PubMed · 13859786
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H E AIKEN. 1962. Cherubism.. https://doi.org/10.1001/archpedi.1962.02080020712011
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Many cases have been published on cherubism since Jones described it first time in three children of the same family [Am. J. Cancer 17 (1933) 946]. Cherubism is an autosomal-dominant disorder in which the normal bone is replaced by cellular fibrous tissue and immature bone. Extracranial skeletal involvement is rarely seen in hereditary and non-hereditary forms of the disorder. The mandible is the most severely affected craniofacial component, in which uncontrolled growth of the malady deteriorates the aesthetic balance of the face. Bilateral swelling of the cheeks, mandibular enlargement and maxillary spongious hypertrophy cause orbital manifestations and tendency of eyes looking up to the sky. Thus, the pathognomic clinical feature resembles the appearance of "raised to heaven" Renaissance cherubs. The sporadic case concerns a child affected by cherubism. Radiographic and clinical data of the patient are presented and brief review of the literature is included.
Cherubism is a benign, hereditary, autosomal dominant disease, with variable penetrance and expressivity. It presents as a characteristic indolent deformity of the lower half of the face, associated with multicystic bone tumors. The definitive diagnosis is established by pathology. Radiology contributes greatly to its diagnosis (CT or MRI scan), and preoperative arterial embolization can provide valuable help to the surgeon when excision of this hemorrhagic lesion is necessary. The present article presents a case showing the advantage of this type of devascularization prior to surgery, not previously described in the literature.