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Aggressive form of cherubism.

Reddy G, Reddy GS, Reddy NS, Badam RK - J Clin Imaging Sci (2012)

Bottom Line: Cherubism is a rare non-neoplastic hereditary disease related to genetic mutations characterized by symmetrically swollen cheeks, particularly over the angles of the mandible, and an upward turning of the eyes.The affected mandible and maxilla begin to swell in early childhood, and gradually increase until the age of puberty.Apparently, surgical intervention is unnecessary unless significant functional, esthetic, or emotional disturbances develop.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral and Maxillofacial Surgery, Panineeya Institute of Dental Sciences, Hyderabad, Andhra Pradesh, India.

ABSTRACT
Cherubism is a rare non-neoplastic hereditary disease related to genetic mutations characterized by symmetrically swollen cheeks, particularly over the angles of the mandible, and an upward turning of the eyes. The affected mandible and maxilla begin to swell in early childhood, and gradually increase until the age of puberty. Apparently, surgical intervention is unnecessary unless significant functional, esthetic, or emotional disturbances develop. In the present paper, we report a case of cherubism, with classic features that was classified as grade 3, managed with surgery and followed up for 2-years after treatment.

No MeSH data available.


Related in: MedlinePlus

Surgical specimen showing decorticated and curetted material along with impacted teeth.
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Figure 5: Surgical specimen showing decorticated and curetted material along with impacted teeth.

Mentions: The grotesque appearance and the parent's insistence on esthetic improvement compelled the surgeon to attempt cosmetic surgical recontouring of the jaws under general anaesthesia. The entire mandible and maxilla was degloved intraorally. The exposed lesion tissue consisted of multiple locules with reddish hue and semi-hard consistency. Buccal surfaces of maxilla and mandible were decorticated and curetted as much as possible to provide optimal cosmetic improvement. Impacted and loose teeth were removed. Specimen was sent for final histopathological examination [Figure 5]. The diagnosis of cherubism was confirmed. Patient had no recurrence of the lesion during the 2-year follow-up period [Figure 6].


Aggressive form of cherubism.

Reddy G, Reddy GS, Reddy NS, Badam RK - J Clin Imaging Sci (2012)

Surgical specimen showing decorticated and curetted material along with impacted teeth.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3307211&req=5

Figure 5: Surgical specimen showing decorticated and curetted material along with impacted teeth.
Mentions: The grotesque appearance and the parent's insistence on esthetic improvement compelled the surgeon to attempt cosmetic surgical recontouring of the jaws under general anaesthesia. The entire mandible and maxilla was degloved intraorally. The exposed lesion tissue consisted of multiple locules with reddish hue and semi-hard consistency. Buccal surfaces of maxilla and mandible were decorticated and curetted as much as possible to provide optimal cosmetic improvement. Impacted and loose teeth were removed. Specimen was sent for final histopathological examination [Figure 5]. The diagnosis of cherubism was confirmed. Patient had no recurrence of the lesion during the 2-year follow-up period [Figure 6].

Bottom Line: Cherubism is a rare non-neoplastic hereditary disease related to genetic mutations characterized by symmetrically swollen cheeks, particularly over the angles of the mandible, and an upward turning of the eyes.The affected mandible and maxilla begin to swell in early childhood, and gradually increase until the age of puberty.Apparently, surgical intervention is unnecessary unless significant functional, esthetic, or emotional disturbances develop.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral and Maxillofacial Surgery, Panineeya Institute of Dental Sciences, Hyderabad, Andhra Pradesh, India.

ABSTRACT
Cherubism is a rare non-neoplastic hereditary disease related to genetic mutations characterized by symmetrically swollen cheeks, particularly over the angles of the mandible, and an upward turning of the eyes. The affected mandible and maxilla begin to swell in early childhood, and gradually increase until the age of puberty. Apparently, surgical intervention is unnecessary unless significant functional, esthetic, or emotional disturbances develop. In the present paper, we report a case of cherubism, with classic features that was classified as grade 3, managed with surgery and followed up for 2-years after treatment.

No MeSH data available.


Related in: MedlinePlus