Endocrine Complications in an Adolescent with Fibrous Dysplasia

Abstract

McCune Albright Syndrome is characterized by the triad of fibrous dysplasia, cutaneous manifestations, and endocrine dysfunction. While this disease is typically associated with precocious puberty, there are other endocrinopathies to consider. An adolescent with fibrous dysplasia was diagnosed with acromegaly, hyperthyroidism, phosphate wasting, and hypogonadotropic hypogonadism. His course was complicated by optic neuropathy and parasellar mass ultimately determined to be due to progression of skull base fibrous dysplasia. There was additional consideration of side effects from octreotide. This case exemplifies the potential for under-recognition of acromegaly in McCune Albright Syndrome, which can be associated with cranial nerve deficits. Fibrous dysplasia progression may be associated with additional endocrine dysfunction depending on the location, with potential for necrosis or structural damage of the pituitary gland. Physicians should have high suspicion for endocrinopathies with low threshold for evaluation to optimize outcomes in this population.