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.     

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.     

Need for intrasellar packing in sellar reconstruction of transsphenoidal surgery: Less is more?

BACKGROUND: This study investigated patients with pituitary macroadenomas who received transsphenoidal surgery using two different sellar reconstruction methods, to determine the need for intrasellar packing. METHODS: The authors reviewed 53 patients with pituitary macroadenomas at a single institution between January 1999 and November 2002. On tumour removal, the group 1 patients underwent traditional sellar reconstruction (intrasellar and sphenoid sinus packing), while the group 2 patients did not undergo intrasellar packing. RESULTS: The two groups did not differ significantly in terms of complications. There was no mortality; notably, no delayed empty sella syndrome occurred in the group 2 patients. CONCLUSIONS: Simplified reconstruction of the sella floor using only sphenoid bone without intrasellar packing is thought to be safe and effective. This technique obviates the need for a second surgical incision, risk of overpacking, complications associated with packing material and interference on postoperative magnetic resonance imaging.     

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.     

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.     

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.     

Adult hydrocephalus and the empty sella

Abstract– A 51-year-old man presented with a pituitary apoplexy with symptoms and signs predominantly of meningitis. Later on, hydrocephalus and an empty sella were disclosed. It is likely that the empty sella and the hydrocephalus were both caused by the adenoma necrosis. Based on this case and cases reported in the literature, it is suggested that concomitant presence of an empty sella and hydrocephalus results from necrosis in a pre-existing pituitary adenoma.     

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.     

Intrasellar syndrome. Analysis of 506 cases of transsphenoidal surgery

The author has performed 506 transsphenoidal surgeries but has analyzed in this study only 404 cases. There were 396 hypophyseal tumors, 4 intrasellar cysts and 4 empty sellas. Based in the symptoms presented by the patients he describes an "intrasellar syndrome", characterized by headache, visual disorders and hormonal abnormalities. He calls attention for intrasellar tumors with normal sella, as seen by x-ray. The diagnosis of these tumors were made only by endocrinological studies. At last he shows the advantages of transsphenoidal surgery in this pathology and the excellent results obtained. The mortality of his cases was only 0.2%.     

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.     

Coexistence of ectopic pituitary adenoma and empty sella in a patient with acromegaly : a case report and review of literature

Ectopic pituitary adenoma with an empty sella is extremely rare. We report an unusual patient with an ectopic growth hormone-secreting pituitary adenoma in the sphenoid sinus with an empty sella. The association is related to a development disorder of the anterior pituitary tissues. Tumor in the sphenoid sinus was completely removed by endoscopic endonasal transsphenoidal approach. During the follow-up, the patient met the criteria for endocrinological cure.     

Ectopic Pituitary Adenoma Associated with an Empty Sella: A Case Report and Review of the Literature

A case of ectopic pituitary adenoma in the sphenoid sinus associated with an empty sella is reported. The coexistence of an ectopic pituitary adenoma and an empty sella is quite rare. The diagnosis was made with an intraoperative finding of the intact dura mater of the sellar floor. In the present case, the hypointense line that coated the pituitary gland was clearly demonstrated on 3‐tesla T2‐weighted magnetic resonance imaging. The hypointense line is considered to be the pituitary capsule and was critical in diagnosing this rare entity.     

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.     

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.     

Spontaneous pneumoventriculogram following radiation of a pituitary adenoma.

Three years after radiation therapy for an intrasellar tumor, a 42-year-old housewife presented with headache, lethargy, and remarkable plain skull roentgenograms, in which dilated lateral and third ventricles were filled with air. Air apparently had entered the cranium through the sphenoid sinus and eroded sellar floor, extending directly through intrasellar remnants of the chromophobe adenoma and into the floor of the third ventricle. Frontal exploration showed an empty sella turcica and no residual tumor. She made an excellent recovery and has done well for 5 years after operative closure of the defect.     

Surgically treatable growth retardation due to non-neoplastic pituitary-hypothalamic dysfunction

Out of 90 children, examined because of growth failure, 15 have been treated surgically. The diagnoses were intrasellar arachnoid diverticulum or empty sella (5 cases), enlarged chiasmatic cisterns (5 cases), chronic 'occult' hydrocephalus (5 cases). Surgery was followed by an immediate increase in growth rate in almost all the cases, even if the result was persistent only in some subjects. In percentage, better results were obtained in patients with enlarged chiasmatic cisterns and chronic occult hydrocephalus than in patients with arachnoid diverticulum or empty sella. The evaluation of prolactin serum levels was demonstrated to be useful both in preoperative diagnosis and postoperative control.     

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.     

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.     

Rathke's cleft cyst presenting as pituitary apoplexy.

Sellar lesions mainly constitute pituitary adenomas, craniopharyngiomas and benign cysts. Rathke's pouch cyst is a developmental sellar and/or suprasellar cystic lesion lined by a single layer of ciliated cuboidal or columnar epithelium, which rarely be comes symptomatic. The authors present an interesting case of intrasellar Rathke's pouch cyst, with a presenting feature of acute pituitary apoplexy. This was a 19 year old healthy male who had developed sudden headache and visual disturbance. Neuro-radiological imaging revealed a mass in the sella. Via transsphenoidal approach a haemorrhagic intrasellar cystic lesion was removed and was confirmed as a haemorrhagic Rathke's cleft cyst by histopathological examination. Interesting clinical presentations and the neuroimaging findings are described and discussed.