Abstract
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.
Highlights
McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and caféau-lait pigmented skin lesions
There were no reports about cyst degeneration of Fibrous dysplasia (FD) in MAS which resulted in abnormal visual acuity and visual fields
We report a female patient with MAS associated with sphenoid bone cysts which resulted in visual deterioration
Summary
Fibrous dysplasia (FD) is primarily a developmental abnormality of the bone-forming mesenchyme in which fibrous tissue gradually expands and replaces the bone. We report a female patient with MAS associated with sphenoid bone cysts which resulted in visual deterioration. A 20-year-old female presented with right temporal hemianopsia and visual loss in the right eye She presented café-au-lait spots first noted at 2 months old. Her vision deteriorated suddenly when she was in hospital for ankle joint sprain 10 days ago. There were several other cysts manifested as hypertensity on T2 weighted images in frontal bone (Fig. 9) Her visual acuity and visual fields were normal 2 months ago. In view of the clinical presentation, cystic pituitary adenoma complicated with hemorrhage was suspected at first, but radiological appearances of the lesion showed that cyst degeneration of fibrous dysplasia can not be excluded because the normal pituitary gland can be seen.
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