Bisphosphonate treatment in craniofacial fibrous dysplasia—a case report and review of the literature

Abstract

Fibrous dysplasia (FD) is a benign skeletal disorder which may affect one or multiple bones. Lesions often involve long bones, ribs, and craniofacial bones and cause pain, fractures, and disfigurement. We describe an adolescent girl with mandibular FD who was successfully treated with bisphosphonates. She presented with a tumor-like lesion of the mandible. During the 2-year follow-up, the lesion expanded and caused significant disfigurement and pain necessitating psychiatric support. Treatment with bisphosphonate administered intravenously resulted in rapid pain relief, normalization of bone turnover, and cosmetic improvement. Management of FD has previously consisted of either conservative follow-up or surgery depending on disease activity and localization. Based on published reports and our experience, bisphosphonates should be considered in the treatment of symptomatic cases. This line of treatment may be suitable especially for craniofacial FD in which surgical treatment is particularly challenging.

Outcome of Long-Term Bisphosphonate Therapy in McCune-Albright Syndrome and Polyostotic Fibrous Dysplasia

“…(52) A last concern is the potential lack or curtailed skeletal uptake in predominantly sclerotic lesions such as those observed in craniofacial FD, but beneficial effects of these agents have also been reported particularly in isolated craniofacial (CF) FD. (53)(54)(55) In our study, treatment with bisphosphonates was well tolerated in adults as well as in children except for one young MAS patient, and only mild side effects were documented despite the high cumulative dose required to achieve an effect in the more severely affected cases of FD described here. We believe our specific individually tailored treatment protocol, based on maintaining a normal bone turnover status, was probably instrumental in the prevention of potential complications associated with the uninterrupted long-term use of these agents such as atypical fractures and osteonecrosis of the jaw.…”

“…A third concern is the use of these agents in the presence of FGF‐23–induced renal phosphate wasting, a relatively common finding, although in variable degrees, in the more severe cases of PFD and MAS, because of the potentially associated disturbance in mineralization . A last concern is the potential lack or curtailed skeletal uptake in predominantly sclerotic lesions such as those observed in craniofacial FD, but beneficial effects of these agents have also been reported particularly in isolated craniofacial (CF) FD …”

Craniofacial Fibrous Dysplasia Involving the Orbit

“…In addition, evidence is scarce regarding the long-term skeletal implications of childhood bisphosphonate use, especially for females in whom the medication stays in the skeleton until future pregnancies. 24 Steroids have also been reported as a successful temporary therapy for acute visual loss due to optic canal involvement. However, data are lacking for long-term reversal of visual loss without surgery.…”

Molecular, Phenotypic Aspects and Therapeutic Horizons of Rare Genetic Bone Disorders

“…A current approach that aims at both strengthening bone and reducing pain is bisphosphonate therapy. Mäkitie et al [10] administered bisphosphonates intravenously in a patient with mandibular FD. The therapeutic approach resulted in rapid reduction of pain, stabilized turnover of bone, and even proved to be cosmetically beneficial.…”