Does pseudotumor cerebri cause the empty sella syndrome?

Eight patients had both the "primary empty sella syndrome," diagnosed by the finding of an air-filled sella turcica at pneumoencephalography, and pseudotumor cerebri, diagnosed by the finding of an elevated cerebrospinal fluid pressure in the presence of normal ventricular size and position on pneumonencephalography. All eight patients were obese women, and six were hypertensive. Six complained of headaches and menstrual irregularities, and two were asymptomatic. Three had visual symptoms and four had papilledema at the time of examination. These two clinical disorders appear to be frequently related, and when they are related, visual field defects and visual loss are more likely to occur than when either entity appears alone. Chronically increased intracranial pressure from pseudotumor cerebri may produce an empty sella if the diaphragma sella is incompetent and the subarachnoid space herniates into the sella turcica.     

Intracranial pressure in patients with the empty sella syndrome without benign intracranial hypertension.

The intracranial pressure was monitored continuously for at least 48 hours in five patients with empty sella syndrome, who did not have clinical benign intracranial hypertension (BIH). It has been suggested that the empty sella syndrome is a result of chronically elevated intracranial pressure in the presence of a congenitally deficient diaphragma sellae. However, whilst the intracranial pressure in two of the five patients was abnormally high, in three patients in whom it was monitored, the CSF pressure was normal. Although these cases may represent "burnt out" forms of intracranial pressure problems, it might be that the normal pulsations of CSF are sufficient to produce the empty sella in the presence of a deficient diaphragma sella.     

Relationship between chronic raised intracranial pressure and empty sella presenting hormonal disturbances

The role of intracranial pressure in the development and maintenance of the primary empty sella has been pointed out in the literature previously. The hormonal changes and clinical features have been evaluated in a 30 year-old female patient examined for a convexity meningioma and a 20 year-old patient examined for chronic hydrocephalus caused by cured meningitis. Histories and investigations revealed an empty sella turcica associated with primary amenorrhoea and delayed puberty. The removal of the convexity meningioma resulted in loss of amenorrhoea and a rise in plasma gonadotrophins. The establishment of a ventriculo-peritoneal shunt did not bring about any changes in hormonal values and clinical features except the subjective headache disappeared. The importance of consideration of intracranial causes in patients who have delayed puberty or absence of menstrual history is briefly emphasized in light of the literature. Our data also demonstrated a correlation between an increase in intracranial pressure and a deficiency of hormonal secretion by the hypophysis.     

Relationship between chronic raised intracranial pressure and empty sella presenting hormonal disturbances

The role of intracranial pressure in the development and maintenance of the primary empty sella has been pointed out in the literature previously. The hormonal changes and clinical features have been evaluated in a 30 year-old female patient examined for a convexity meningioma and a 20 year-old patient examined for chronic hydrocephalus caused by cured meningitis. Histories and investigations revealed an empty sella turcica associated with primary amenorrhoea and delayed puberty. The removal of the convexity meningioma resulted in loss of amenorrhoea and a rise in plasma gonadotrophins. The establishment of a ventriculo-peritoneal shunt did not bring about any changes in hormonal values and clinical features except the subjective headache disappeared. The importance of consideration of intracranial causes in patients who have delayed puberty or absence of menstrual history is briefly emphasized in light of the literature. Our data also demonstrated a correlation between an increase in intracranial pressure and a deficiency of hormonal secretion by the hypophysis.     

Asymptomatic enlargement of the sella turcica.

Forty-six patients with enlarged sella turcica and pneumographic evidence of an intrasellar mass were initially untreated. The courses were variable, and 50% did not require subsequent treatment. All patients with initial visual involvement developed progressive visual impairment. Of patients with clinical evidence of pituitary insufficiency, 66% developed visual field defects and required treatment. Only one patient, whose sole symptom was headache, was subsequently treated, and no asymptomatic patient developed subsequent symptoms and required treatment. Asymptomatic patients with an enlarged sella turcica should have an air study to exclude an "empty sella" syndrome or primary hypothyroidism.     

Anatomical variants in the floor of the third ventricle; implications for endoscopic third ventriculostomy

Longstanding hydrocephalus and raised intracranial pressure can lead to unusual anatomical variants in the floor of the third ventricle, which may be important when performing endoscopic third ventriculostomy. Two middle aged patients with symptomatic longstanding hydrocephalus had scans that showed ventricular hydrocephalus, an empty sella, and a dilated infundibular recess which herniated into the sella turcica. Endoscopic third ventriculostomy confirmed that instead of the tuber cinerum and infundibular recess, the anterior inferior floor of the third ventricle was hanging down ventral to the pons into the sellar floor. Third ventriculostomy to the prepontine cistern was made on the dorsal wall of the dilated infundibular recess to the area surrounded by the dorsum sellae, the basilar artery trunk, and the left superior cerebellar artery, with good symptomatic control. Association of the empty sella and persistence of the infundibular recess must be carefully evaluated by MRI before attempting endoscopic third ventriculostomy. Herniation of the anterior inferior floor of the third ventricle into the empty sella can lead to loss of anatomical landmarks that require special attention during third ventriculostomy.     

Idiopathic intracranial hypertension in female homozygous twins.

The authors report on female homozygous twins with idiopathic intracranial hypertension. At the age of 12 years, both twins simultaneously developed visual disturbances with photophobia. At the age of 19 years, an ophthalmological examination disclosed papilloedema in both their eyes. At the age of 22 years, a lumbar puncture showed raised CSF pressure over (200 mm H2O) in both twins. Their neurological and radiological examinations were extremely similar; both of them had severely impaired visual acuity and impaired visual field, bilateral optic nerve atrophy, intracranial hypertension, an enlarged and partial empty sella turcica, digital markings of the calvalium, and an enlarged frontal subarachnoid space. This is the first case report describing idiopathic intracranial hypertension occurring in homozygous twins.     

Spontaneous haemorrhage into an empty sella turcica mimicking pituitary apoplexy

We present a case of spontaneous haemorrhage into an empty sella turcica with the features of subclinical pituitary apoplexy. A 66-year-old woman with a previously resected pituitary adenoma presented four months later with progressive headache and visual deterioration. Cranial MRI demonstrated hyperacute blood products in a recurrent pituitary adenoma. Operative findings were of subacute blood in an empty sella turcica. There was no operative or subsequent histological evidence of tumour recurrence. The intrasellar haemorrhage was evacuated via a trans-sphenoidal approach, resulting in a rapid improvement in visual function. Endocrine deficits required thyroxine, corticosteroid and desmopressin supplementation. Haemorrhage into an empty sella turcica has not been previously described and needs to be suspected as a clinical entity in patients presenting with the features of pituitary apoplexy. Awareness of this clinical condition will prevent preoperative misdiagnosis.     

Analysis of Empty Sella Secondary to the Brain Tumors

Objective

The definition of empty sella syndrome is ''an anatomical entity in which the pituitary fossa is partially or completely filled with cerebrospinal fluid, while the pituitary gland is compressed against the posterior rim of the fossa''. Reports of this entities relating to the brain tumors not situated in the pituitary fossa, have rarely been reported.

Methods

In order to analyze the incidence and relationship of empty sella in patients having brain tumors, the authors reviewed preoperative magnetic resonance imaging (MRI) of 72 patients with brain tumor regardless of pathology except the pituitary tumors. The patients were operated in single institute by one surgeon. There were 25 males and 47 females and mean patient age was 53 years old (range from 5 years to 84 years). Tumor volume was ranged from 2 cc to 238 cc.

Results

The overall incidence of empty sella was positive in 57/72 cases (79.2%). Sorted by the pathology, empty sella was highest in meningioma (88.9%, p = 0.042). The empty sella was correlated with patient''s increasing age (p = 0.003) and increasing tumor volume (p = 0.016).

Conclusion

Careful review of brain MRI with periodic follow up is necessary for the detection of secondary empty sella in patients with brain tumors. In patients with confirmed empty sella, follow up is mandatory for the management of hypopituitarism, cerebrospinal fluid (CSF) rhinorrhea, visual disturbance and increased intracranial pressure.     

Chronic intracranial hypertension secondary to neurobrucellosis

Summary Chronic intracranial hypertension in the presence of hydrocephalus and/or arachnoiditis is a rare presentation of neurobrucellosis. The present case is exceptional because neither hydrocephalus nor arachnoiditis were present. Brucellosis was diagnosed by serological tests. The patient developed asthenia, anorexia, weight loss, violent headaches, explosive vomiting, bilateral papilloedema, diplopia with paralysis of the abducens nerves, left supranuclear facial paralysis and left hemiparesis. A skull radiograph showed destruction of the sella turcica. Rapid recovery was attained with the use of antibiotics. The pathogenesis of this intracranial hypertension syndrome with destruction of sella turcica is discussed.     

Secondary partial empty sella syndrome in an elite bodybuilder.

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient and the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiological mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.     

Secondary partial empty sella syndrome in an elite bodybuilder

Abstract

The pituitary gland is a hormone-responsive gland and is known to vary in size depending on the hormonal status of the patient snd the multifaceted positive and negative feedback hypothalamic-pituitary-gonadal axis. Partial empty sella syndrome with an atrophied pituitary gland is seen in primary neuroendocrinopathies such as growth hormone deficiency, primary hypothyroidism, central diabetes insipidus and hypogonadism. Partial empty sella has also been shown to occur in patients with elevations in intracranial pressure. Secondary partial empty sella syndrome with significant pituitary gland atrophy from negative feedback inhibition of long-term exogenous hormonal use has not been previously reported. We are reporting on a case of partial empty sella syndrome occurring in an elite bodybuilder with a long history of exogenous abuse of growth hormone, testosterone and thyroid hormone. The pathophysiologal mechanisms of secondary partial empty sella syndrome from exogenous hormone use and the possibility for elevations in intracranial pressure contributing to this syndrome will be discussed.     

垂体腺瘤经蝶切除术后发生迟发性视功能减退原因及处理探讨

目的 探讨经蝶切除垂体腺瘤术后继发迟发性视功能减退的原因，尤其是其与空蝶鞍的关系，提出积极防治的意义。方法 分析了北京协和医院诊治过的4例经蝶手术切除垂体腺瘤术后继发不同程度空蝶鞍的迟发性视功能减退患者，并结合文献加以讨论。结果 这4例患者中2例再次经颅手术，1例再次经蝶手术，1例接受保守治疗，治疗后其视功能均有不同程度地改善或恢复正常。结论 结合文献复习，认为经蝶术后合并空蝶鞍的患者发生迟发性视功能减退与视神经和视交叉局部的手术后瘢痕组织机械性牵拉以及血运改变有关。综合治疗后4例患者的视功能均有不同程度的改善或恢复正常，这既说明了上述原因的存在，又显示了积极防治的意义。     

Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella.

We present a case of growth hormone (GH)-secreting ectopic pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella. It has been well known that acromegaly is sometimes associated with an empty sella. However, such a case usually has a macroadenoma and an empty sella that is large. The authors considered the possible mechanisms of the association between a normal-sized empty sella and an ectopic pituitary adenoma in the sphenoid sinus as the following. Primary empty sella existed originally, and the pituitary adenoma developed later. The adenoma extended into the sphenoid sinus because of the pulsatile intracranial cerebrospinal fluid pressure.     

Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings

A 37-year-old man with visual loss was found to have hypopituitarism and primary polydipsia associated with sarcoidosis. Neuroradiologic studies demonstrated a dramatic evolution of CNS lesions, including a left thalamic infarct, an enhancing suprasellar mass, and ultimately an empty sella turcica. The patient has been clinically stable in spite of these changes. This case is likely to be the first reported of CNS sarcoidosis with an empty sella turcica documented by computed tomography.     

Does bilateral transverse cerebral venous sinus stenosis exist in patients without increased intracranial pressure?

Objective

Transverse cerebral venous sinus stenosis (TSS) is common among patients with idiopathic intracranial hypertension. TSS likely also exists among individuals with normal intracranial pressure (ICP) but the prevalence is unclear. The goal of this study was to identify patients with incidental TSS and normal ICP and describe their characteristics.

Methods

Among 240 adult patients who underwent brain magnetic resonance imaging (MRI) with magnetic resonance venography (MRV) with contrast at our institution between September 2009 and September 2011, 44 had isolated TSS without further substantial imaging abnormality. Medical records were reviewed for symptoms of increased ICP, papilledema, cerebrospinal fluid (CSF) constituents and opening pressure (OP), and reason for brain imaging. Of these, 37 were excluded for confirmed or possible idiopathic intracranial hypertension. Of the remainder, 5 had CSF-OP ≤25 cmH₂O without papilledema, and 2 did not have measured ICP, but had no papilledema or symptoms of increased ICP. Imaging was re-interpreted to assess for signs suggestive of elevated ICP and to characterize the TSS further.

Results

All patients were women (mean age: 41, mean BMI: 37.1). CSF contents were normal, but OPs were at the upper limit of normal (22–25 cmH₂O). Indications for MRI/MRV included query pituitary abnormality (1), migraine (4), and anomalous-appearing optic nerves (2). All had bilateral TSS. Six had short TSS and an empty sella; 1 had long TSS and no empty sella; 1 had flattening of the posterior sclera; 2 had prominence of peri-optic nerve CSF.

Conclusion

Asymptomatic bilateral TSS exists in patients with ICP ≤ 25 cmH₂O, but is likely uncommon. CSF-OP was at the upper limit of normal in our patients, who also had other radiologic signs suggestive (but not specific) of chronically-raised ICP. Findings of bilateral TSS on imaging should prompt funduscopic examination for papilledema.     

Endocrinology of pseudotumor cerebri

This article will review the endocrine function of obese young women with pseudotumor cerebri and primary empty sella syndrome. The literature can be difficult to interpret. Much of it predates the era of CT scanning. Often, cases of primary and secondary empty sella syndrome are comingled. The author reviews specific endocrinologic disorders that have sometimes been associated with increased intracranial pressure excluding pituitary adenomas.     

Pathogenesis of non-traumatic cerebrospinal fluid rhinorrhea

15 consecutive patients with non-traumatic cerebrospinal fluid rhinorrhea were studied. 13 operations were performed on 10 patients. In 8 transcranial operations, an assumed defect in the anterior fossa was plugged with muscle, but only 3 operations were successful. In 4 operations, either transcranial or transsphenoidal, the sella was packed with muscle and rhinorrhea ceased immediately.
Based on radiological and operative findings, 3 groups of patients appeared (1) 9 patients had pathology related to the pituitary gland or the sella turcica: enlarged sella, empty sella, pituitary tumour, intrasellar cyst or erosion of the sellar osseous border. (2) 2 patients had rhinorrhea from extrasellar origin. (3) In 4 patients no abnormality could be found.
Prior to the rhinorrhea, 6 patients (5 from group 1 and 1 from group 3) had experienced episodes of neurological symptoms, compatible with a pituitary apoplexy.
It is suggested that non-traumatic cerebrospinal fluid rhinorrhea in most cases is the result of a spontaneous necrosis in a pituitary adenoma, which has caused sellar bony erosion.     

The empty sella and pituitary adenomas

In 44 consecutive patients with sellar volume larger than 1100 mm³, computer tomography showed that 20 had an empty or partly empty sella. None had radiological evidence of a suprasellar tumor. 10 of the 20 patients had experienced episodes with acute neurological symptoms presumably reflecting a pituitary apoplexy. It is suggested that an unknown proportion of intrasellar adenomas may disappear as a result of an infarction, which may comprise the entire adenoma or part of it - leaving an empty or partly empty sella as diagnosed by computer tomography. Air encephalography will demonstrate the empty sella only if the diaphragmatic aperture is large enough to allow cisternal herniation. The infarction may present clinically with no, slight or severe acute neurological symptoms. Late consequences of a pituitary adenoma infarction may be rhinorrhea or hydrocephalus.     

Hyperprolactinaemia and the empty sella

Hyperprolactinaemia is an endocrine abnormality seen not infrequently in the population with empty sella; a radiological and anatomical diagnosis of a deformed and enlarged sella turcica. Often there is no associated pathology within the pituitary gland itself, lending to the hypothesis that the empty sella syndrome per se has a yet-to-be defined role in hyperprolactinemia. We report a patient who presented initially with non-specific symptoms of meningeal irritation and viral illness on a long background of galactorrhoea. The patient demonstrated elevated serum prolactin, and a diagnosis of empty sella was made on the basis of MRI findings.