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[空蝶鞍综合征。20例临床、影像学及内分泌学分析]

[The empty sella syndrome. Clinical, radiological and endocrinologic analysis in 20 cases].

作者信息

Degli Uberti E C, Teodori V, Trasforini G, Tamarozzi R, Margutti A, Bianconi M, Rossi R, Ambrosio M R, Pansini R

出版信息

Minerva Endocrinol. 1989 Jan-Mar;14(1):1-18.

PMID:2659952
Abstract

Empty sella syndrome is an anatomical entity in which the pituitary fossa is enlarged and partially filled with cerebrospinal fluid owing to the arachnoid herniation, while the pituitary gland is compressed against the posterior rim of the fossa. This condition can be due to an inherent weakness of the diaphragm sella and/or to an increase in intracranial pressure which promote the herniation of the arachnoid membrane into the pituitary fossa (primary empty sella) or it can results following surgery, radiation or vascular and tumorous pituitary diseases (secondary empty sella). Empty sella can be associated with neuroradiological and endocrine symptoms. This study reports the clinical, endocrine, and roentgenographic features in 20 patients with primary empty sella syndrome. Disturbances of hypothalamic-pituitary function were detected in 6 patients (hyperprolactinemia, hypopituitarism, central diabetes insipidus, hypothalamic hypothyroidism). Three patients exhibited hypergonadotropic hypogonadism. This report supports the following conclusions: a) there is no correlation between size of pituitary fossa, type an extension of arachnoid herniation and the degree of hypothalamic-pituitary dysfunction; b) endocrine alterations are frequent in the empty sella syndrome; c) the association of empty sella and primary diabetes insipidus is not a very rare event.

摘要

空蝶鞍综合征是一种解剖学实体,其中由于蛛网膜疝,垂体窝扩大并部分充满脑脊液,而垂体被挤压在垂体窝后缘。这种情况可能是由于鞍膈固有薄弱和/或颅内压升高,促进蛛网膜膜疝入垂体窝(原发性空蝶鞍),也可能是手术、放疗或血管及垂体肿瘤性疾病(继发性空蝶鞍)后的结果。空蝶鞍可伴有神经放射学和内分泌症状。本研究报告了20例原发性空蝶鞍综合征患者的临床、内分泌和影像学特征。6例患者检测到下丘脑-垂体功能紊乱(高催乳素血症、垂体功能减退、中枢性尿崩症、下丘脑性甲状腺功能减退)。3例患者表现为高促性腺激素性性腺功能减退。本报告支持以下结论:a)垂体窝大小、蛛网膜疝类型和扩展与下丘脑-垂体功能障碍程度之间无相关性;b)空蝶鞍综合征中内分泌改变常见;c)空蝶鞍与原发性尿崩症的关联并非罕见事件。

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