Fibrous Dysplasia FD of bone is a rare non-inheritable congenital disease characterized by a focal proliferation of fibrous tissue in the bone marrow leading to osteolytic lesions deformities and fractures. FD can be presented in a monostotic or polyostotic form this last one is usually more severe. FD primarily affects adolescents and young adults and in some cases in an asymptomatic way. We describe the case of a male patient with long evolution form of polyostotic fibrous dysplasia of bone with delayed clinical presentation. It is also highlighted the importance of an activity and involvement form evaluation as well as possible endocrine abnormalities associated in order to establish the correct treatment and prognosis.
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Life (Basel)
November 2024
Pediatric Orthopedics and Traumatology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.
Background: Fibrous dysplasia (FD) is a rare condition in which normal spongy and cortical bone is replaced by non-neoplastic fibrous tissue, leading to weakened bone matrix and increased risk of pathological fractures and deformities. Treating these deformities poses a significant challenge for surgeons. While various cases of surgical stabilization and limb lengthening using intramedullary nails have been reported, there is limited evidence on the use of Motorized Intramedullary Limb-Lengthening Nails (MILLNs) in FD patients.
View Article and Find Full Text PDFBrain Sci
November 2024
Department of Neurological Surgery, Tulane University School of Medicine, New Orleans, LA 70112, USA.
Background: Fibrous dysplasia (FD) is often difficult for skull base surgeons to address. FD arises due to the abnormal proliferation of fibroblasts, ultimately resulting in immature osseous tissue replacing normal cancellous bone. When the skull base is involved, it can result in cranial nerve compression.
View Article and Find Full Text PDFCalcif Tissue Int
January 2025
Rheumatology department, Hôpital E. Herriot, Lyon, France.
This study explores FD/MAS patient's perceptions about their disease and its impact on their quality of life. We have evaluated quality of life (QoL) in French Fibrous Dysplasia/MacCune-Albright Syndrome (FD/MAS) patients using a qualitative approach with focus groups to explore perceptions, symptoms and limitations associated with FD/MAS and a quantitative method with the Short Form-36 (SF36) to quantify QoL. Focus groups revealed the heterogeneity of FD forms and allowed for understanding the reasons of reduced QoL.
View Article and Find Full Text PDFBone
December 2024
Department of Otorhinolaryngology - Head and Neck Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, POBox 263, FI-00029 HUS Helsinki, Finland. Electronic address:
Eur J Endocrinol
November 2024
Department of Pediatric Surgery and Urology, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France.
Objective: Adreno CorticoTropic Hormone (ACTH)-independent Cushing's syndrome (CS) in children is very rare but potentially fatal. In bilateral nodular hyperplasia, synchronous bilateral adrenalectomy (SBA) represents the definitive treatment to correct hypercortisolism. We aim to report the multidisciplinary management of this rare condition.
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