Familial Multilocular Cystic Disease of the Jaws

Jones, William A.

doi:10.1158/ajc.1933.946

Cited by 232 publications

(111 citation statements)

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“…5 Cherubism is a rare benign fibro-osseous disease of the jaws. 6 Affected children usually present before five years of age with painless progressive swelling of the cheeks, frequently associated with dental malformations.…”

Section: Discussionmentioning

confidence: 99%

Ramon's Syndrome: A Rare Entity

Lnu¹,

Peter²,

Nampoothiri³

et al. 2010

World Journal of Dentistry

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Introduction Ramon et al in 1967 described a condition, which included mental retardation, fibrous dysplasia of the maxilla and stunted growth. De Pino et al described a Brazilian family of four who had the same features as that of Ramon's syndrome in association with juvenile arthritis. Cherubism was first described in 1933 by Jones as ‘familial multilocular cystic lesion of the jaws’, a rare benign fibroosseous disease of the jaws, which is transmitted as an autosomal dominant trait. Affected children usually present before five years of age with painless progressive swelling of the cheeks, frequently associated with dental malformations. It progresses until puberty, and shows partial or complete spontaneous involution in adulthood; therefore, management is mostly conservative. The condition was initially characterized as familial, particularly as a form of craniofacial fibrous dysplasia. The children affected with cherubism do not usually show mental or physical deformities, but when cherubism is associated with other syndromes like Noonan-like syndrome, Ramon syndrome, and Fragile X syndrome, mental and physical deformities may be seen. Case Report This is a case report of a 12-year-old boy who reported with a massive painless bilateral swelling of the face, which has been increasing since the age of 2 years. The patient had multiple unerupted teeth, gingival hyperplasia, hearing loss and mental retardation. A detailed case report, including the histopathology, radiographic features (extraoral, CT and MDCT), and management of the case will be discussed in detail.

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Section: Discussionmentioning

confidence: 99%

Ramon's Syndrome: A Rare Entity

Lnu¹,

Peter²,

Nampoothiri³

et al. 2010

World Journal of Dentistry

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“…It was first described as "familial multilocular cystic disease of the jaws" by William Jones in 1933 [1] . The name "cherubism" later became standard nomenclature used to describe the condition due to the marked fullness of the cheeks and jaws with a slight upward tilting of the eyes, resembling cherubs from Renaissance paintings [2,3] .…”

Section: Introductionmentioning

confidence: 99%

Cherubism in a 4-year-old boy managed with tumor curettage, mandibular osteotomies and repositioning

Garlick¹,

Willis²,

Donato³

et al. 2018

PAR

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Cherubism is a rare disease characterized by significant loss of medullary bone which is replaced by excessive amounts of fibrous tissue growth within the mandible and maxilla. We present a case of a 4-year-old boy with a rapidly enlarging mandible and maxilla, causing significant change in the facial contour, malocclusion and phonation difficulties. He was treated with aggressive tumor curettage, lateral mandibular cortex osteotomies with medial repositioning. This allowed obliteration of the enlarged medullary space and restoration of the normal mandibular anatomy. At 12 months postoperatively, the patient had significant improvement in facial contour, normal outward appearance, and stable dentition.

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“…William a. Jones 1933, a Canadian radiologist described the condition first as familial multilocular cystic disease of the jaws in three Jewish siblings [1]. The upward gaze or 'eyes to heaven' results from lower lid retraction in relation to the diffuse enlargement of the lower half of the face or to an upward displacement of the eye from a mass involving the orbital floor [2].…”

Section: Introductionmentioning

confidence: 99%

Aggressive case of postpubertal cherubism: A case report

Aljumah¹,

Nasser²,

Alomar³

et al. 2017

Dent Oral Craniofac Res

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Cherubism is a non-neoplastic fibro-osseous lesion that is diagnosed among children based on its characterization by bilateral painless enlargement of jaws that offers a cherubic appearance to the affected persons. Cherubism treatment is combative given that it is believed that the lesion regress at puberty but cases with severe facial disfiguration can be corrected surgically. This case reports a 23-year-old male reported to the Department of Oral and Maxillofacial surgery at National Guard Health Affairs, King Abdulaziz Medical City, Riyadh, Saudi Arabia by his mother with chief complaint of asymptomatic, progressive and bilateral enlargement of the mandible. Based on facts and the clinical, historical, family and radio-graphical findings this report prepared a cherubism diagnosis of the patient. The purpose of this case report is to present the unusual postpubertal cherubism.

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Familial Multilocular Cystic Disease of the Jaws

Cited by 232 publications

References 0 publications

Ramon's Syndrome: A Rare Entity

Ramon's Syndrome: A Rare Entity

Cherubism in a 4-year-old boy managed with tumor curettage, mandibular osteotomies and repositioning

Aggressive case of postpubertal cherubism: A case report

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