Two-Stage Surgical Treatment of Severe Cherubism

Abstract

Cherubism is an autosomal-dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration followed by fibrous tissue hyperplasia. Eight patients (age, 6-15 years; mean, 11 years) with severe cherubism were treated with a 2-stage operation by the same senior surgeon (C.M.R.-A.) over an 18-year period, 1987 through 2005. Severity was based on a modified Motamedi classification system. The diagnosis was established clinically, radiographically, and histologically. Postoperative follow up ranged from 2 to 18 years (mean, 5.1 years). All patients underwent 2-stage surgical treatment. In the first stage, the anterior wall of the maxilla was osteotomized and removed (n = 5). The cherubic lesion was curetted from the maxilla and orbits. The maxilla was recontoured on the back table and then fixed orthotopically with wires. Alternatively, cortical bone windows were created, the maxillary and orbital lesions were curetted, and the maxilla was infractured and recontoured in situ with manual pressure (n = 3). Six months after the maxillary/orbital procedure, all patients underwent second-stage curettage and recontouring of the mandible using bony access windows and manual infracture. Satisfactory esthetic results were achieved in all patients. The patients remained clinically and radiographically disease-free throughout the follow-up period. The most profoundly affected patient sustained bilateral lower eyelid ectropion that resolved with full-thickness skin grafting. Although cherubism tends to abate by the fourth decade of life, early 2-stage surgical curettage provides a simple and reliable treatment that not only delivers immediate results, but also seems to arrest the growth of any remaining cherubic tissue.

Aggressive case of postpubertal cherubism: A case report

“…Curettage was the dominating procedure in reports based on larger clinical studie [8,17]. Cherubism is known to be a hemorrhagic lesion, Raposo-Amaral et al [6] reported an extensive resection was performed in two stages to prevent excessive blood loss in 8 children age 6 to 15 years old with severe cherubism. Henry.F et al…”

“…Usually The first signs of the disease appear between 1 and 3 years of age [4]. Motamedi [5] proposed a grading system for cherubism in 1998 which was modified by Raposo-Amaral et al [6] in 2007 (Table1). The authors in this article are reporting a case of a 23-year-old male patient with bilateral swelling of the lower jaw which was diagnosed with cherubism grade I class 5.…”

Comprehensive surgical management of cherubism with orbital involvement

“…It is a self-limited disease, and some cases of spontaneous regression have been reported. Approximately 250 cases of cherubism have been reported, but, to the best of our knowledge, only 11 papers describe an orbital involvement [1][2][3][4][5][6][7][8][9][10][11] and only 4 [12][13][14][15] on rehabilitation with dental implants. * Corresponding author at: Ctra Colmenar km.…”

Chérubisme : présentation d’un cas clinique suivi pendant 10 ans et revue de littérature

“…On retrouve au niveau odontologique des anomalies de nombre, de forme, d'érup-tion, des résorptions radiculaires. La classification de Motamedi, modifiée par Raposo-Amaral, établit la gravité des atteintes [7][8]. L'évolution histologique de la maladie a été décrite par Chomette [9] en trois phases : phase ostéolytique pendant l'enfance, phase de réparation conjonctive à la puberté, puis réparation osseuse.…”