CHERUBISM – CLINICAL MANIFESTATIONS, DIAGNOSIS & TREATMENT
Imaging Characteristics of Cherubism, American Journal of Radiology, 182: 1051-1054, 2004, Beaman, Francesca; Bancroft, Laura W.; Peterson, Jeffrey J.; Kransdorf, Mark J.; Murphey, Mark D.; Menke, David M.
The authors compare a variety of imaging techniques used for the purpose of diagnosing cherubism. Key findings include:
- “CT clearly depicted bilateral osseous involvement and expansive remodeling.”
- “CT also accurately delineated the extent of involvement and was especially useful in characterizing osseous matrix.”
- “MRI provides an anatomic outline that allows accurate determination of lesion extent and location.”
- CT results “provided clear evidence of lesion extension and involvement in the mandibular condyle” (not previously detectable through panorex)
The results suggest using multiple imaging technologies before starting invasive treatment.
Cherubism: Clinopathological Features, Skeletal Radiology 28: 350-353, 1999, Yamaguchi, Dorfman & Eisig.
The authors of this article examine and describe the microscopic features of bone lesions associated with cherubism. They document cherubism’s histological (cellular) similarity to giant-cell reparative granuloma and histological difference from fibrous dysplasia. The article has good diagnostic images of cherubism, including a panoramic radiograph of the jaws, CT scan of the jaws, and photomicrographs of cells in cherubic lesions.
Radiographic, CT and MRI Features of Cherubism. Pediatric Radiology 36: 1099-1104, 2006, Jain, Vaibhav and Sharma, Raju.
This article provides a detailed description of the characteristics of radiographic, MRI and CT images of cherubism found in a 5 year old Indian girl. The article includes numerous images from all three sources. The authors report that MRI images were particularly useful for “elucidating the exact anatomical location and extent of the cherubism lesions, particularly their relationship to the orbits and the optic nerves.” The MRI also showed involvement in the condoyle of the mandible, which “was not apparent on radiographs or CT images.”
Cherubism: A Clinical, Radiographic, and Histopathological Comparison of 7 Cases, Journal of Oral and Maxillofacial Surgeons 64: 924-930, 2006, Penarrocha, Miguel; Minguez, Juan; Bagan, Jose Vincente; Minguez, Ignacio
The authors followed 7 cherubism patients of various ages for an average of 8.5 years. For each, they characterized histopathological parameters as absent, few, moderate or marked. These parameters included the density of giant multinucleated cells, interstitial hemorage, inflammatory activity, and the density of fibrous tissue. In addition, the authors assessed the progression of the disease (improved, no change, modest progression, marked progression). The authors argue for a conservative approach to treatment: “surgical treatment appears to be unnecessary for grade 1 and 2 cases [involvement of only the mandibular ascending rami or the mandibular ascending rami and involvement of the maxillary tuberosities] in the absence of secondary disturbances,” but it may be necessary in aggressive cases. Their recommendation is curettage with remodeling of the cortical layer as others have found bone grafts may result in spreading of the disease. The article provides good radiographic and photographic images showing the progression of the disease.
Treatment of Cherubism with Locally Aggressive Behavior Presenting in Adulthood: Report of Four Cass and a Proposed New Grading System Journal of Oral and Maxilliofacial Surgeons 56:1336-1342, 1998, Motamedi, Mohammad Hosein Kalantar.
The author describes 4 cases of cherubism in an Iranian family, in which onset was late in 3 (patients were in their 20s) and typical in the fourth. The author noted the inability to classify the patients according to Arnott’s (1979) grading scale and proposed an alternative: Grades I –V specify the location of lesions in the mandible and maxilla and the extent of root resorption caused by the lesions. Classes 1-5 within the grades specify whether the lesions are solitary or multiple and the location of the lesions within the affected bones. Subclasses A-D designate the “mandibular involvement within anyone of the 4 grades.” The author argues that it is critical to recognize the impact that root resorption has on permanent dentition in patients. They emphasize as well the need to test relatives of patients who receive clinical diagnoses of cherubism, given its hereditary nature. This article presents a framework for discussing the variability of penetrance of the disease in individuals sharing the same genetic mutation.
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Study of the Cell Biology and Biochemistry of Cherubism, Journal of Clinical Pathology 51: 831-837,1998, Southgate, John; Sarma, Usha; Townend, John V. and Flanagan, Adrianne M.
The authors study 4 English children of two brothers known to be cherubs: three had aggressive cherubism affecting the maxilla and mandible and the fourth had minimal radiographic evidence. The article provides detailed discussion of the methodology and results of invitro bone resorption studies, cytogenetic studies and biochemical analysis. The authors also gave calcitonin to the three severely affected children and discussed the feasibility of using this treatment to halt the progression of lesions. These results were inconclusive.
Cherubism in Siblings: A Case Report, Journal of the Canadian Dental Association 69 (3): 150-154, 2003, Ongole, Ravikiran; Pillai, Rajeev S.; Pai, Keerthilatha M.
This article provides a good overview of the state of clinical, genetic and histopathological knowledge about cherubism. It also briefly outlines the case of 2 Indian siblings with the condition.
Temporal Bone Involvement in Cherubism, Brazilian Dental Journal 15 (1), 2004, Fonseca, Luciana Cardoso; Freitas, Joa batista de; Maciel, Pedro Hernane; Cavalcanti, Marcelo Gusmao Paraiso.
The authors document how CT scans can detect maxillary lesions of cherubism that do not appear as radiolucent regions in panoramic radiography. They also discuss the presence of maxillary lesions found in the temporal bone of a 10 year old Brazilian girl, a region of the maxilla previously undocumented in published literature.
Clinicopathologcal Study of 24 Cases of Cherubism, International Journal of Oral Maxillofacial Surgery 34: 350-356, 2005, Meng, W. M. ; Yu, S. F, and Yu, G. Y.
This article reviews the nature of radiographic, histopathological and biochemical results for 24 cases of familial and non-familial cherubism in Chinese nationals with various ages of onset. The authors review the various grading systems developed to classify cherubism and the importance of separating aggressive fast growing lesions from others. Based on what is known of the natural course of the disease, the authors recommend conservative treatment: “curettage of the affected tissue, preserving the teeth as long as possible” and surgical recontouring when necessary.