Aggressive case of cherubism: 17-year follow-up

Pontes, Flávia Sirotheau Corrêa; Ferreira, A.G.M.; Kato, Alberto Mitsuyuki de Brito; Pontes, Hélder Antônio Rebelo; Almeida, Diele S.; Rodini, Camila Oliveira; Pinto, Décio dos Santos

doi:10.1016/j.ijporl.2007.01.017

Cited by 31 publications

(33 citation statements)

References 16 publications

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“…1 a Shows clinical appearance of the bilateral swelling. b and c, shows gingival hypertrophy without defined interdental papilla missing second and third molars, and cervical lymphadenopathy [1,2,4]. These features are required for a diagnosis of cherubism in conjunction with its characteristic radiographic features-see below.…”

Section: Discussionmentioning

confidence: 98%

“…Studies involving long term follow-up of patients with cherubism show contradictory results. In some cases remission of the disease without any treatment was achieved while it may be aggressive in other instance [4,38]. In view of the reported regression of the size of the swelling as narrated by the patients' parents, it was decided to observe the patient until therapeutic intervention is required.…”

Section: Discussionmentioning

confidence: 99%

“…Cherubism is a non-neoplastic, hereditary, jaw disease of the childhood characterized by painless, bilateral symmetrical enlargements [1][2][3][4][5]. The name cherubism was used to describe active and intelligent children with normal interests and social curiosity but their facial profile was characterized by a rounded appearance with an upward gaze [1].…”

Section: Introductionmentioning

confidence: 99%

“…Transmission may either be of maternal or paternal in origin, however, the disease manifest early in the former mode of transmission [5]. Usually the disease is diagnosed between 6 and 10 years of age and progresses until puberty, then the lesion demonstrate arrest and often regress, leading to a normal adult appearance in most patients [2][3][4][5]. Cherubism have been described in association with other genetic disorders [7].…”

Section: Introductionmentioning

confidence: 99%

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Cherubism Combined with Epilepsy, Mental Retardation and Gingival Fibromatosis (Ramon Syndrome): A Case Report

Suhanya

Aggarwal

Mohideen

et al. 2009

Head and Neck Pathol

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Cherubism is an inherited, autosomal dominant disorder that characteristically affects the jaws of children. The disease typically manifest as a bilateral swelling with associated submandibular lymph node enlargements and usually regresses as age advances. The disease is microscopically indistinguishable from other giant cell lesions and is essentially a clinical diagnosis. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred as Ramon syndrome. We report a case of Ramon syndrome in an 8 year old girl.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Cherubism Combined with Epilepsy, Mental Retardation and Gingival Fibromatosis (Ramon Syndrome): A Case Report

Suhanya

Aggarwal

Mohideen

et al. 2009

Head and Neck Pathol

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“…Radiotherapy is contraindicated because of the high incidence of complications such as the formation of fibrosarcoma and osteoradionecrosis in the irradiated area. 32 Orthodontic treatment is considered appropriate after growth is completed and after the regression of the disease. 2 However, Kau et al performed a successful orthodontic treatment during the disease process to correct the malocclusion and reposition an impacted molar in a 15-year-old girl.…”

Section: Discussionmentioning

confidence: 99%