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.     

Empty sella syndrome]

The authors present two middle-aged female patients with empty sella revealed at imaging studies (CT, MRI). Their main complaint was severe fronto-parietal and fronto-temporal headache. Physical examination showed obesity, hypertension, and local hypersensitivity on deep palpation and percussion in the above-mentioned regions, in both cases. Endocrine function of pituitary gland, visual fields and fundi were normal as was EEG. The CSF composition and pressure also showed no abnormalities. The diagnostic and therapeutic problems of empty sella syndrome are discussed.     

Empty sella syndrome in childhood

The empty sella syndrome is common in middle-aged women, usually presenting with headache, and only occasionally associated with endocrine or visual abnormalities. It is rare in childhood. Childhood cases tend to present either with endocrine disturbances, visual symptoms, or with craniofacial syndromes. We present three cases of complete empty sella with childhood onset, each discovered unexpectedly during evaluation of endocrine or visual dysfunction.     

Treatment of empty sella syndrome with ventriculoperitoneal shunt.

A symptomatic empty sella developed in a female patient undergoing bromocriptine therapy for microprolactinoma. Placement of a ventriculoperitoneal shunt dramatically improved the symptoms of headache and blurred vision. The post-operative imaging showed resolution of the empty sella. She was able to resume bromocriptine therapy without recurrence of her previous symptoms and give birth to a baby 20 months later. An MRI 44 months after surgery and on bromocriptine therapy showed no recurrence of the empty sella. We conclude that ventriculoperitoneal shunt may be a simple, and durable treatment for drug induced empty sella and allows resumption of bromocriptine therapy for preexisting microprolactinoma.     

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.     

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.     

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.     

侵袭性催乳素腺瘤治疗后海绵窦残留或伴空蝶鞍综合征

目的 探讨侵袭性催乳素(PRL)腺瘤经治疗后海绵窦残留伴空蝶鞍综合征的临床特征和治疗策略.方法 18例侵袭性PRL腺瘤(Knosp分级Ⅲ或Ⅳ级)经治疗后出现海绵窦肿瘤持续残留,首选药物治疗者9例,首选手术治疗结合药物和(或)放疗9例.结果 平均随访55个月,8例PRL水平正常,7例视力改善.14例因鞍内肿瘤吸收遗留空蝶鞍,7例PRL仍升高,其中5例有不同程度的视交叉下疝(P<0.05).PRL水平正常、无临床症状的7例患者采用低剂量溴隐亭治疗达到PRL水平和肿瘤体积的长期控制.结论 PRL腺瘤治疗后海绵窦肿瘤残留伴发空蝶鞍,这一现象应该引起高度重视,尤其对伴有视交叉下疝的病例,长期的密切随访是必需的.无内分泌和压迫症状的海绵窦残留病例可以采用低剂量的药物治疗达到长期的肿瘤控制和内分泌控制.     

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.     

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.     

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.     

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.     

Magnetic Resonance Imaging in Empty Sella Phenomena

Of 500 consecutive unselected patients who underwent brain magnetic resonance imaging (MRI), 20 (12 females, 8 males; mean age, 41 yr) were found to have empty sella. The clinical and imaging findings were compared to those in 20 normal adult control subjects, and 20 patients of comparable age and sex from the group of 500 consecutive patients. Variations in the pituitary infundibulum, the optic chiasm, and the sellar floor did not correlate to symptoms. Little difference in clinical symptomatology between patients with empty sella and control subjects was found. It is concluded that empty sella is often an insignificant finding in adults.     

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.     

Epilepsy and tremor in the XXY syndrome]

Extrapyramidal tremor and epilepsy uncommon in Klinefelter syndrome were found in a 30 year old patient. CT scans revealed partially empty sella and subcortical cerebral atrophy. Chromosomal studies showed the karyotype 47, XXY.     

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.     

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.