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Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome

  • Noh, Jung-Hoon (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kong, Doo-Sik (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Seol, Ho Jun (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Shin, Hyung Jin (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2014.03.28
  • 심사 : 2014.09.06
  • 발행 : 2014.09.28

초록

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, caf$\acute{e}$-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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참고문헌

  1. Akintoye SO, Chebli C, Booher S, Feuillan P, Kushner H, Leroith D, et al. : Characterization of gsp-mediated growth hormone excess in the context of McCune-Albright syndrome. J Clin Endocrinol Metab 87 : 5104-5112, 2002 https://doi.org/10.1210/jc.2001-012022
  2. Albright F, Butler AM, Hampton AO, Smith P : Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females : reports of five cases. N Engl J Med 216 : 727-746, 1937 https://doi.org/10.1056/NEJM193704292161701
  3. Chanson P, Dib A, Visot A, Derome PJ : McCune-Albright syndrome and acromegaly : clinical studies and responses to treatment in five cases. Eur J Endocrinol 131 : 229-234, 1994 https://doi.org/10.1530/eje.0.1310229
  4. Cremonini N, Graziano E, Chiarini V, Sforza A, Zampa GA : Atypical McCune-Albright syndrome associated with growth hormone-prolactin pituitary adenoma : natural history, long-term follow-up, and SMS 201-995--bromocriptine combined treatment results. J Clin Endocrinol Metab 75 : 1166-1169, 1992
  5. Dou W, Lin N, Ma W, Yang Y, Zhu H, Sun J, et al. : Transsphenoidal surgery in a patient with acromegaly and McCune-Albright syndrome : application of neuronavigation. J Neurosurg 108 : 164-169, 2008 https://doi.org/10.3171/JNS/2008/108/01/0164
  6. Gessl A, Freissmuth M, Czech T, Matula C, Hainfellner JA, Buchfelder M, et al. : Growth hormone-prolactin-thyrotropin-secreting pituitary adenoma in atypical McCune-Albright syndrome with functionally normal Gs alpha protein. J Clin Endocrinol Metab 79 : 1128-1134, 1994
  7. Holdaway IM : Treatment of acromegaly. Horm Res 62 Suppl 3 : 79-92, 2004
  8. Madsen H, Borges MT, Kerr JM, Lillehei KO, Kleinschmidt-Demasters BK : McCune-Albright syndrome : surgical and therapeutic challenges in GH-secreting pituitary adenomas. J Neurooncol 104 : 215-224, 2011 https://doi.org/10.1007/s11060-010-0461-9
  9. McCune DJ : Osteitis fibrosa cystica : the case of nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. Am J Dis Child 52 : 743-747, 1936
  10. Shenker A, Weinstein LS, Sweet DE, Spiegel AM : An activating Gs alpha mutation is present in fibrous dysplasia of bone in the McCune-Albright syndrome. J Clin Endocrinol Metab 79 : 750-755, 1994
  11. Weinstein LS, Shenker A, Gejman PV, Merino MJ, Friedman E, Spiegel AM : Activating mutations of the stimulatory G protein in the McCune-Albright syndrome. N Engl J Med 325 : 1688-1695, 1991 https://doi.org/10.1056/NEJM199112123252403