Clinical and Surgical Management of an Aggressive Cherubism Treated with Autogenous Bone Graft and Calcitonin

Abstract

Cherubism is a rare autosomal-dominant inherited syndrome and is usually self-limiting; it starts in early childhood and involutes by puberty. It is a benign fibroosseous disease, characterized by excessive bone degradation of the upper and lower jaws followed by development of fibrous tissue masses. The purpose of this clinical report is to describe a rare and aggressive form of cherubism on an adult female patient that has been treated in our Bioscience Center for Special Health Care Needs-CEBAPE. The patient was firstly submitted to the surgical procedure with partial curettage of the lesion, and the cavity was filled with autogenous cancellous bone and bone marrow grafts. Furthermore, the support treatment used was the administration of salmon calcitonin by nasal spray during the first year after the preconized procedure. At 4-year followup, we confirmed the stomatognathic system improvement and esthetic rehabilitation, which led to a significant increase in the patient's quality of life.

Cherubism in 12 year young female

“…Before puberty, medical treatment by systemic calcitonin, tacrolimus had reported success due to inhibition of bone resorption. [910]…”

Innovative Surgical Treatment of Severe Cherubism

“…Careful evaluation of the aggressiveness of the cherubism prior to the intervention should be performed to assess for areas of bone weakness where the bone may be more susceptible to fractures and infection. It is recommended that only partial curettage is performed if possible since total removal can result in pathological fractures (Gross, 1997; Fernandes et al, 2011). In our case, we opted for a more aggressive surgical treatment with curettage, resection, recontour of both the maxilla and the mandible, and infracture of the mandible to address the contour deformity in addition to removal of lesions.…”

“…The management of cherubism varies considerably and must be individually determined for each patient. It ranges from conservative management to more radical surgery and is primarily dependent on the rate of progression of the lesion, likelihood of clinical regression, the potential for pathologic fracture, extent of the tissues involved, and the emotional state of the patient (Carvalho et al, 2007; Mortellaro et al, 2009; Fernandes et al, 2011; Hernandez-Alfaro et al, 2011; Lannon and Earley, 2011). Severe physiological dysfunctions (eg, respiration, vision, deglutition) and psychological distress due to serious disfigurement may necessitate surgical intervention (Mortellaro et al, 2009; Fernandes et al, 2011; Lannon and Earley, 2011; Kau et al, 2012).…”

Cherubism: A Case Report

“…Since the lesion undergoes regression, the surgical intervention is usually delayed until puberty. However, in patients with functional, cosmetic problems or emotional disturbances [23], surgical intervention consisting of debulking of lesions with surgical recontouring can be considered.…”