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Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-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 c...

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Detalles Bibliográficos
Autores principales: Noh, Jung-Hoon, Kong, Doo-Sik, Seol, Ho Jun, Shin, Hyung Jin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Neurosurgical Society 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4217071/
https://www.ncbi.nlm.nih.gov/pubmed/25368777
http://dx.doi.org/10.3340/jkns.2014.56.3.281
Descripción
Sumario:McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, café-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.