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1/26. Successful management of sellar and suprasellar arachnoid cysts with stereotactic intracavitary irradiation: an expanded report of four cases.

    OBJECTIVE: Sellar and suprasellar arachnoid cysts may be asymptomatic or may cause headache, optic nerve compression, endocrine dysfunction, or hydrocephalus. We propose a minimally invasive treatment strategy when intervention is indicated. methods: Four patients with sellar and suprasellar arachnoid cysts presented with headache, visual compromise, and endocrine dysfunction. Two of the four patients previously had undergone unsuccessful surgical intervention. The imaging studies of two patients were diagnostic of an arachnoid cyst. RESULTS: All four patients underwent stereotactic intracavitary radiation with cyst regression and symptomatic improvement. In each patient, the optic chiasm was decompressed successfully. There were no complications from the procedure. CONCLUSION: Stereotactic intracavitary irradiation of arachnoid cysts proved to be safe and effective. The procedure obviated the need for open cyst fenestration or shunting.
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2/26. Extradural endodermal cyst of posterior fossa: case report, review of the literature, and embryogenesis.

    OBJECTIVE AND IMPORTANCE: Posterior fossa endodermal cysts are rare. They are located in the midline, in ventral or ventrolateral locations, or intrinsic to the neural axis. Accordingly, various theories of embryogenesis have been proposed. We report the first case of an extradural, dorsolaterally situated endodermal cyst. CLINICAL PRESENTATION: An adult male patient presented with a short history of headache and cerebellar ataxia. neuroimaging revealed an extra-axial cystic posterior fossa mass. INTERVENTION: An entirely extradural cyst was found and was totally excised. immunohistochemistry confirmed the diagnosis of endodermal cyst. CONCLUSION: The extradural, dorsal location of the endodermal cyst suggests gaps at the cranial end of the notochord causing ectodermal-endodermal adhesions during early gastrulation and the persistence of endodermal remnants in the dorsal mesenchyme of the blastemal cranium. The literature is reviewed, and proposed theories of embryogenesis are discussed.
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3/26. syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by rathke's cleft cyst: a case report.

    We report a case of a seventy-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by Rathke's cleft cyst. She experienced nausea, vomiting, diarrhea, and headache and disturbance of consciousness induced by hyponatremia at a serum sodium level of 100 mEq/l. In spite of severe hyponatremia, urinary sodium excretion was not suppressed and serum osmolality (270 mOsm/kg) was lower than urine osmolality (304 mOsm/kg), and arginine vasopressin (AVP) remained within normal range. SIADH was diagnosed because she was free from other diseases known to cause hyponatremia such as dehydration, cardiac dysfunction, liver dysfunction, renal dysfunction, hypothyroidism, and adrenal insufficiency. Cranial computed tomographic (CT) scan and cranial magnetic resonance (MR) imaging showed a cystic lesion of approximately 2 cm in diameter in the pituitary gland. These images suggested that the cystic lesion was a Rathke's cleft cyst, which was the cause of SIADH. water restriction therapy normalized her serum sodium concentration and improved her symptoms. After one year, she suffered from general fatigue, appetite loss, fever, and body weight loss (5 kg/2 months). She had neither hypotension nor hypoglycemia, but her serum sodium level was low and serum cortisol, ACTH, and urine free cortisol were very low. Therefore, secondary adrenal insufficiency was suspected and diagnosed by stimulation tests. After start of hydrocortisone replacement therapy (10 mg/day), her symptoms disappeared. In conclusion, Rathke's cleft cyst should be kept in mind as a potential cause in a patient with SIADH, hypopituitarism, and/or adrenal insufficiency.
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4/26. Endoscopic fenestration of a symptomatic cyst of the septum pellucidum.

    We present the case of a 25-year-old woman suffering from chronic severe headaches and syncope associated with an expanding cyst of the septum pellucidum. After a simple endoscopic fenestration of the cyst the symptoms resolved, resulting in an excellent clinical outcome. This case shows that simple endoscopic fenestration may produce a rapid and maintained symptomatic improvement in patients with an expanding cyst of the septum pellucidum.
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5/26. A case of Rathke's Cleft Cyst inflammation presenting with diabetes insipidus.

    Rathke's Cleft Cyst (RCC), which is located at the intrasellar region, is considered to be the distended remnants of Rathke's pouch, an invagination of the stomodeum. Lined with columnar or cuboidal epithelium of ectodermal origin, RCC usually contains mucoid material and it is found in 13-22% of normal pituitary glands. The cyst rarely leads to the development of symptoms but, when it does, the most common presenting symptoms are headache, visual impairment, hypopituitarism and hypothalamic dysfunction. However, in some cases it presents symptoms of diabetes insipidus, decreased libido and impotence. Recently we experienced a case of RCC inflammation presenting with diabetes insipidus and treated with transsphenoidal surgery. To our knowledge, this is the first report of RCC presenting with symptoms of diabetes insipidus in korea.
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6/26. 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.
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7/26. Endoscopic endonasal removal of an intra-suprasellar Rathke's cleft cyst: case report and surgical considerations.

    An endoscopic endonasal approach was performed to remove an intra-suprasellar Rathke's cleft cyst. Rathke's cleft cyst are benign lesions, rarely diagnosed because they are often asymptomatic. To the best of our knowledge, at least 475 cases of Rathke's cleft cysts have been reported. They seem to arise from remnants of Rathke's pouch, an invagination of the stomodeum. A 52-year-old woman, complaining of bilateral frontal headaches, was operated on by using an endoscopic endonasal approach, for an intra-suprasellar tumor. The pre-operative diagnosis was non-functioning pituitary adenoma. Intra-operatively a creamy-coloured viscous tissue was found. After the removal of the cyst contents and of the capsule, the suprasellar structures were seen well. The chiasmatic cistern, the chiasm, the pituitary stalk and the pituitary gland were visualized with 0 and 30 degree endoscopes. The pathological findings showed a well-differentiated cuboidal epithelium. The diagnosis was Rathke's cleft cyst. No post-operative complications were observed. The endoscopic technique was particularly suitable in this case, both for the Rathke's cleft features and for an excellent outcome. The Rathke's cleft cyst was easily removed by suction and the cyst wall was entirely removed with curettes and pituitary punches. The hypophysis was distinguished from the cyst and was preserved. The surgical manoeuvres were all done under direct visual control. The absence of nasal packing and of breathing difficulties made comfortable the post-operative outcome. Thus, the endoscopic endonasal approach can be considered the favourite technique in case of either intra- and/or suprasellar Rathke's cleft cysts.
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8/26. Tension pneumocyst after transsphenoidal surgery for Rathke's cleft cyst: case report.

    OBJECTIVE AND IMPORTANCE: Tension pneumocephalus is a rare but well-described complication of transsphenoidal surgery. It is usually associated with postoperative cerebrospinal fluid fistulae causing lower intracranial pressure, with air located in the subdural, subarachnoid, or intraventricular space. We report a case of suprasellar tension pneumocyst that caused visual deterioration to develop after an operation for a Rathke's cleft cyst. Only one similar case has been reported previously. CLINICAL PRESENTATION: A 54-year-old woman with a cystic sellar-suprasellar mass compressing the chiasm was operated on via a standard transsphenoidal approach. The intraoperative diagnosis was Rathke's cleft cyst, and the floor of sella was left open to avoid recurrence. The sphenoid sinus was filled with a fat graft, and the rostrum of the sphenoid was reconstructed with a bone fragment. The patient's postoperative course was uneventful, and her vision improved. Ten days after discharge, the patient was readmitted to the emergency service with headache and visual impairment. Emergent computed tomography confirmed a suprasellar tension pneumocyst. INTERVENTION: The patient underwent immediate reoperation via an endonasal endoscopic approach. After the trapped air was evacuated, the sella was closed with fascia lata and muscle using fibrin glue. The patient's vision improved postoperatively. CONCLUSION: Suprasellar tension pneumocyst is an extremely rare complication of transsphenoidal surgery. To avoid this complication, the sellar floor should be repaired in a watertight fashion, and patients should be instructed to avoid blowing the nose, sneezing, straining, and coughing postoperatively.
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9/26. Rathke's cleft cyst presenting with hyponatremia and transient central diabetes insipidus.

    We describe an 18-year-old female who complained of general weakness, nausea, vomiting, headache, and lightheadedness. On physical examination, she was euvolemic without visual or neurological deficits. The striking biochemical abnormality was hyponatremia (125 mmol/l). This hyponatremia met the laboratory diagnostic criteria for the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Two litres of normal saline were given per day for 4 days and this did not correct her hyponatremia. A spontaneous diuresis (6.6 l) developed in 1 day, causing a rise in her PNa of 26 mmol and a final PNa of 152 mmol/l. magnetic resonance imaging revealed a dumbell-shaped intrasellar and suprasellar cyst. During transsphenoidal surgery, a Rathke's cleft cyst (RCC) lined with columnar epithelium containing mucoid material was resected. We speculate that the growing RCC may have produced critical compression over the stalk, thus contributing to the transition from SIADH with hyponatremia to transient central diabetes insipidus with hypernatremia.
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10/26. Congenital predisposition to spontaneous intracranial hypotension: a case report.

    Dural rupture, cerebrospinal fluid leakage, and spontaneous intracranial hypotension may complicate significant or minimal spinal trauma and cause chronic headache with a positional component. While such cases typically reflect no pre-existing predilection, we encountered a patient whose cervicothoracic anatomy appeared to predispose him to this complication.
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