Raiti S, Albrink M J, Maclaren N K, Chadduck W M, Gabriele O F, Chou S M
Am J Dis Child. 1976 Sep;130(9):1009-12. doi: 10.1001/archpedi.1976.02120100099017.
A 15-year-old boy had growth failure and failure of sexual development. The probable onset was at age 10. Endocrine studies showed hypopituitarism with deficiency of growth hormone and follicle-stimulating hormone, an abnormal response to metyrapone, and deficiency of thyroid function. Luteinizing hormone level was in the low-normal range. Posterior pituitary function was normal. Roentgenogram showed a large sella with some destruction of the posterior clinoids. Transsphenoidal exploration was carried out. The sella was empty except for a whitish membrane; no pituitary tissue was seen. The sella was packed with muscle. Recovery was uneventful, and the patient was given replacement therapy. On histologic examination,the cyst wall showed low pseudostratified cuboidal epithelium and occasional squamous metaplasia. Hemosiderin-filled phagocytes and acinar structures were also seen. The diagnosis was probable rupture of an intrasellar epithelial cyst, leading to empty sella syndrome.
一名15岁男孩出现生长发育迟缓及性发育障碍。可能于10岁起病。内分泌检查显示垂体功能减退,生长激素和促卵泡激素缺乏,对甲吡酮反应异常,甲状腺功能减退。黄体生成素水平处于低正常范围。垂体后叶功能正常。X线片显示蝶鞍增大,后床突有部分破坏。进行了经蝶窦探查。蝶鞍内除一层白色薄膜外为空的,未见垂体组织。蝶鞍内充满肌肉。恢复过程顺利,给予患者替代治疗。组织学检查显示,囊肿壁为低柱状假复层上皮,偶见鳞状化生。还可见含铁血黄素吞噬细胞和腺泡结构。诊断可能为鞍内上皮囊肿破裂,导致空蝶鞍综合征。