[Familial fibrous dysplasia: a symptom of a syndrome or simply of cherubism?].

INTRODUCTION

Classified among non-neoplastic bone diseases, familial fibrous dysplasia is characterized by unilateral or bilateral change of shape and size in the facial part of the skull causing transposition of teeth. The aim of this study was to present two related patients with these symptoms who appeared for orthodontic consultation.

MATERIALS AND

METHODS

The study was done in 2 related patients, C.A. aged 58 and her daughter aged 25, basing on the medical history, clinical and radiologic examination, and documentation supplied by the patients.

RESULTS

Extraoral clinical examination of the mother revealed structural anomalies of the mandible and maxilla in the form of asymmetry caused by a tumor localized in the alveolar process of the mandible predominantly on the left side, in the maxillary bone, and in other bones of the skeleton. Intraoral examination disclosed missing teeth, small and atypical teeth, diastemata, carious and noncarious lesions in the form of cuneiform defects, pathologic attrition, and alveolar swelling with an extensive tumor situated in the region of missing teeth 34 and 35. Panoramic radiographs revealed hyperdense foci and multiple cyst-like radiolucencies corresponding to fibrous dysplasia and located bilaterally in the maxilla and mandible. The daughter presented with hirsutism and acne rosacea. Her radiograms revealed lesions resembling periapical periodontitis corresponding to Noonan-like multiple giant-cell lesion syndrome and Gorlin and Ramon syndrome.

DISCUSSION: Adult patients with non-neoplastic fibro-osseous lesions accompanied by malocclusion and transposition of teeth often seek help of the orthodontist. The age of our patients (mother 58 years and daughter 25 years) not being typical for progressing cherubism, as well as radiologic findings and other systemic disorders suggest that the lesions may be due to an undiagnosed systemic disease in which familial fibrous dysplasia is one of the symptoms.