An unusually extensive case of cherubism

Ramon, Yochanan; Engelberg, Isaac S.

doi:10.1016/0278-2391(86)90085-6

Cited by 47 publications

(27 citation statements)

References 7 publications

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“…It is clinically justified by the additional phase of rapid growth, the huge deformity of the mandibular body, and especially the bilateral posterolaterosuperior extension of maxillary lesions, with penetration into the retrobulbar areas of orbital cavities. Radiographically, it is justified by the perforation and disruption of wide areas of the bony cortex and marked root resorption, which has long been considered to be a more aggressive process 1,5,15,19,28 as was found in some cases presented in our current report. SILVA et al 24 reported an extreme case of cherubism that behaved in a locally aggressive manner.…”

Section: Discussionmentioning

confidence: 67%

“…SEWARD & HANKEY 23 suggested a grading system for cherubism, based on the radiographic location of the lesions in the jaws, which has been used in later studies and has been supplemented with several subdivisions in other studies 1,15,19 . RAMON & ENGELBERG 19 classified a case of cherubism with monstrous deformity of the jaws not restricted to elevation of the orbital floor but penetrating the orbit, as severity Grade IV.…”

Section: Discussionmentioning

confidence: 99%

“…RAMON & ENGELBERG 19 classified a case of cherubism with monstrous deformity of the jaws not restricted to elevation of the orbital floor but penetrating the orbit, as severity Grade IV. The same grade was also used by AYOUB & EL-MOFTY 1 in their case of massive extension involving the mandibular condyles.…”

Section: Discussionmentioning

confidence: 99%

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Clinicopathologic study of 24 cases of cherubism

Meng

2005

International Journal of Oral and Maxillofacial Surgery

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Section: Discussionmentioning

confidence: 67%

Section: Discussionmentioning

confidence: 99%

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Clinicopathologic study of 24 cases of cherubism

Meng

2005

International Journal of Oral and Maxillofacial Surgery

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“…5,10 Cherubism is reported to be associated with some well described syndromes, including neurofibromatosis type 1, Noonan-like/multiple giant cell lesion syndrome, Ramon syndrome, and JaffeCampanacci syndrome. 11,12 Ramon and Engelberg 13 have proposed a grading system for cherubism based on the area of involvement, as follows:…”

Section: Discussionmentioning

confidence: 99%

Cherubism, a rare childhood disease with a brief review of literature

Shah¹

2013

IOSR-JDMS

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“…12,20,21 We grade the severity of cherubism using a modified Motamedi system because it incorporates both the anatomic involvement and aggressiveness of the disease. 21 Motamedi originally divided the severity of cherubism into 5 grades.…”

Section: Grading Systemmentioning

confidence: 99%

Two-Stage Surgical Treatment of Severe Cherubism

Raposo-Amaral¹,

Guidi²,

Warren³

et al. 2007

Annals of Plastic Surgery

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Cherubism is an autosomal-dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration followed by fibrous tissue hyperplasia. Eight patients (age, 6-15 years; mean, 11 years) with severe cherubism were treated with a 2-stage operation by the same senior surgeon (C.M.R.-A.) over an 18-year period, 1987 through 2005. Severity was based on a modified Motamedi classification system. The diagnosis was established clinically, radiographically, and histologically. Postoperative follow up ranged from 2 to 18 years (mean, 5.1 years). All patients underwent 2-stage surgical treatment. In the first stage, the anterior wall of the maxilla was osteotomized and removed (n = 5). The cherubic lesion was curetted from the maxilla and orbits. The maxilla was recontoured on the back table and then fixed orthotopically with wires. Alternatively, cortical bone windows were created, the maxillary and orbital lesions were curetted, and the maxilla was infractured and recontoured in situ with manual pressure (n = 3). Six months after the maxillary/orbital procedure, all patients underwent second-stage curettage and recontouring of the mandible using bony access windows and manual infracture. Satisfactory esthetic results were achieved in all patients. The patients remained clinically and radiographically disease-free throughout the follow-up period. The most profoundly affected patient sustained bilateral lower eyelid ectropion that resolved with full-thickness skin grafting. Although cherubism tends to abate by the fourth decade of life, early 2-stage surgical curettage provides a simple and reliable treatment that not only delivers immediate results, but also seems to arrest the growth of any remaining cherubic tissue.

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An unusually extensive case of cherubism

Cited by 47 publications

References 7 publications

Clinicopathologic study of 24 cases of cherubism

Clinicopathologic study of 24 cases of cherubism

Cherubism, a rare childhood disease with a brief review of literature

Two-Stage Surgical Treatment of Severe Cherubism

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