Cases reported "Empty Sella Syndrome"

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1/160. Extradural balloon obliteration of the empty sella report of three cases (intrasellar balloon obliteration).

    empty sella syndrome is an anatomical and clinical entity composed of intrasellar reposition of the CSF and compression of the pituitary tissue, resulting in a clinical picture of headache, visual field defect, CSF rhinorrhea and some mild endocrinological disturbances. While some cases are primary with no appreciable aetiology, secondary cases are associated with prior operation or radiotherapy of the region. In our series, 3 patients with primary empty sella syndrome were treated by the current approach of extradural filling of the sellar cavity. This technique was first described by Guiot and widely accepted thereafter. We used a detachable silicon balloon filled with HEMA or liquid silicone for obliteration of the sellar cavity and obtained clinically satisfactory results without complications. Visual symptoms regressed and headache disappeared. But at long term follow-up all the balloons were found to be deflated. Despite the facility and efficacy of the technique we do not recommend it in the treatment of the empty sella because the filling of the sella is only transient and relapses may occur.
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2/160. Large empty sella with an intrasellar herniation of an elongated third ventricle. Case report.

    A 73-year-old female presented with a large empty sella with herniation of an elongated third ventricle concomitant with herniation of the surrounding subarachnoid space into the sella, manifesting as visual impairment and amenorrhea without galactorrhea. magnetic resonance imaging and computed tomography cisternography clearly showed the large empty sella, without evidence of either hydrocephalus or benign intracranial hypertension, which is extremely rare.
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3/160. Gitelman disease associated with growth hormone deficiency, disturbances in vasopressin secretion and empty sella: a new hereditary renal tubular-pituitary syndrome?

    Gitelman disease was diagnosed in two unrelated children with hypokalemic metabolic alkalosis and growth failure (a boy and a girl aged 7 mo and 9.5 y, respectively, at clinical presentation) on the basis of mutations detected in the gene encoding the thiazide-sensitive NaCl cotransporter of the distal convoluted tubule. GH deficiency was demonstrated by specific diagnostic tests in both children. Hypertonic saline infusion tests showed a partial vasopressin deficiency in the girl and delayed secretion of this hormone in the boy. magnetic resonance imaging revealed an empty sella in both cases. Up to now, hypomagnesemia and hypocalciuria have been considered obligatory criteria for the diagnosis of Gitelman disease; however, our two patients had hypomagnesemia and hypocalciuria in less than half the determinations. GH replacement treatment was associated with a good clinical response in both children. It appears that these cases represent a new phenotype, not previously described in Gitelman disease, and that the entity may be considered a new complex hereditary renal tubular-pituitary syndrome.
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4/160. The empty sella.

    OBJECTIVE: To discuss the diagnostic imaging findings of an empty sella in a chiropractic patient with emphasis on magnetic resonance imaging (MRI) of normal and abnormal pituitary appearances. CLINICAL FEATURES: A 44-year-old woman started having headache, dizziness, nausea, vomiting, and diarrhea after an argument with her boyfriend. She had been treated for acute torticollis for three weeks when the new symptoms began. Consultation with an internist led to an MRI examination of the cerebellopontine angles to exclude an acoustic neuroma. The MRI demonstrated an enlarged empty sella. There was no history of pituitary tumor or other sellar disease. INTERVENTION AND OUTCOME: There was complete remission of the symptoms after 1 additional dizzy spell that occurred 3 days after the initial symptom. No intervention was performed, but the stress levels in her life had been reduced. CONCLUSION: An enlarged empty sella can be present without symptoms and can represent an incidental finding on radiography and MRI. However, an enlarged sella seen on lateral cervical spine radiographs should prompt further evaluation to rule out pituitary disease. The normal pituitary has a varied appearance and signal intensity on MRI depending on the patient's age and pregnancy status.
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5/160. Adrenal adenocarcinoma and empty sella syndrome in a 37-year-old woman.

    The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. The patient underwent hormonal evaluation including circadian rhythm of cortisol, gonadotropin-releasing hormone/thyroid-stimulating hormone (GnRH/TRH) test, corticotropic-releasing hormone (CRH) test and dexamethasone suppression test. She also underwent pelvic and adrenal ultrasound examination, adrenal computed axial tomography (CAT) scan and cranial nuclear magnetic resonance (NMR). A mass about 10 cm in size was detected in the left adrenal region. The sella was empty and the pituitary displaced downward. Suspected adrenal adenocarcinoma was confirmed by histological examination after surgical removal of the mass. This case is of interest for physicians because of the mixed androgen and cortisol secretion of the adenocarcinoma in a hyperprolactinemic patient with empty sella. Moreover, it suggests the need to investigate the adrenal gland in patients with hyperprolactinemia and hirsutism.
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6/160. empty sella syndrome: does it exist in children?

    OBJECT: The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome. Therefore, the aims of this paper are to increase neurosurgeons' awareness of the existence of this phenomenon in children and to add to the scant body of literature on the subject. methods: The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces. The causes of ESS in these children were high intracranial pressure, neglected or improperly treated hydrocephalus, and suprasellar arachnoid cyst. Primary ESS was found as well. Most of the children presented with headache, abnormal body weight (the majority being underweight), and short stature. The results of hormone assays were normal in all children. CONCLUSIONS: If undiagnosed and untreated, ESS in children may lead to serious consequences, including impairment of pituitary and hypothalamic function and damage to the optic chiasm. It is important to raise awareness in the neurosurgical community about the existence of ESS in children so that it can be diagnosed and treated at an early stage. A classification system for the diaphragma sellae is recapitulated.
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keywords = sella, sella turcica, turcica
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7/160. Isolated thyrotropin deficiency secondary to primary empty sella in a patient with differentiated thyroid carcinoma: an indication for recombinant thyrotropin.

    The primary empty sella syndrome is a common radiographic finding that is rarely associated with clinical pituitary dysfunction. Studies using dynamic endocrine testing, however, have shown altered pituitary reserve in some patients with the primary empty sella syndrome. We describe a patient with a primary empty sella and an isolated deficiency of thyrotropin reserve. This case is complicated by the presence of an aggressive metastatic papillary epithelial carcinoma of the thyroid. Standard treatment with radioactive iodine was unlikely to be effective in this patient due to the need for adequate thyrotropin (TSH) stimulation of the malignant tissue to optimize uptake of radioactive iodine by the tumor cells. Consequently, this patient was treated with human recombinant TSH before receiving radioactive iodine. The utility of this novel therapeutic agent and a review of hormonal abnormalities associated with the primary empty sella are also discussed.
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8/160. Osteopenia in children and adolescents with hyperprolactinemia.

    Three patients with hyperprolactinemia due to pituitary adenomas (two patients) or empty sella (one patient) and osteopenia are described. Their ages at presentation ranged from 8 to 17 years. Each patient was treated with cabergoline. serum prolactin levels became normal in all patients within one month. bone density and pubertal stage improved after 12 months of treatment.
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9/160. adult reversible cardiomyopathy with pituitary adrenal insufficiency caused by empty sella--a case report.

    The authors report a first case of reversible cardiomyopathy in an adult with adrenal insufficiency. A 62-year-old man was administered to hospital because of congestive heart failure. The electrocardiogram showed prolonged QT interval and negative T wave on the right precordial leads and frequent multifocal ventricular premature contractions. On the echocardiogram, the left ventricle was revealed to be diffusely hypokinetic with remarkable enlargement. 201Thallium single photon emission computed tomography (SPECT) revealed multiple flow defects in the left ventricular wall. He also had pituitary adrenal insufficiency secondary to an empty sella, which was diagnosed by hormonal studies and magnetic resonance images of the brain. After the replacement therapy with hydrocortisone, heart failure was sufficiently resolved. Abnormal flow defects in the myocardium on SPECT were also improved. To the authors' knowledge, adrenal insufficiency with cardiomyopathy has not been previously described in adult patients.
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10/160. gonadotropin-releasing hormone-induced partial empty sella clinically mimicking pituitary apoplexy in a woman with a suspected non-secreting macroadenoma.

    pituitary apoplexy has been reported as a rare complication of dynamic testing used for the study of pituitary functional reserve. In 1993, a diagnosis of non-secreting macroadenoma with moderate functional hyperprolactinaemia was made in a 43-year-old woman. Soon after the start of therapy with bromocriptine up to 5 mg/die, the patient complained of nausea and postural hypotension. As the symptoms persisted even when the dose was reduced to 2.5 mg/die, the patient was transferred to therapy with quinagolide at the dosage of 37.5 microg/die. PRL levels quickly normalized (range 1.4-5.7 ng/ml) as well as menstrual cycles, and no side-effect was reported. In 1995 a sellar magnetic resonance imaging (MRI) showed no shrinkage of the known macroadenoma. In 1996, few hours after a gonadotropin-releasing-hormone (GnRH) test, which showed normal LH and FSH response and with baseline PRL levels in the normal range, the patient started complaining of severe frontal headache, nausea and vomiting. No gross visual defects were present. An emergency computed tomography (CT) showed no evident hemorrhagic infarction in the macroadenoma. The symptoms completely resolved in few days with steroidal and antiemetic therapy. A new MRI performed in 1998 showed a partial empty sella and PRL levels were in the normal range under dopaminergic treatment. The pituitary functional reserve proved normal on dynamic testing. The temporal association between the onset of symptoms and the GnRH test strongly suggests an association between the two events. No evident signs of pituitary apoplexy (either on emergency CT or hormonal evaluation) were detected. The authors suggest that GnRH can cause severe side-effects that mimic pituitary apoplexy without related morphological evidence and that, in our particular case, it can have caused the gradual disappearance of the non-secreting macroadenoma. Moreover, a causal role of the chronic dopaminergic treatment cannot be completely ruled out.
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