Cacciari E, Zucchini S, Ambrosetto P, Tani G, Carlà G, Cicognani A, Pirazzoli P, Sganga T, Balsamo A, Cassio A
First Pediatric Clinic, University of Bologna, Italy.
J Clin Endocrinol Metab. 1994 Mar;78(3):767-71. doi: 10.1210/jcem.78.3.8126155.
Several computed tomographic scan studies have described empty sellae in children with hypothalamic-pituitary disorders. Magnetic resonance imaging, however, is a more precise technique for visualizing the intrasellar content, such as the stalk and pituitary lobes. Using magnetic resonance imaging, we studied 339 children and adolescents (mean age +/- SD, 12.7 +/- 4.5 yr) with possible hypothalamic-pituitary disorders to ascertain the frequency of primary empty sella and examine its relationships with other intrasellar abnormalities, pituitary function, and adverse perinatal events. One hundred and ninety-three patients had isolated GH deficiency, 43 had multiple pituitary hormone deficiency, 10 had diabetes insipidus, 17 had hypogonadotropic hypogonadism, 5 had idiopathic delayed puberty, 47 had precocious puberty, and 24 had other hypothalamic pituitary disorders of hyperfunction. One tenth (10.9%) of the patients (37 cases) had empty sella, with a marked variation of incidences among the disorders listed above. A statistically higher frequency of subjects with empty sellae was found only in patients with multiple pituitary hormone deficiency. Patients with and without empty sellae were not different in regard to age or sex. The incidence of empty sella in the various groups of patients was as follows: isolated GH deficiency, 8.8% (17 cases); multiple pituitary hormone deficiency, 34.9% (15 cases); hypogonadotropic hypogonadism, 5.9% (1 case); idiopathic delayed puberty, 40% (2 cases); and precocious puberty, 4.2% (2 cases). No patients with isolated diabetes insipidus or other hypothalamic-pituitary disorders had empty sellae. In the patients with empty sellae, abnormalities of the stalk or posterior lobe were found in 1 patient with isolated GH deficiency (5.9%), 13 patients with multiple pituitary hormone deficiency (86.7%), and no patients with puberty disorders. Likewise, adverse perinatal events were found only in 1 patient with isolated GH deficiency and 9 patients with multiple pituitary hormone deficiency. These findings suggest that empty sella is not rare in children and adolescents evaluated for hypothalamic-pituitary disorders, particularly if there is multiple pituitary hormone deficiency. Empty sella can be found regardless of abnormalities of the stalk and posterior lobe, and adverse perinatal events do not seem to be the primary etiological factor. Empty sella is usually associated with pituitary hypofunction, but it can be found in patients with hyperfunction of the hypothalamic-pituitary-gonadal axis.
多项计算机断层扫描研究描述了患有下丘脑 - 垂体疾病的儿童存在空蝶鞍。然而，磁共振成像对于观察蝶鞍内结构，如垂体柄和垂体叶，是一种更精确的技术。我们使用磁共振成像对339名可能患有下丘脑 - 垂体疾病的儿童和青少年（平均年龄±标准差，12.7±4.5岁）进行了研究，以确定原发性空蝶鞍的发生率，并研究其与其他蝶鞍内异常、垂体功能及围产期不良事件之间的关系。其中193例患者患有孤立性生长激素缺乏症，43例患有多种垂体激素缺乏症，10例患有尿崩症，17例患有低促性腺激素性性腺功能减退症，5例患有特发性青春期延迟，47例患有性早熟，24例患有其他下丘脑 - 垂体功能亢进疾病。十分之一（10.9%）的患者（37例）有空蝶鞍，在上述疾病中的发生率有显著差异。仅在患有多种垂体激素缺乏症的患者中发现空蝶鞍的发生率在统计学上更高。有空蝶鞍和无空蝶鞍的患者在年龄或性别方面无差异。各患者组中空蝶鞍的发生率如下：孤立性生长激素缺乏症，8.8%（17例）；多种垂体激素缺乏症，34.9%（15例）；低促性腺激素性性腺功能减退症，5.9%（1例）；特发性青春期延迟，40%（2例）；性早熟，4.2%（2例）。没有孤立性尿崩症或其他下丘脑 - 垂体疾病的患者有空蝶鞍。在有空蝶鞍的患者中，1例孤立性生长激素缺乏症患者（5.9%）、13例多种垂体激素缺乏症患者（86.7%）发现垂体柄或后叶异常，而青春期疾病患者中未发现。同样，仅在1例孤立性生长激素缺乏症患者和9例多种垂体激素缺乏症患者中发现围产期不良事件。这些发现表明，在接受下丘脑 - 垂体疾病评估的儿童和青少年中，空蝶鞍并不罕见，尤其是在存在多种垂体激素缺乏症的情况下。无论垂体柄和后叶是否异常均可发现空蝶鞍，围产期不良事件似乎不是主要病因。空蝶鞍通常与垂体功能减退有关，但也可在患有下丘脑 - 垂体 - 性腺轴功能亢进的患者中发现。
