Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome

Dalben, Gisele da Silva; Richieri-Costa, Antônio; Taveira, Luís Antônio de Assis

doi:10.1111/j.1601-0825.2008.01457.x

Cited by 12 publications

(13 citation statements)

References 17 publications

(33 reference statements)

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“…32,33 Furthermore, the occurrence of supernumerary and hypodontia in patients with MFS has recently been reported. 8 The association between the hypohyperdontia condition and syndromes is poorly recorded in the literature. 34 Similarly, several theories, including atavism, dichotomy, hyperactivity of the dental lamina, and the concept of multifactorial inheritance, have been proposed to explain the etiology of supernumerary teeth.…”

Section: Discussionmentioning

confidence: 99%

“…3 The term "concomitant hypohyperdontia" has been used to describe the presentation of hypodontia and hyperdontia in the same individual. 7,8 Thus, hypohyperdontia can be considered an extremely rare condition, with reported prevalence figures ranging from 0.002% to 3.1%. 6 Since then, the term "concomitant hypohyperdontia" has frequently been used to describe this phenomenon.…”

Section: Introductionmentioning

confidence: 99%

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Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case

Mallineni

Jayaraman

Yiu

et al. 2012

J of Invest & Clin Dent

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Marfan syndrome is an autosomal dominant genetic disorder of connective tissue origin that can affect multiple organs. Various oral manifestations have been associated with this syndrome. Hypohyperdontia is a condition of mixed numeric variations that presents a combination of hypodontia with hyperdontia within a single human dentition. The purpose of this paper is to report a case of Marfan syndrome with a concomitant occurrence of hypohyperdontia. A narrative review was also included to describe the other syndromes that had been associated with concomitant hypohyperdontia.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case

Mallineni

Jayaraman

Yiu

et al. 2012

J of Invest & Clin Dent

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“…Fifty percent of cases have been reported in G/BBB syndrome (15), and it is evident that syndromic hypohyperdontia is reported very rarely. A rare example of syndromic hypo-hyperdontia in the same arch (maxilla) was reported in a patient with G/BBB syndrome (15). Further case studies and research will be needed to clarify the association of hypo-hyperdontia with various syndromes.…”

Section: Discussionmentioning

confidence: 99%

“…The average age of the reported patients was 11.29 years. Based on occurrence, the bimaxillary type (65%) was the most commonly reported (2)(3)(4)(5)(6)13,16,17, followed by the maxillary (21%) type (1,3,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and mandibular (14%) type (3,16,(19)(20)(21)(22)(23)(24)(25)(26)(27), the results being statistically significant (P < 0.001) (Fig. 1).…”

Section: Resultsmentioning

confidence: 99%

“…Syndromes associated with hypohyperdontia have included Down syndrome (37,42), Dubowitz syndrome (31), Ellis-van Creveld syndrome (39,46), fucosidosis (32), G/BBB syndrome (15) Marfan syndrome (6), bilateral cleft lip and palate (4), and cleft palate and abnormalities of the cervical vertebrae (34). Fifty percent of cases have been reported in G/BBB syndrome (15), and it is evident that syndromic hypohyperdontia is reported very rarely. A rare example of syndromic hypo-hyperdontia in the same arch (maxilla) was reported in a patient with G/BBB syndrome (15).…”

Section: Discussionmentioning

confidence: 99%

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A comprehensive review of the literature and data analysis on hypo-hyperdontia

et al. 2014

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The objectives of this study were i) to conduct a comprehensive review of the literature on hypo-hyperdontia, and ii) to categorize the reported cases based on occurrence. An extensive search of the literature covering the period from January 1966 to January 2014 was conducted using the Embase, Google Scholar, Medline, and PubMed databases. The key words used in the search strategy were "concomitant", "agenesis", "hypodontia", "hyperdontia", "supernumerary teeth", "syndromes", and "hypohyperdontia" in various combinations. The retrieved data were analyzed based on gender, occurrence, and occurrence of both hypodontia and hyperdontia. Descriptive statistics were carried out using the chi-squared test. For hypo-hyperdontia overall, seven case studies and 40 cases involving 103 patients were reported. Our comprehensive review revealed that hypo-hyperdontia occurs most commonly in males (P < 0.05), and the bimaxillary type (65%) was reported most commonly in comparison with the maxillary, pre-maxillary and mandibular types (P < 0.001). Twenty syndromic cases and two case studies on familial occurrence of hypohyperdontia were reported. Almost 57% of cases affected the anterior region, whereas 43% of cases affected the anteroposterior region. The anterior region was most commonly affected by hyperdontia whereas the posterior region was most commonly affected by hypodontia. Hypo-hyperdontia is commonly seen in males, and the most common type is bimaxillary occurrence. G/BBB syndrome is commonly associated with hypo-hyperdontia, being relatively frequent in the anterior region, where mesiodens is frequently seen, and second premolars are the most commonly missing teeth. (J Oral Sci 56, 295-302, 2014)

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Syndromes with supernumerary teeth

Lubinsky

Kantaputra

2016

American J of Med Genetics Pt A

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While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein–Taybi syndrome; Nance–Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg–Pakistani syndrome (craniosynostosis and dental anomalies), and insulin‐resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann–Streiff syndrome, Fabry disease, Ehlers–Danlos syndrome, Apert and Crouzon syndromes, Zimmermann–Laband syndrome, and Ellis–van Creveld syndrome. © 2016 Wiley Periodicals, Inc.

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Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome

Abstract: The presence of mandibular anterior supernumerary teeth and ankyloglossia should be investigated in the clinical evaluation of patients with suspected diagnosis of the G/BBB syndrome.

Cited by 12 publications

References 17 publications

Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case

Concomitant occurrence of hypohyperdontia in a patient with Marfan syndrome: a review of the literature and report of a case

A comprehensive review of the literature and data analysis on hypo-hyperdontia

Syndromes with supernumerary teeth

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