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.     

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.     

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.     

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.     

The 'empty' sella.

Records of all patients diagnosed as having an "empty" sella turcica at the University of Michigan Medical Center after 1972 were retrospectively reviewed. Nineteen patients satisfied the two criteria, absence of prior surgical or radiotherapeutic intervention and air within the sella turcica at pneumoencephalography. Associated clinical signs and symptoms were varied and nonspecific. Two patients had relative visual field deficits without increased intracranial pressure. Three patients had papilledema and were diagnosed as having had benign increased intracranial pressure. Increased intracranial pressure was not a consistent enough finding to be considered an etiology for the empty sella syndrome.     

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.     

Empty sella in children as a key for diagnosis

We performed magnetic resonance imaging of the pituitary gland in 354 children who were under the care of our pediatric neurological outpatient department. Among them, an empty sella was recognized in 11 children, who all showed normal hormonal results. The frequency of an empty sella was significantly high in idiopathic intracranial hypertension (4/4; P<0.0001) and nevoid basal cell carcinoma syndrome (3/5; P<0.0001). The ratio of an empty sella in children, excluding patients with idiopathic intracranial hypertension and nevoid basal cell carcinoma syndrome, is estimated to be 4/345 (1.2%), which is markedly lower than that in adults. Magnetic resonance imaging of the pituitary gland in children could provide important clues for the diagnosis of idiopathic intracranial hypertension or nevoid basal cell carcinoma syndrome.     

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.     

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.     

原发性高颅压中垂体和蝶鞍形态演变

目的 :证实原发性高颅压中垂体和蝶鞍形态改变的演化规律。方法 :10例经临床确诊的原发性高颅压病人 ,通过比较复查前后 MR正中矢状面垂体和蝶鞍出现的形态学改变。结果 :全部病人在首次 MR检查中均发现不同程度的空蝶鞍或出现蝶鞍扩大。在复查 MR中 ,有 9例病人的垂体形态和蝶鞍扩大程度出现改变 ,1例空蝶鞍和蝶鞍稍扩大病人在复查 MR中未见明显变化。结论 :原发性高颅压可引起的鞍区形态学改变 ,是与颅压增高程度和病程长短有关的动态演变过程。垂体形态改变要早于蝶鞍改变 ;而蝶鞍扩大出现较晚 ,提示已经存在长时期的明显的颅压增高     

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.     

Delayed cerebrospinal fluid rhinorrhea after gamma knife surgery in a patient with a growth hormone-secreting adenoma

We report a patient who developed delayed cerebrospinal fluid (CSF) rhinorrhea 11 years after gamma knife radiosurgery for a growth hormone (GH)-secreting adenoma. The treatment dose was 18 Gy for the tumor margin (50% isodose). One year later, an MRI of the head revealed that the tumor size had decreased. Eleven years later, the patient developed CSF rhinorrhea from the left nostril. Subsequent MRI examination revealed complete remission of the tumor in the sella turcica and the empty sella. The patient was admitted for direct endoscopic surgical repair of the skull base. We suggest that the cause of the CSF rhinorrhea is secondary empty sella. The other potential causes may be the original invasiveness of the tumor or delayed radiation damage to the mucous membranes of the skull. Long-term follow-up is required to monitor recurrence of CSF rhinorrhea.     

Aggressive rhabdoid meningioma with osseous,papillary and chordoma‐like appearance

Meningiomas are the most common primary intracranial tumors. They are usually benign and slowly growing; however, they may show histologically malignant features categorizing them into grade II or III of World Health Organization (WHO) classification. Rhabdoid meningioma (RM) is an uncommon meningioma variant categorized as WHO grade III. The clinical course of RM is determined by local recurrences, invasion of adjacent brain and/or dura, widespread leptomeningeal dissemination, remote metastases and fatal clinical outcome. Herein we report a case with recurrent aggressive left occipital parasagittal region RM in which the patient initially declined radiation treatment. The tumor was resected four times in 5 years. Histopathological examination revealed a rhabdoid meningioma with metaplastic, papillary and chordoid differentiation. Six months after her fourth operation the patient died of progressive disease. RM is a rare subtype of malignant meningioma and the role of different adjuvant therapeutic options are still unknown. Clinical presentation, radiological features and pathologic findings of this uncommon tumor are discussed.     

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.     

Hormonal dysfunction of nonpituitary lesions from midline and perisellar area]

OBJECTIVE: To analyse clinical and/or laboratorial preoperative hormonal dysfunction, of the nonpituitary intracranial lesions from midline and parasellar area. METHOD: Forty-four patients were evaluated with nonpituitary intracranial lesions, who had images studies (computed tomography or magnetic resonance) and preoperative basal hormonal level; 16 had preoperative hypothalamus-hypophysial function tests (megatests). These patients were divided in two groups. Group I - 34 lesions from midline: 11 craniopharyngiomas, 8 meningiomas, 3 germinomas, 3 tumors of sphenoid sinus, 2 empty sella syndrome, 2 pylocitic astrocytomas, 1 giant aneurysm, 2 mucoceles, 1 III ventricle diverticulum and 1 Rathke's cleft cyst; Group II - 10 lesions from parasellar area: 9 meningiomas and 1 giant aneurysm. RESULTS: In group I, 25/34 (73.5%) patients showed laboratorial hormonal deficit (14 without clinical manifestations) 18/34 (52.9%) hyperprolactinemia (5 with galactorreia) and 8 (53.3%) showed growth hormone deficiency in 15 megatests available in this group; 3 (8.8 %) patients presented central diabetes insipidus (CDI). In group II, 6/10 (60%) patients showed laboratorial hormonal deficit (5 without clinical manifestations), 1 (10%) hyperprolactinemia and 1 growth hormone deficiency (single megatest realized in this group); no patient had preoperative CID. CONCLUSIONS: The presence of nonspecific or poorly valorized clinical manifestations, does not indicate absence of hormonal dysfunction; in this present serie, 19/38 (50%) patients with laboratorial abnormalities, didn't show clinical manifestations. Hormonal dysfunction is frequent in sellar and perisellar nonpituitary lesions, specially involving midline.     

Hemiballismus due to contralateral sphenoid ridge meningioma

Primary intracranial tumour is a very uncommon cause of hemiballismus. In our review of the literature only two verified cases were found: one meningioma and one meningoblastoma. We report a patient with right hemiballismus due to a contralateral meningioma of the sphenoid ridge. His symptoms disappeared completely after resection of the tumour. The patient had no signs of increased intracranial pressure. Possible pathogenic mechanisms are briefly discussed. A consequence of such an experience is that magnetic resonance imaging or computed tomography scans should be made of every patient with uncommon extrapyramidal disorders, particularly if they do not respond to drug therapy.     

Three patients with hypopituitarism accompanied by primary empty sella presenting mental symptoms

We report here three patients with hypopituitarism accompanied by primary empty sella, whose first manifestations were various mental symptoms. Endocrine studies revealed that two patients showed panhypopituitarism and the other had isolated adrenocorticotropin (ACTH) deficiency. Although several different types of pituitary dysfunctions have been described in a mild form, empty sella is usually asymptomatic. Their first manifestations were mental symptoms; consciousness disturbance, psychomotor agitation, visual hallucination and delusion. Isolated ACTH deficiency is an uncommon disease which etiology is still undetermined. A case with isolated ACTH deficiency associated with an empty sella has been reported before. It is suggested that empty sella might have a role in pathogenesis of isolated ACTH deficiency. The empty sella was confirmed by metrizamide cisternography and magnetic resonance imaging (MRI). These imaging studies are good tools to disclose empty sella. Replacement with cortisone and levothyroxine resulted in an improvement in the mental symptoms in two patients with panhypopituitarism. No alteration was observed following cortisone administration in the patient with isolated ACTH deficiency. Delusion and visual hallucination in this patient poorly responded to treatment with neuroleptics.     

Spontaneous nerve root cerebrospinal fluid leaks and intracranial hypotension: case report

Spontaneous intracranial hypotension is a rare syndrome, characterized by pressure in the cerebrospinal fluid ranging between 50 and 70 mmH2O and postural headache. Its diagnosis is made through the clinical presentation, measurement of the cerebrospinal fluid pressure and neurorimage features. The clinical recognition of this pathology has been increasing and the differential diagnosis must be made with inflammatory meningeal processes and tumor. We report a case of spontaneous nerve root cerebrospinal fluid leaks in a 34 year-old man and intracranial hypotension. A literature review was performed evaluating the clinical, diagnostic and therapeutic aspects of this unusual pathology.     

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。