OLIVERI Maria Beatriz
congresos y reuniones científicas
Clinical and radiographic features observed in a case series of patients with fibrous displasia. Our experience.
MASTAGLIA SR; GONZALEZ D; BAGUR A; FRIGERI A; GOMEZ GLORIOSO , DOLORES; OLIVERI B
Congreso; 44 European Calcified Tissue International; 2017
Fibrous dysplasia (FD) is a benign bone disease characterized by the proliferation of fibroblasts and impaired differentiation of osteoblasts, which lead to replacement of normal bone with fibrous connective tissue. Objective: To analyze our data-base and perform a descriptive analysis of the observed clinical and radiographic features. Materials and Methods: The following data were retrieved from the clinical records: age at diagnosis, symptoms, clinical form [polyostotic (POT) or monostotic (MOT)], form of diagnosis, and treatment. Results: In the present cohort comprising 20 patients with FD (including patients from 2003), age at diagnosis was early (X±SD: 16.5±11 years; range: 5-37), and prevalence was higher among women (70%) than men (30%). The following symptoms were observed in 75%of patients: pain (35%), pathological fracture (15%); swelling/tumor (10%), and precocious puberty (15%) [as part of the McCune Albright syndrome (MAS]; FD was anincidental finding in the remaining 25%. Thirty percent of patients (30%) had MOT FD, and 70% had POT FD. The most frequently affected bone was the femur (35%), followed by craniofacial bones (30%) and the pelvis (20%). Diagnosis was established based on imaging techniques alone in 70% of cases, and presumptive diagnosis was confirmed by biopsy in 30% of cases. The most frequent treatment was bisphosphonate therapy (80%), and the regimen proposed by Chapurlat was the most used. Only patients with the POT form of the disease required surgical treatment (25%), and had renal tubular phosphate loss (5%). Discontinuation of medical follow up was higher in non-symptomatic patients as compared to symptomatic patients (severe pain and MAS). Conclusions: In the present cohort, the POT form of FD was the most severe, and was associated with severe pain, MAS, renal tubular phosphate loss, and need for surgical treatment.