Journal article

Severe Fibrous Dysplasia in McCune-Albright Syndrome: A Need for Continuous Surveillance

Francisca Grob, Jonathan Clark, Margaret Zacharin

HORMONE RESEARCH IN PAEDIATRICS | KARGER | Published : 2020

Abstract

INTRODUCTION: McCune-Albright syndrome (MAS) is a rare condition, in which GNAS mutations affect multiple organs. Fibrous dysplasia (FD), affecting only one or multiple skeletal territories, may severely affect craniofacial structures. Concomitant occurrence of acromegaly aggravates skull deformity, leading to eye, ear, and posterior cranial fossa compromise. CASE PRESENTATION: A 30-year-old man diagnosed with MAS at the age of 3 developed almost all known complications of the syndrome. The craniofacial component of his polyostotic FD increased over time, aggravated by difficult to control acromegaly. Acute onset of severe headache and neurologic compromise, caused by subarachnoid haemorrhag..

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