Pituitary Unit, Department of Endocrinology.
Institute of Radiology.
Eur J Endocrinol. 2017 Dec;177(6):R275-R285. doi: 10.1530/EJE-17-0505. Epub 2017 Aug 5.
Primary empty sella (PES) is characterized by the herniation of the subarachnoid space within the sella, which is often associated with variable degrees of flattening of the pituitary gland in patients without previous pituitary pathologies. PES pathogenetic mechanisms are not well known but seem to be due to a sellar diaphragm incompetence, associated to the occurrence of upper sellar or pituitary factors, as intracranial hypertension and change of pituitary volume. As PES represents in a majority of cases, a neuroradiological findings without any clinical implication, the occurrence of endocrine, neurological and opthalmological symptoms, due to the above describes anatomical alteration, which delineates from the so called PES syndrome. Headache, irregular menses, overweight/obesity and visual disturbances compose the typical picture of PES syndrome and can be the manifestation of an intracranial hypertension, often associated with PES. Although hyperprolactinemia and growth hormone deficit represent the most common endocrine abnormalities, PES syndrome is characterized by heterogeneity both in clinical manifestation and hormonal alterations and can sometime reach severe extremes, as occurrence of papilledema, cerebrospinal fluid rhinorrhea and worsening of visual acuity. Consequently, a multidisciplinary approach, with the integration of endocrine, neurologic and ophthalmologic expertise, is strongly advocated and recommended for a properly diagnosis, management, treatment and follow-up of PES syndrome and all of the related abnormalities.
原发性空蝶鞍(PES)的特征是蛛网膜下腔疝入蝶鞍,常伴有既往无垂体病变患者的垂体不同程度扁平。PES 的发病机制尚不清楚,但似乎与鞍膈功能不全有关,与颅内压升高和垂体体积变化等鞍上或垂体因素有关。由于 PES 在大多数情况下是一种无任何临床意义的神经影像学发现,因此由于上述描述的解剖改变而发生的内分泌、神经和眼科症状与所谓的 PES 综合征不同。头痛、月经不规律、超重/肥胖和视力障碍构成了 PES 综合征的典型特征,可能是颅内压升高的表现,常与 PES 相关。尽管高泌乳素血症和生长激素缺乏是最常见的内分泌异常,但 PES 综合征在临床表现和激素改变方面具有异质性,有时甚至会达到严重极端,如视乳头水肿、脑脊液鼻漏和视力恶化。因此,强烈提倡并推荐采用多学科方法,整合内分泌、神经和眼科专业知识,对 PES 综合征及其所有相关异常进行适当的诊断、管理、治疗和随访。