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

Abstract

Introduction: McCune-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 presentation: A 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. Conclusion: CT 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. © 2009 Li et al; licensee BioMed Central Ltd.