Symptomatic Fibrous Dysplasia Research Articles

Response to Zoledronic Acid Infusion in Children With Fibrous Dysplasia.

Objective: This retrospective study evaluated the outcome and safety of long-term treatment with zoledronic acid, in both polyostotic and mono-ostotic fibrous dysplasia (FD) of children.Methods: The case records of children and adolescents with symptomatic FD who received zoledronic acid (0.1 mg/kg IV infusion over 1 h) and have completed at least 2 years follow-up were analyzed. The relevant details were recorded in a predesigned chart. Clinical assessment [pain assessment by visual analog scale (VAS) and incidence of new fracture], radiological changes (cortical thickening, ossification, and decrease in the diameter of the osteolytic lesions) and biochemical parameters [alkaline phosphatase (ALP)] were used to evaluate the improvement.Results: The mean age of presentation was 9.1 years, with four males and six females. All patients had symptomatic FD in the lower limb with complaints of pain, tenderness, swelling, or deformity. Four children had associated pathological fracture. The radiological evaluation with bone scan revealed polyostotic involvement in eight patients and mono-ostotic involvement in two patients. Three patients had associated systemic features like café-au-lait spots or precocious puberty. The fracture united within 3 months and the radiological improvement was evident in the form of filling of the osteolytic defect. The pain score in six patients showed significant improvement (VAS < 3). The ALP level decreased to 544.12 ± 47.35 IU/L from an initial value of 895.75 ± 79.64 IU/L (p = 0.04) at 12 months. One patient had symptomatic hypocalcaemia after zoledronic acid infusion.Conclusion: The clinical and radiological response of zoledronic acid treatment in FD of children is promising. Further randomized control trials with a larger sample size are required to establish this drug as a first-line medical treatment in FD.

Conservative treatment of Fibrous Dysplasia

Fibrous Dysplasia (FD) is a developmental disorder of bone that can affect one bone (monostotic type) or multiple bones (polyostotic type). The disease can be associated with hyperpigmentation and endocrinological disorders. In general, FD is found in teenagers, in the second decade of life. It often involves the long bones, craniofacial bones, ribs and pelvis. Approximately 30% of monostotic FD (MFD) lesions are found in the cranial, rather facial bones. It frequently appears in the posterior region of the jaw bone and is usually unilateral. The etiology of FD is not clear but even so genetic predisposition is suspected. The diagnosis is based on radiological and histopathological examination.We present an unusual case of symptomatic FD. A 15-year-old female patient was admitted to the clinic, complaining gradually increased swelling on her left side of the mandible one year ego with severe and unusual pain at related region. The bony enlargement was extending from median mandible to the crestal marginal level. Surgical shaving and recountering of mandible through the delicate preservation of the mental nerve in the left side of mandible was carried out.The aim of this report is to present the conservative treatment as sufficient treatment modality for the treatment of FD during puberty.

Fibrous dysplasia of the frontal sinus: a case report

Fibrous dysplasia is a rare and indolent benign tumor of bone. Although it is a benign tumor, it has malignant transformation potential. Fibrous dysplasia can involve a single or multiple bones. The maxilla and mandible are the most common sites in the head and neck region. Paranasal sinus involvement is rare. Surgical excision is the preferred treatment of patients with symptomatic fibrous dysplasia. In this article, a 54-year-old male patient diagnosed with fibrous dysplasia of the frontal bone is presented.

Fibrous dysplasia of the sinuses and orbit: Surgical techniques

Symptomatic fibrous dysplasia of the orbits and paranasal sinuses causing deformity or functional impairment is best treated with total or near total resection and reconstruction. Modern craniofacial and skull base surgical techniques permit this approach with relatively little patient morbidity. Autogenous split cranial bone grafts are optimal for reconstruction with allogenic cortical bone and hydroxyapatite cement providing additional contouring materials. If computed tomography imaging reveals optic canal encroachment and ophthalmologic evaluation shows visual disturbance from fibrous dysplasia involvement, then 3-wall optic nerve decompression should be performed concurrently with resection of the dysplasia. Careful and long-term follow-up of these young patients is essential for documenting the advantageous outcome of these treatment approaches.