Dental anomalies occur because of a variety of genetic

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1 CLINICIAN S CORNER Multiple hyperdontia: Report of an unusual case Marilia Nalon Pereira, a Luiz Eduardo de Almeida, b Marcelo Tarcısio Martins, c Marcio Jose da Silva Campos, d Marcelo Reis Fraga, e and Robert Willer Farinazzo Vitral f Juiz de Fora, Minas Gerais, Brazil Supernumerary teeth are an infrequent developmental anomaly that can appear in any area of the dental arch and can affect any dental organ. Multiple supernumerary teeth, or hyperdontia, is rare in people with no other associated diseases or syndromes. Conditions commonly associated with hyperdontia include cleft lip and palate, trichorhinophalangeal syndrome, cleidocranial dysplasia, and Gardner s syndrome. A black girl, aged 11 years 8 months, came for consultation; radiographs showed 81 teeth: 18 deciduous, 32 permanent, and 31 supernumerary. The main concern initially was to determine whether she was syndromic, and she was referred to a geneticist. G banding analysis showed pericentric inversion of chromosome 9; the chromosome formula was 46, XX, inv (9) (p13q21). Orthodontic treatment for this patient will be a clinical challenge because of the great number of teeth to be extracted and the alterations in the shapes of the teeth. Treatment goals should be established by a multidisciplinary team, where oral surgeon, orthodontist, periodontist, and prosthodontist come together to solve a medical and dental puzzle, eliminating the pieces that do not fit and searching for new ones to obtain an occlusion that will give the patient physiologic conditions of normality and esthetic satisfaction. (Am J Orthod Dentofacial Orthop 2011;140:580-4) Dental anomalies occur because of a variety of genetic and environmental factors. Combinations of dental anomalies are associated with specific syndromes. A few cases of multiple dental anomalies have been reported in patients with no generalized abnormalities. 1 The presence of supernumerary teeth, or hyperdontia, is an infrequent developmental alteration that appears in any area of the dental arches and can affect any dental organ. 2 The etiology of supernumerary teeth is not completely understood. There are various theories for the different types of supernumerary teeth. One theory suggests that a supernumerary tooth is created as a result of a dichotomy of the tooth bud. Another theory, well supported in the literature, is the hyperactivity theory, which suggests that supernumeraries are formed as From Juiz de Fora Federal University, Juiz de Fora, Minas Gerais, Brazil. a Associate professor, Department of Restorative Dentistry. b Postgraduate student, Department of Restorative Dentistry. c Postgraduate student, Department of Radiology. d Postgraduate student, Department of Orthodontics. e Professor, Department of Orthodontics. f Associate professor and chair, Department of Orthodontics. The authors report no commercial, proprietary, or financial interest in the products or companies described in this article. Reprint requests to: Robert Willer Farinazzo Vitral, Av Rio Branco 2595/1694, Juiz de Fora, MG, , Brazil; , robertvitral@acessa.com. Submitted, January 2010; revised and accepted, February /$36.00 Copyright Ó 2011 by the American Association of Orthodontists. doi: /j.ajodo a result of local, independent, and conditioned hyperactivity of the dental lamina. Heredity might also play a role in this anomaly. 3 Occurrence can be single or multiple, unilateral or bilateral, erupted or impacted, and in 1 jaw or both jaws. Multiple supernumerary teeth are rare in people with no other associated diseases or syndromes. The conditions commonly associated with an increased prevalence of supernumerary teeth include cleft lip and palate, trichorhinophalangeal syndrome, cleidocranial dysplasia, and Gardner s syndrome. 4,5 Although missing and supernumerary teeth are asymptomatic in most cases, they can lead to malocclusions, and esthetic, functional, and psychological problems. 6 The presence of supernumerary teeth is associated with different alterations in neighboring teeth; the most common are overretained teeth or delayed eruption, ectopic eruption, dental malposition, occlusal problems, diastemas, and rotated neighboring teeth, among a series of associated pathologies. 2 An early diagnosis prevents or reduces the risk of complications and, when combined with early removal, has a better prognosis. 7 Removal of a supernumerary tooth preventing permanent tooth eruption usually slloed the eruption of the tooth, if adequate space is available in the arch to accommodate it. 8 This article presents an unusual case of multiple hyperodontia in a girl aged 11 years 8 months with 31 supernumerary teeth. 580

2 Pereira et al 581 Fig 1. Intraoral photographs. Fig 2. Lateral cephalometric radiograph. Fig 3. Panoramic radiograph. CASE REPORT A black girl, aged 11 years 8 months, came for consultation for removal of root fragments of the maxillary deciduous incisors. During the clinical examination, it was noted that her only permanent teeth were the 4 first molars and the maxillary right first premolar. The following deciduous teeth were present in the oral cavity: 53, 55, 63 through 65, 71 through 74, and 81 through 84 (Fig 1). Panoramic and lateral cephalometric radiographs were requested, and they showed a significant clinical finding: multiple supernumerary teeth (Figs 2 and 3). Cone-beam computed tomography (CBCT) was ordered to evaluate 3-dimensionally the number and the correct positions of the supernumerary teeth. The CBCT images showed a total of 81 teeth: 18 deciduous, 32 permanent, and 31 supernumerary (Figs 4 and 5). The scans showed alterations of the forms of the teeth; this made it difficult to differentiate between the supernumerary and the other teeth. Cervical and thorax computed tomography images showed normal osseous structures, no expansive lesions, and intact muscular-adipose planes. During the medical history evaluation, it was found that the patient s mother was thalassemic; however, no alteration in her number of teeth was found. American Journal of Orthodontics and Dentofacial Orthopedics October 2011 Vol 140 Issue 4

3 582 Pereira et al Fig 4. CBCT right and left sagittal cuts. DISCUSSION Fig 5. CBCT frontal cut (panoramic). Because multiple supernumerary teeth are associated with diseases or syndromes, a thorough study should be conducted to establish a differential diagnosis in such patients. The presence of 31 supernumerary teeth caused suspicion. No case of a genetic syndrome in the family related to hyperdontia was reported in the patient s medical history. The fact that her mother was thalassemic did not seem to be relevant, because supernumerary teeth are not a characteristic of this type of anemia. Tooth crown sizes (mesiodistal and buccolingual diameters) of thalassemic male and female patients were significantly smaller than those of unaffected control subjects. 9 Beta-thalassemia major is associated with greater dental caries experience. 10 The conditions commonly associated with an increased prevalence of supernumerary teeth are cleft lip and palate, trichorhinophalangeal syndrome, cleidocranial dysplasia, and Gardner s syndrome. 4,5 During the clinical evaluation, it was noted that the patient did not have cleft lip and palate. Cleidocranial dysostosis is a rare congenital disorder of bone with an autosomal dominant hereditary mode of inheritance. This condition is characterized by clavicular aplasia or deficient formation of the clavicles, delayed and imperfect ossification of the cranium, moderately short stature, and a variety of other skeletal abnormalities. The oral manifestations are delayed exfoliation of deciduous teeth, delayed or failed eruption of the permanent dentition, and multiple supernumerary teeth. The most characteristic and pathognomonic feature of this disorder is hypoplasia or aplasia of the clavicles, which results in hypermobility of the shoulders, October 2011 Vol 140 Issue 4 American Journal of Orthodontics and Dentofacial Orthopedics

4 Pereira et al 583 Fig 7. CBCT sagittal cut, showing alteration in the frontal bone (arrow). Fig 6. CBCT 3-dimensional skull reconstruction. allowing the patient to approximate the shoulders in the midline. 11 Of the characteristics commonly found in patients with cleidocranial dysostosis, only oral manifestations were present. Clavicular disorders could not be verified. The CBCT images showed hyperostosis in the region of the frontal bone (Figs 6 and 7). The presence of intestinal polyposis is a marker for Gardner s syndrome. 12 Familial adenomatous polyposis is associated with a number of extraintestinal manifestations, which include osteomas, epidermoid cysts, and desmoid tumors, often referred to as Gardner s syndrome. 13 It is a phenotypic variant of familial adenomatous polyposis characterized by the classic clinical triad of skin and soft tissue tumors, osteomas, and intestinal polyposis, but disease patterns with pairs of these findings have also been reported. 12 We found no report associated with the characteristics of Gardner s syndrome in the patient s medical history. Trichorhinophalangeal syndrome is a rare genetic disease characterized by typical alterations such as a long bulbous nose, hair alterations, a long flat philtrum, 1-shaped epiphyses of the phalanges, and musculoskeletal alterations that at the first view might simulate juvenile idiopathic arthritis. These musculoskeletal deformities could imply the differential diagnosis with rheumatic diseases. 14 Other characteristics are slowly growing and sparse scalp hair, medially thick and laterally thin eyebrows, bulbous tip of the nose, long flat philtrum, thin upper lip with vermilion border, and protruding ears. In addition, those with trichorhinophalangeal syndrome generally share skeletal and bone anomalies, including shortening of the phalanges and metacarpals (mild to severe brachydactyly), coneshaped epiphyses, hip dysplasia, and short stature. 15 The available data at the clinical examination did not permit us to draw a conclusion about the diagnosis. When a syndrome is suspected, the collaboration of dentists with clinical geneticists is important for an early diagnosis. 11 Thus, the first decision was to refer the patient to a geneticist for proper diagnosis. The G banding differential staining was applied for the chromosome identification. The G banding analysis of 30 cells in metaphase, with approximately 400-band resolution, showed pericentric inversion of chromosome 9 at p13, q21. Thus, the chromosome formula of this patient was 46, XX, in(9) (p13 q21). Chromosomal inversions are detected in humans with increasing frequency because of the application of chromosomal banding American Journal of Orthodontics and Dentofacial Orthopedics October 2011 Vol 140 Issue 4

5 584 Pereira et al techniques. Inversions have occurred in all chromosomal groups. These structural aberrations can lead to infertility, reproductive loss, or abnormal offspring. 16 As for the patient s dental problems, prophylactic surgical removal of the supernumerary teeth is generally the treatment of choice. Therapy might include removal of supernumerary teeth, surgical exposure of impacted teeth, and orthodontic treatment. Orthodontic treatment is usually indicated to direct the eruption of the malposed and often impacted teeth. The treatment of this patient, however, is a challenge. With so many supernumerary teeth, dental extractions should be carefully scheduled not to jeopardize the osseous integrity of the maxilla and the mandible. There is no definite morphologic differentiation between supernumerary and permanent teeth. Most nonerupted teeth show alterations of form, making it impracticable to position them in the dental arches as part of the objectives of the orthodontic treatment. Treatment goals should be established by a multidisciplinary team, where oral surgeon, orthodontist, periodontist, and prosthodontist solve this medical and dental puzzle by eliminating the pieces that do not fit, and searching for new ones to obtain an occlusion that will give the patient normal physiologic conditions associated with esthetic satisfaction. We thank Dr Ricardo Villela Bastos, C^ortes Villela Clinical Analysis Laboratory, for the genetic diagnosis and Prof Ant^onio Augusto Falci Rodrigues for his help with this study. REFERENCES 1. Suprabha BS, Sumanth KN, Boaz K, George T. An unusual case of non-syndromic occurrence of multiple dental anomalies. Indian J Dent Res 2009;20: Dıaz A, Orozco J, Fonseca M. Multiple hyperodontia: report of a case with 17 supernumerary teeth with non syndromic association. Med Oral Patol Oral Cir Bucal 2009;14:E Garvey MT, Barry HJ, Blake M. Supernumerary teeth an overview of classification, diagnosis and management. J Can Dent Assoc 1999;65: Scheiner MA, Sampson WJ. Supernumerary teeth: a review of the literature and four case reports. Aust Dent J 1997;42: Yag ue-garcıa J, Berini-Aytes L, Gay-Escoda C. Multiple supernumerary teeth not associated with complex syndromes: a retrospective study. Med Oral Patol Oral Cir Bucal 2009;14:E Peker I, Kaya E, Darendeliler-Yaman S. Clinic and radiographical evaluation of non-syndromic hypodontia and hyperdontia in permanent dentition. Med Oral Patol Oral Cir Bucal 2009;14: E Yassin OM, Hamori E. Characteristics, clinical features and treatment of supernumerary teeth. J Clin Pediatr Dent 2009;33: Mitchell L, Bennett TG. Supernumerary teeth causing delayed eruption a retrospective study. Br J Orthod 1992;19: Hattab FN, Abu-Alhaija ESJ, Yassin OM. Tooth crown size of the permanent dentition in subjects with thalassemia major. Dent Anthropology 2000;14: Al-Wahadni AM, Taani DQ, Al-Omari MO. Dental diseases in subjects with beta-thalassemia major. Community Dent Oral Epidemiol 2002;30: Farronato G, Maspero C, Farronato D, Gioventu S. Orthodontic treatment in a patient with cleidocranial dysostosis. Angle Orthod 2009;79: Herrmann SM, Adler YD, Schmidt-Petersen K, Nicaud V, Morrison C, Paul M, et al. The concomitant occurrence of multiple epidermal cysts, osteomas and thyroid gland nodules is not diagnostic for Gardner syndrome in the absence of intestinal polyposis: a clinical and genetic report. Br J Dermatol 2003;149: Davies DR, Armstrong JG, Thakker N, Horner K, Guy SP, Clancy T, et al. Severe Gardner syndrome in families with mutations restricted to a specific region of the APC gene. Am J Hum Genet 1995;57: Freire de Carvalho J. Tricho-rhino-phalangeal syndrome: first Brazilian case. Acta Reumatol Port 2009;34: Hilton MJ, Sawyer JM, Gutierrez L, Hogart A, Kung TC, Wells DE. Analysis of novel and recurrent mutations responsible for the tricho-rhino-phalangeal syndromes. J Hum Genet 2002;47: Martin AO, Simpson JL, Deddish RB, Elias S. Clinical implications of chromosomal inversions. A pericentric inversion in no. 18 segregating in a family ascertained through an abnormal proband. Am J Perinatol 1983;1:81-8. October 2011 Vol 140 Issue 4 American Journal of Orthodontics and Dentofacial Orthopedics