. Although there was a somewhat more common occurrence of such changes in the patient group compared to the control group and the number of affected women was greater than the number of men, none of these differences reached statistical significance. Furthermore, COD or widening of the periradicular periodontal space was not found to be associated with facial tumor type in NF1. CONCLUSION: The investigation revealed that COD is not a diagnostic feature of NF1. There is no clear association of the rare finding of COD with gender. These studies should be compared with patient groups of other ethnic backgrounds.

Can we measure patients' perception during dental impressions? The Burdens in Dental Impression-Making Questionnaire - BiDIM-Q.

PURPOSE: To develop a reliable and valid instrument for the comprehensive assessment of patients' burdens during dental impression making, the Burdens in Dental Impression Making Questionnaire, BiDIM-Q. MATERIALS: The item pool was generated in a convenience sample of 20 prosthodontic patients using semi-structured face-to-face interviews. The final instrument was tested in 145 consecutively recruited patients, and psychometric properties of the BiDIM-Q were determined. Four different impression materials were used according to the manufacturers' instructions and indications: alginate, c-silicone, polyvinylsiloxane, and polyether. RESULTS: The final BiDIM-Q consisting of 12 items showed sufficient reliability, indicated by Cronbach's alpha of .82 and an average inter-item correlation of .29. Validity was supported by Pearson correlation coefficients for the correlation between the instrument's total score with the patients' overall satisfaction rating (r=.63), and by the correlation matrix for the correlations of the patients' perceptions with the practitioners' satisfaction ratings. Overall, patient perceived burdens were low with highest burdens observed when using polyether in partially dentate patients for pick-up impressions, while lowest burdens were reported when using c-silicone for impressions of edentulous jaws. CONCLUSION: The BiDIM-Q is a reliable and valid tool for assessing patient-based process-related quality of care in dentistry allowing a deeper insight into patients' perspective during dental impression making.

Supernumerary Molars and Wisdom Tooth Shape Alterations in Patients with Neurofibromatosis Type 1.

OBJECTIVES: The purpose of the present study was the systematic investigation of numerical and morphological aberrations of teeth in the wisdom teeth region in neurofibromatosis type 1 patients. MATERIAL AND METHODS: The panoramic radiographs of 179 patients were analysed for supernumerary molars and dysplastic wisdom teeth with special reference to facial tumour type. The results were compared to radiographic findings obtained in panoramic radiographs of age- and sex-matched controls. RESULTS: The total number of dysplastic or supernumerary molars was 21 (17 individuals, 4.75%) and predominantly a finding of the maxilla (maxilla: 15, mandible: 1, maxilla and mandible: 1). Differences of dental findings between neurofibromatosis type 1 (NF1) patients (n = 15) and the control group (n = 2) were statistically significant (P < 0.01). All individuals with supernumerary molars were diagnosed as being affected with NF1. This difference achieved statistical significance when it was considered that the event may occur at least once per quadrant, and thus the individual potentially needs to be considered four times (P = 0.0038). CONCLUSIONS: This investigation revealed that supernumerary molars and aberrations in wisdom tooth form are more common in patients than in age- and sex-matched controls. There is no association between these findings and a specific feature of the disease, id est facial manifestation of a trigeminal nerve sheath tumour (plexiform neurofibroma). The cause of this phenomenon is unknown. A review of these dental findings on other neurofibromatosis type 1 study groups should be attempted.