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KMID : 1001920140560030281
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Journal of Korean Neurosurgical Society
2014 Volume.56 No. 3 p.281 ~ p.283
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Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome
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Noh Jung-Hoon
Kong Doo-Sik
Seol Ho-Jun
Shin Hyung-Jin
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Abstract
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McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, cafe-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.
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KEYWORD
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Fibrous dysplasia, McCune-Albright syndrome, Acromegaly
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