Cases reported "Empty Sella Syndrome"

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1/22. 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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2/22. 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.
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3/22. Spontaneous cerebrospinal fluid rhinorrhea associated with chronic renal failure--case report.

    A 39-year-old woman was admitted with complaints of headache and nasal discharge on the left for 3 months which was later on proved to be cerebrospinal fluid (CSF). Neurological examination found no abnormalities except bilateral papilledema. neuroimaging demonstrated enlargement of the lamina cribrosa foramina through which the olfactory nerves pass, as well as empty sella and cerebral cortical atrophy. Bone mineral densitometry showed osteopenia. CSF Ca and blood parathyroid hormone levels were elevated. CSF pressure was 280 mmH2O. Bilateral frontal craniotomy was performed to expose the anterior fossa. Foraminal enlargement at the lamina cribrosa was confirmed, and islands of extra-osseous calcifications on the arachnoid membrane were identified. The base of the anterior fossa was repaired intradurally with fascial graft and fibrin glue on both sides. No CSF leakage was noted at 1-year follow up. Spontaneous CSF leakage probably resulted from enlargement of the foramina at the lamina cribrosa due to Ca mobilization from bones and pseudotumor cerebri not to the extent of hydrocephalus caused by poor CSF absorption at the arachnoid granulations obliterated by extra-osseous calcareous accumulation.
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4/22. 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.
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5/22. association of Bartter's syndrome and empty sella.

    Bartter's syndrome is characterized by hypochloremia, hypokalemia, metabolic alkalosis associated with renal potassium leakage, and normal blood pressure despite increased plasma renin activity. Although association of empty sella with gitelman syndrome has been reported, no association has been reported with Bartter's syndrome. Here we report a patient with Bartter's syndrome and empty sella. A 12 month-old male patient presented with a history of nausea, vomiting, abdominal distension, constipation, and edema in the lower extremities that had begun in the early postnatal period. The patient was born at 32 weeks gestation by operative delivery for polyhydramnios. blood pressure was normal. serum sodium, potassium, calcium, phosphate, chloride, albumin and alkaline phosphatase levels were 129 mEq/l, 2.5 mEq/l, 9 mg/dl, 3.8 mg/dl, 72 mg/dl, 4.2 g/dl and 1285 IU/l, respectively. serum magnesium level was normal. Arterial blood gas levels revealed pH 7.55 (normal, 7.35-7.45), PCO2 33.6 mm/Hg (36-46), base excess 7.1 ( /- 2.3), and total CO2 33.6 mmol/l (23-27). renin and aldosterone levels were elevated. urine had pH 8.0 and specific gravity 1.010. Urinary calcium excretion was 22.8 kg/day (urine calcium/creatinine ratio 0.46). Urinary potassium and chloride levels were elevated. MRI of the brain was normal except for partially empty sella. We present the first pediatric patient with the association of Bartter's syndrome and empty sella.
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6/22. 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.
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7/22. 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.
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8/22. A new variety of "empty sella" with cystic intrasellar dilatation of the recessus infundibuli.

    A new variety of the "empty sella" consisting of polycystic intrasellar dilatation of the infundibular recess is described. The underlying embryonal developmental anomaly of the pituitary gland and diaphragma sellae and the possible effect of increased intracranial pressure are discussed and the malformation is compared with embryonal stages of ventricular development.
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9/22. A case of 'low tension glaucoma' with primary empty sella.

    A case of 'low tension glaucoma' with primary empty sella is reported. The visual field defect and optic disc change were characteristic of glaucoma. The intraocular pressure was within normal limits. X-ray examination and the metrizamide-CF procedures revealed a primary empty sella. The coexistence of 'low tension glaucoma' and empty sella is discussed.
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10/22. Primary empty sella syndrome and benign intracranial hypertension.

    The combination of the empty sella syndrome (ESS) and benign intracranial hypertension (BIH) is illustrated by two case histories. The causal relationship between the ESS and the BIH can be explained by two mechanisms. Raised intracranial pressure could produce a herniation of the subarachnoid cistern into the sella turcica, if the diaphragma sellae is incomplete. Alternatively an infarction in a pituitary adenoma could result in both an ESS and cerebrospinal fluid flow obstruction, which could lead to BIH.
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