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Glaucomatous optic neuropathy and field loss in primary empty sella syndrome.

Primary empty sella syndrome is associated with certain visual field defects. Typical glaucomatous field defects, however, have rarely been reported. We reviewed eight cases of primary empty sella syndrome. Seven patients had typical glaucomatous optic disc and visual field changes. Three patients had low-tension glaucoma and four had typical primary open-angle glaucoma. We recommend that in addition to neurologic and endocrinologic assessment, patients with primary empty sella syndrome and visual field loss should have a detailed ophthalmologic examination, particular attention being paid to intraocular pressure and optic disc appearance to rule out glaucoma as a cause of the field loss.

Adult↗

[Empty sella syndrome].

An empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid (CSF). Empty sella is occasionally found as a normal anatomical variation, which is referred to as primary empty sella. On the other hand, empty sella is also seen after surgery, irradiation or medical treatment of pituitary adenoma, which is called secondary empty sella. Magnetic resonance imaging (MRI) is useful in diagnosing empty sella. Primary empty sella is usually free from clinical symptoms but sometimes associated with headache, obesity, visual disturbance, non-traumatic CSF rhinorrhea and pituitary insufficiency. These associated findings constitute the empty sella syndrome. CSF rhinorrhea, visual disturbance and severe increased intracranial pressure are the indications for surgical treatment. Non-symptomatic cases require no treatment but periodical follow up is necessary.

Adenoma↗

Familial pseudotumor cerebri and the empty sella syndrome.

The empty sella syndrome may be a later complication of pseudotumor cerebri, based on the relationships of clinical features of both and their visual complication. Pseudotumor cerebri may follow a genetic predisposition but further research is needed to confirm this.

Adult↗

The empty sella syndrome.

The empty sella syndrome (ESS) presents a varied clinical and radiographic picture. It may remain asymptomatic or may stimulate an intrasellar growth thereby causing diagnostic and therapeutic problems. An air encephalogram (AEG) is required for diagnosis. The purpose of this paper is to review the clinical and radiological features of the ESS and to discuss the pathogenetic mechanisms involved.

Humans↗

[A case of Hashimoto's thyroiditis associated with renal tubular acidosis, Sjögren syndrome and empty sella syndrome].

This report describes a 48-year old female patient with Hashimoto's thyroiditis, distal-type renal tubular acidosis (d-RTA), Sjögren syndrome (SjS), and empty sella syndrome (ESS). She has been receiving replacement of thyroxine for Hashimoto's thyroiditis since 1967. She felt muscle weakness and numbness in the extremities and was found to have low serum potassium (2.9 mEq/l) in 1987. Since then she has been administrated potassium chloride orally. She was admitted to our hospital because of recurrence of muscle weakness and numbness of the extremities in November 1990. Laboratory examination revealed that her serum levels of antimicrosomal antibody and anti-thyroglobulin antibody were highly positive (MCHA: x 2(10) x 100, and TGHA: x 100). Furthermore, she was revealed to have 1) d-RTA by oral tolerance tests with the administration of NH4Cl and NaHCO3, 2) SjS by Schirmer test and sialography, and 3) ESS by computed tomography and magnetic resonance imaging examinations of the pituitary. Association of Hashimoto's thyroiditis, d-RTA, SjS and ESS in this case may possibly be caused by common autoimmune mechanism.

Acidosis, Renal Tubular↗

CSF rhinorrhea associated with the empty-sella syndrome.

The empty-sella syndrome consists of the abnormal extension into the sella turcica of an arachnoid diverticulum filled with CSF, which displaces and compresses the pituitary gland. Such a diverticulum can erode through the sellar floor and lead to CSF rhinorrhea through the sphenoidal sinus. Empty-sella syndrome should be considered a diagnostic possibility in patients with nontraumatic CSF rhinorrhea. Diagnosis and treatment are best accomplished with a team approach involving the otolaryngologist, neurosurgeon, neuroradiologist, and neuroendocrinologist. Definitive diagnosis requires pneumoencephalography and thin-section polytomography. Attention should be directed to the possibility of a coexisting pituitary adenoma. Treatment can be accomplished by a transseptal, transsphenoidal approach with localization and repair of the leak.

Adult↗

Empty sella syndrome presenting as galactorrhoea.

A prolactin secreting tumour is the commonest cause of the amenorrhoea-galactorrhoea syndrome. Galactorrhoea is a rare presentation of an empty sella syndrome. The empty sella syndrome commonly presents with headache and visual impairment and occasionally with endocrine disturbances in hypertensive middle aged women. The authors present a case of hyperprolactinemia resulting in galactorrhoea in a middle aged lady associated with a primary empty sella syndrome.

Empty Sella Syndrome↗

Primary empty sella syndrome and amenorrhea.

The empty sella syndrome is defined anatomically and radiologically. A case report of an amenorrheic patient who was diagnosed as having the primary empty sella syndrome is presented. Its pathogenesis, clinical profile, and endocrine relationships are discussed. Specific reference is directed toward the capability of the empty sella syndrome to produce pituitary impairment and possibly amenorrhea.

Adult↗

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.

Adolescent↗

[Case report on the empty sella syndrome].

The patient with "empty sella" syndrome was dealt with in this paper. This syndrome represented a remarkable differential diagnostic problem and was treated surgically.

Adult↗

The clinical and radiological features of the empty sella syndrome.

The importance of the empty sella syndrome has increased with recent interest in pituitary micro-adenomas, trans-sphenoidal hypophysectomy and computerised tomography. Four cases of the empty sella syndrome which illustrate the clinical and radiological features are reported, and the literature is reviewed. Headache, visual symptoms and endocrine disturbances are commonly found in association with the empty sella syndrome, and the coexistence of pituitary micro-adenomas is well recognised. Symmetrical ballooning of the sella turcica without changes in configuration is the typical finding with plain radiography, but occasionally more extensive destructive changes in the region of the pituitary fossa may occur. Provided that the correct angulation, collimation and level of cut are chosen, computerised tomography now may be used to make a definitive diagnosis, making air encephalography unnecessary.

Adenoma↗

Empty sella syndrome in an adolescent.

Empty sella syndrome (ESS) is a condition in which the sella turcica is partially or completely filled with cerebrospinal fluid resulting in a displacement of the normal pituitary. The condition is more common and benign in adults, although associated endocrine abnormalities have been reported. In children and adolescents, the syndrome is rare (27 reported cases), and the clinical picture is much less benign, with an increase in familial incidence, associated skeletal disorders, and endocrine abnormalities. Definitive diagnosis can be made by CT scan of the head. Our case report is of an adolescent who appeared clinically to have growth retardation and normal endocrine function. The CT scan of the head was compatible with ESS. Further follow-up showed improved growth rate. Although our patient did not appear to have symptoms related to ESS, based on the literature we agree that this syndrome is much less benign in children and adolescents than in adults.

Brain↗

Transsphenoidal chiasmopexy for long standing visual failure in the secondary empty sella syndrome.

The development of an empty sella syndrome is a well recognized complication in the treatment of pituitary tumours and usually presents with visual failure. Transsphenoidal chiasmopexy has been successful in reversing the visual loss when performed in the acute stage. A case, where a delay of 18 months in the surgery does not appear to have affected the outcome, is presented and the literature reviewed.

Adenoma↗

[43 cases of primary empty sella syndrome: a case series].

Primary empty sella syndrome (ESS) is an anatomo-radiological picture characterized by the presence of an arachnoid herniation filled with liquor that compresses the pituitary against the sellar wall. ESS occurs particularly in obese, hypertensive, cephalalgic women. It is often asymptomatic but may be associated with ophthalmologic, neurologic and non-characterizing endocrine disorders. We report here 43 cases of primary ESS observed and assessed in our Departments of Internal Medicine from June 1983 to May 1993. The following endocrinological diagnostic procedures were carried out: hormonal (RIA) basal profile: FT3, FT4, TSH, PRL, ACTH, FSH, LH, 8.00 a.m. and p.m., blood cortisol, aldo, PRA, DHEA-S, FTe, E2, P, PTH, CT, and calcemia and phosphoremia; provocative tests: TRH, GnRH, etc.; inhibition tests: high dose dexamethasone. Clinical, neurologic (skull radiographs, sellar stratigraphy, computed tomography scan and magnetic resonance), and ophthalmologic (fundus, visual fields) assessments were also made. Our findings fit with the data in the literature concerning common symptoms of ESS, associated endocrinopathies and other illness. We found obesity (62.7%), oligo-amenorrhea (16.6%), galactorrhea (14.6%), hyperPRL (11.6%), hypopituitarism (9.3%), hypogonadism (4.6%), diabetes insipidus (2.3%), (micro-)polycystic ovary syndrome (19%), hyperACTH (2.3%). In 9.3% of the cases, endocrinopathy referred to pituitary adenomas. Moreover, we noted a high frequency of psychological disorders, to our knowledge not previously reported in the literature, including anxiety or dysthymic disorders with altered behavior (chiefly oral compulsion). We also make the hypothesis that obesity (occurring in 62.7% of our patients) and hypertension (62.7%) may be related to hypothalamic alterations.

Adult↗

No evidence for autoimmunity as a major cause of the empty sella syndrome.

OBJECTIVE: The cause of empty sella syndrome (ESS) remains largely unknown. We measured eleven organ-specific autoantibodies in serum in order to evaluate possible autoimmune components in ESS. PATIENTS: Thirty patients with ESS and 50 healthy blood donors participated in the study. MEASUREMENTS: Detection of pituitary autoantibodies was performed by immunoblotting with human pituitary cytosol as antigen. Thyroid peroxidase (TPO) and TSH receptor (TRAK) autoantibodies were analysed by radioimmunoassay. The remaining eight autoantibodies were detected by in vitro transcription and translation of the autoantigens and immunoprecipitation. RESULTS: The majority of the ESS patients (18/30) exhibited no immunoreactivity at all. None of the remaining 12 ESS patients reacted against more than one autoantigen. No immunoreactivity was found more frequently among ESS patients than healthy blood donors. Pituitary autoantibodies were not correlated to the ESS patients' pituitary function or sellar size, although the results indicated a tendency of increased autoimmunity in patients with hypopituitarism and normal sella size respectively. CONCLUSION: Detection of autoantibodies is a valuable tool in the diagnostic work-up of autoimmune diseases. By analysing a large number of organ-specific autoantibodies we found no evidence of ESS being associated with any specific autoimmune disease. The pathogenesis of ESS is believed to be heterogeneous and our findings suggest autoimmune components to be of minor importance. In some selective cases, ESS in combination with hypopituitarism may be the result of an autoimmune disease in the pituitary gland but this needs further investigation.

Adult↗