Complication Of Fibrous Dysplasia Research Articles

Optic disc edema in fibrous dysplasia/McCune-Albright syndrome: Prevalence, etiologies, and clinical implications.

Fibrous dysplasia (FD) is a rare disorder of expansile fibro-osseous lesions that may be associated with extraskeletal features as part of McCune-Albright syndrome (MAS). Optic disc edema is a potentially serious ophthalmologic finding that has been rarely reported in patients with FD/MAS. The purpose of this study was to investigate the prevalence and potential clinical associations of optic disc edema in a large cohort. Clinical records were reviewed from subjects in an ongoing FD/MAS natural history study. Computed Tomography scans were evaluated for the presence of structural craniofacial abnormalities associated with optic disc edema, including Chiari I malformation and space-occupying lesions. Craniomorphometric analyses were performed to determine optic canal diameter and intracranial volume. Statistical analyses were performed to compare clinical and radiographic features between subjects with and without optic disc edema. Optic disc edema was diagnosed in 7/187 subjects, for a prevalence of 3.7%. All subjects with optic disc edema were diagnosed before age 18years and had mild, non-progressive disease. Radiographic structural abnormalities, including Chiari I malformation, aneurysmal bone cysts, and arachnoid cysts, were associated with higher odds of optic disc edema (odds ratio [OR] 24.3; 95% confidence interval [CI], 4.2 to 121.4; p<0.01) (OR 18.0; 95% CI, 3.4 to 108.2; p<0.01). Treatment with leuprolide, a gonadotropin releasing hormone analog, was also associated with optic disc edema (OR 26.0; 95% CI 3.3 to 177.5; p<0.05). There was no significant association of optic disc edema with other MAS endocrinopathies, medications, optic canal diameter, or intracranial volume. Optic disc edema is an uncommon but potentially serious complication of craniofacial FD, which may occur more frequently in pediatric patients and those with structural craniofacial abnormalities. The potential association of leuprolide therapy with optic disc edema in this population warrants further study.

Sphenoid mucocoele as a complication of fibrous dysplasia of the facial bones.

We report on a 28-year-old man with severe headache. Imaging studies included CT and MR. A large sphenoid mucocoele was demonstrated as well as findings compatible with fibrous dysplasia of the facial bones. Both disorders had been previously unknown. Imaging findings suggested that the sphenoid mucocoele was related to an obstruction caused by the expansile bone of fibrous dysplasia. These findings were confirmed surgically. Fibrous dysplasia with subsequent outflow obstruction is an extremely rare cause of sphenoid mucocoele development.

MR and CT findings of cyst degeneration of sphenoid bone in McCune-Albright syndrome: a case report

IntroductionMcCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and café-au-lait pigmented skin lesions. Cystic change is rare in fibrous dysplasia (FD), especially in McCune-Albright syndrome. There were no reports about cyst degeneration in MAS which resulted in abnormal visual acuity and visual fields. Herein, we report a female patient with MAS associated with sphenoid bone cysts which resulted in visual deterioration to describe the computed tomography (CT) and magnetic resonance (MR) imaging findings of cyst degeneration in McCune-Albright syndrome.Case presentationA 20-year-old female presented with right temporal hemianopsia and visual loss in the right eye suddenly. A café-au-lait spot was found on her neck and left shoulder. Endocrinologic examination revealed elevated basal level of serum PRL, FT3 and FT4 with decreased serum TSH. Fibrous dysplasia (FD) generally manifest as round-glass appearance with well defined borders and cystic areas within involved bone were seen as hypointensity on CT. They were showed as hypointense in T1-weighted sequences and as hyperintense in T2-weighted sequences of MRI. After surgery the right temporal hemianopsia improved.ConclusionCT combined with MRI is the most effective method to evaluate the extent and complications of fibrous dysplasia in patients with MAS. The treatment of surgery can not cure MAS but relieve the symptom.