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Panoramic Radiographic Evaluation of Dental Anomalies, Dental Mineralization Stage, and Mandibular Angle

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Panoramic Radiographic Evaluation of Dental Anomalies, Dental Mineralization Stage, and Mandibular Angle www.scielo.br/jaos http://dx.doi.org/10.1590/1678-775720160170 Fragile X syndrome: panoramic radiographic evaluation of dental anomalies, dental mineralization stage, and mandibular angle Aida SABBAGH-HADDAD1, Denise Sabbagh HADDAD2, Edgard MICHEL-CROSATO3, Emiko Saito ARITA2 1- Associação Paulista de Cirurgiões-Dentistas, Departamento de Odontologia para Pacientes com Necessidades Especiais, São Paulo, SP, Brasil. 2- Universidade de São Paulo, Faculdade de Odontologia, Departamento de Estomatologia, Disciplina de Radiologia, São Paulo, SP, Brasil. 3- Universidade de São Paulo, Faculdade de Odontologia, Departamento de Odontologia Social, São Paulo, SP, Brasil. Corresponding address: Denise Sabbagh Haddad - Av. Professor Lineu Prestes, 2227 - Cidade Universitária - São Paulo - SP - 05508-000 - Phone: 55(11)3091-7831 - e-mail: [email protected] 6XEPLWWHG$SULO0RGL¿FDWLRQ$XJXVWVW$FFHSWHG$XJXVW ABSTRACT ragile X syndrome (FXS) is a disorder linked to the chromosome X long arm (Xq27.3), FZKLFKLVLGHQWL¿HGE\DFRQVWULFWLRQQDPHGIUDJLOHVLWH,WGHWHUPLQHVYDULRXVFKDQJHV VXFKDVEHKDYLRUDORUHPRWLRQDOSUREOHPVOHDUQLQJGLI¿FXOWLHVDQGLQWHOOHFWXDOGLVDELOLWLHV Craniofacial abnormalities such as elongated and narrow face, prominent forehead, broad nose, large and prominent ear pavilions, strabismus, and myopia are frequent characteristics. Regarding the oral aspects, deep and high-arched palate, mandibular prognathism, and malocclusion are also observed. Objective: The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography. Material and Methods: The patients were in the range of 6-17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS), which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions. Results: The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05). In this group, supernumerary deciduous teeth (2.83%), giroversion of permanent teeth (2.31%), and partial anodontia (1.82%) were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05). Conclusion: We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals. Keywords: Fragile X syndrome. Intellectual disability. Tooth abnormalities. Panoramic radiography. Dentistry. INTRODUCTION region of the gene, which is located at the distal portion (Xq27.3) of the X-chromosome long arm. Fragile X (Martin-Bell) Syndrome (FXS) is an The fragile region is a gap, which is not stained inherited genetic disease, which is little known by cytological dyes and usually involves both by most professionals in the health area. For this chromatids at a point where the chromosome is reason, its actual incidence in the population is still susceptible to break. This characteristic can be unknown although its prevalence is known to be FRQ¿UPHG E\ NDU\RW\SH H[DPLQDWLRQ VLQFH WKH high. Recent studies have shown a pre-mutation VSHFL¿F LQYHVWLJDWLRQ WHFKQLTXH IRU IUDJLOH VLWHV prevalence in men (1:430) and women (1:209) in is used. In men, the diagnosis is made indirectly the USA16. through the polymerase chain reaction (PCR) The FXS designation is related to a fragile method. In women, however, this method is not J Appl Oral Sci. 518 2016;24(5):518-23 Fragile X syndrome: panoramic radiographic evaluation of dental anomalies, dental mineralization stage, and mandibular angle VXI¿FLHQWWRLQGLFDWHPXWDWLRQLQWKHJHQHDQGWKH fragile X syndrome. use of Southern Blot and Hybridization methods LVQHFHVVDU\IRU¿QDOGLDJQRVLVRIWKHV\QGURPH1. MATERIAL AND METHODS 0ROHFXODU VWXGLHV LGHQWL¿HG WKLV PXWDWLRQ LQ the Fragile X Mental Retardation type-1 (FMR1) This study was approved by the Institutional gene, which is located on the X chromosome, Review Board (CAAE 46419215.7.0000.0075) of and explains the fragile site in the subterminal the School of Dentistry, University of São Paulo portion of its long arm. Variable effects can (FOUSP). be observed in the phenotypic constitution of Forty clinical forms were selected. They included individuals with the syndrome due to a gene panoramic radiographic exams of individuals aged permutation and expansions observed in the Fragile between 6-17 years. These forms are part of the X Mental Retardation Protein (FMRP) (greater database of the author’s private clinic. or smaller amount of CGG nucleotides)6,9. The The sample was divided into two groups: 20 alteration in the FMRP is repeated in body cells, SDWLHQWVGLDJQRVHGZLWK);6FRQ¿UPHGE\PROHFXODU affecting various organic structures and functions, analysis (method of double digestion of genomic mainly those linked to the cognitive ability. Thus, DNA by the EcoRI and Eagle enzymes, followed by intellectual disability is the most important clinical Southern blot and hybridization with the StB12.3 manifestation, which is caused because the FMRP is probe), named FXS group and 20 individuals absent in the brain of these patients18,19. The degree compatible with the normal pattern, which were of intellectual disability is extremely variable, even called control group. Both groups were matched among individuals from the same family. However, for gender and age, including individuals with VHYHUHGH¿FLHQF\VHHPVWREHWKHPRVWIUHTXHQW deciduous, mixed, and permanent dentition. All of manifestation, which occurs in 42.0% of men them had anamnesis, oral clinical examination, and affected by this mutation7. panoramic radiographic examination. Given the variable clinical aspects, the consensus By panoramic radiographic evaluation (Figure in the literature is that the chromosomal or molecular 1), two radiologists analyzed the mandibular study of individuals with intellectual disability angle size, chronology of eruption according to of unknown origin is mandatory to identify the Nolla’s criteria10, and the dental anomalies related individuals affected by the FXS mutation1,6,7,9,16,18,19. to changes in shape, volume, position such as In the FXS, the typical clinical presentation shows macrodontia, microdontia, fusion, gemination, a classic triad, formed by macroorchidism (in men), concrescence, taurodontism, root fusion and large and prominent ear pavilions, and elongated laceration, dens in dente, transposition, giroversion, and narrow face7. In these individuals, the face is imperfect amelogenesis, partial anodontia, longer because their mandibles suffer a downward dental, root supernumerary, dental number of all rotation4. Their cephalic perimeter is increased and deciduous and permanent teeth, whether erupted the bizygomatic diameter and internal intercanthal or not. The kappa and intraclass correlation (ICC) distance are diminished. In addition, their height, coeI¿cients were used to test the intra- and inter- wingspan, and length of hands, feet, and digits are rater reliability. DOVRVLJQL¿FDQWO\LQFUHDVHG2. Nolla10 calculated the dental mineralization The behavioral characteristics present in the stage dividing the development of each tooth in );6 LQFOXGH SRRU H\H FRQWDFW ÀDSSLQJ KDQGV 10 stages, since the presence of dental crypt until defensive physical contact, and impulsivity, as well the complete formation of the apex10. The author as hyperactivity aggressiveness, anxiety, and self- separated tables for men and women, where the mutilation5,13. DYHUDJHFDOFL¿FDWLRQVWDJHVZHUHUHFRUGHGIRUHDFK The oral and facial clinical examination is a tooth in the age range of 6-17 years. priority in Dentistry, but few studies were found in The panoramic radiographs were analyzed the databases addressing the oral manifestations using a negatoscope, and the dental mineralization of the FXS. However, some authors cite deep and stages were interpreted using the Nolla’s 10-stage high-arched palate and prominent jaw as the main diagrams of dental development10. characteristics of FXS5,11. Furthermore, the presence Measurements of the mandibular angle were of macroglossia, partial anodontia, stains and obtained by the intersection of linear measurements enamel hypoplasia, shape anomalies, macrodontia, tangential to the mandible ramus and inferior and unilateral and bilateral crossbite have also been border. reported13. All data were compiled into a spreadsheet using The purpose of this study was to evaluate the WKH2I¿FH0LFURVRIW([FHO 0LFURVRIW&RUSRUDWLRQ dental structure alterations, mandibular angle Redmond, WA, USA) program. The SPSS 19® (IBM measurements, and dental mineralization stage Corporation; Armonk, NY, USA) program was through panoramic radiography, in individuals with used to obtain the descriptive statistics, Pearson’s J Appl Oral Sci. 519 2016;24(5):518-23 SABBAGH-HADDAD A, HADDAD DS, MICHEL-CROSATO E, ARITA ES correlation coefficients, and Student’s t test. intra- and interobserver agreement in all dental Differences, associations, and correlations were anomalies. ICC was used to test intra- and inter- FRQVLGHUHG VLJQL¿FDQW ZKHQ WKH WHVW GHVFULSWLYH rater reliability in the Nolla stage and mandibular level (p) was lower than 0.05. DQJOH PHDVXUHPHQWV %RWK FRHI¿FLHQWV LQGLFDWH The relationship between measurements was strong correlation (>0.8)
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