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Page 4 of 5 Garlick et al. Plast Aesthet Res 2018;5:29 I http://dx.doi.org/10.20517/2347-9264.2018.36

The patient’s left maxilla had a significant deformity as well. Using a left upper buccal sulcus incision, the
tumor was identified eroding through the anterior wall of the maxillary sinus and protruding into the soft
tissue. The tumor was removed in similar piecemeal fashion by curettage. There were no intra-operative
complications.

The postoperative course was uncomplicated and at 6, 8, and 12 months follow-ups, the patient had
significant improvement in facial contour with a normal outward appearance and much improved
occlusion [Figure 1D-F]. Repeat CT scan examination showed normalization of the mandibular anatomy
and significant ossification around the molars providing dental stability at the site of the mandibular bone
repositioning [Figure 2C and D].

DISCUSSION
Cherubism is a rare disease with just over 300 reported cases in the literature documenting the disease
process and management. However, there is no consensus in regards to treatment guidelines. The variability
of presentation makes it difficult to establish a “one size fits all” treatment modality. There is little argument
that patients who present with minimal involvement should be followed on a regular basis, and those with
severe disfigurement should be strongly considered for surgery. Yet, there is no accepted approach for the
majority of patients who fall into the “grey zone” between these two extremes of presentations, as was our
patient’s case.

From a psychological standpoint, early surgical intervention can have a very positive impact by preventing
social ridicule and promote acceptance in the child’s formative developmental years . Furthermore, early
[7]
tumor removal in the disease process, as advocated by the current report, could prevent any long-term
sequelae requiring significantly more complex reconstructive surgery. Additionally, it has also been shown
that early operation, with curettage during the growth phase can arrest the tumor growth, and not prompt
rapid regrowth, thereby making it a favorable option in preventing further bony deformities [7,13,15,16] . Our
report further strengthens this group of patients who have good outcomes with arrested tumor growth
and excellent facial contouring following early surgical intervention. With careful review of the literature
it becomes apparent that the majority of patients within the “grey-zone” who opted for observation are at
high risk of suffering complete tooth loss in their late twenties and early thirties and additionally require
eventual surgical interventions or at a minimum extensive dental work . In our view, this negates the
[7]
argument that a “wait and watch” approach evades any eventual surgery. We should mention that we did
not find any published review of these cases that looked at the number of these patients which require
surgical interventions later following observation alone, but we feel this knowledge would be valuable for
future investigation.

Surgical treatment modalities used in the management of patients with cherubism range from tooth
extractions in the lesioned areas , orthodontics, fixed and removable prosthetic implants , osteoplastic
[16]
[17]
surgery to intraosseous curettage of lesions and bone grafting . Taking advantage of the qualities of the
[13]
[13]
Piezo Electric bone cutter, our surgical approach was achieved through minimal soft tissue disruption, but
allowed for aggressive tumor removal, as well as mandibular cortical repositioning. Our technique restored
the normal anatomy of the mandible without damage to the deciduous teeth, the permanent teeth, or the
buccal soft tissue.

We hope that the current report will aid surgeons who manage cherubism patients discern more clearly the
reasons why early surgical intervention should be considered and add the minimally invasive technique
presented herein to their surgical armamentarium.