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1/30. hyponatremia-induced metabolic encephalopathy caused by Rathke's cleft cyst: a case report.

    Rathke's cleft cysts are sometimes associated with aseptic meningitis or metabolic encephalopathy due to hyponatremia. We treated such a case manifest by lethargy, fever and electroencephalographic abnormalities. A 68-year-old man was admitted to our ward after experiencing general malaise, nausea and vomiting and then high fever and lethargy. On admission, he was drowsy and had nuchal rigidity and Kernig's sign. Physically, he was pale with dry, thickened skin. He had lost 5.0 kg of body weight in the last month. His serum sodium was 115 mEq/l. He had a low serum osmotic pressure (235 mOsmol/l) and a high urine osmotic pressure (520 mOsmol/l). His urine volume was 1200-1900 ml/24 h with a specific gravity of 1008-1015. The urine sodium was 210 mEq/l. He did not have an elevated level of antidiuretic hormone. Electroencephalograms showed periodic delta waves over a background of theta waves. With sodium replacement, the patient become alert and symptom free, and his electroencephalographic findings normalized. However, the serum sodium level did not stabilize, sometimes falling with a recurrence of symptoms. magnetic resonance imaging clearly delineated a dumbbell-shaped intrasellar and suprasellar cyst. The suprasellar component subsequently shrunk spontaneously and finally disappeared. An endocrinologic evaluation showed panhypopituitarism. The patient was given glucocorticoid and thyroxine replacement therapy, which stabilized his serum sodium level and permanently relieved his symptoms. A transsphenoidal approach was performed. A greenish cyst was punctured, and a yellow fluid was aspirated. The cyst proved to be simple or cubic stratified epithelium, and a diagnosis of Rathke's cleft cyst was made. The patient was discharged in good condition with a continuation of hormonal therapy. Rathke's cleft cyst can cause aseptic meningitis if the cyst ruptures and its contents spill into the subarachnoid space. Metabolic encephalopathy induced by hyponatremia due to salt wasting also can occur if the lesion injures the hypothalamus and pituitary gland.
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2/30. Transsphenoidal supradiaphragmatic intradural approach: technical note.

    Presellar extension of the bone window combined with removal of the sellar floor results in the transsphenoidal supradiaphragmatic intradural approach. One tuberculum sella meningioma and another suprasellar Rathke's cleft cyst confined to the pituitary stalk were removed via this approach. The presellar extension of the bone window was performed with the sublabial transseptal transsphenoidal technique. Furthermore, the dissection of the anterior intercavernous sinus, diaphragma sella, and arachnoid trabecula has allowed a wide surgical field of pre- and suprasellar areas and facilitates safe removal of lesions without significant surgical complications in selected cases.
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keywords = cleft
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3/30. Rathke's cleft cyst with large frontal extension: unusual shape and pathomechanism of its formation.

    The patient was a 62 year old woman who presented with a visual field defect. Magnetic resonance images showed an intra-and supra-sellar low intensity mass on T1 weighted images. The preoperative diagnosis was craniopharyngioma, Rathke's cleft cyst (RCC) or arachnoid cyst. The patient underwent transsphenoidal surgery & the pathological diagnosis was RCC.We conclude that although RCC has a round, ovoid or dumb-bell shaped configuration, it may present a unusual shape in cases with preconditions, as were seen in our patient.
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keywords = cleft
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4/30. Rathke's cleft cyst associated with hypophysitis: MRI.

    We report a symptomatic Rathke's cleft cyst associated with hypophysitis in a 61-year-old woman. We demonstrate the MRI features and discuss the pathophysiology. To the best of our knowledge this is the first description of a Rathke's cleft cyst shrinking after high-dose steroid therapy.
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keywords = cleft
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5/30. 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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6/30. Rathke's cleft cyst abscess.

    Pituitary abscesses are rare. Occasionally they will arise in pre-existing pituitary pathology. We report such an occurrence within a Rathke's cleft cyst. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology.
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keywords = cleft
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7/30. Rathke's cleft cyst presenting as sphenoid sinusitis.

    Rathke's cleft cysts are developmental abnormalities of the craniopharyngeal duct composed of retained stratified ciliated cuboidal cells of the respiratory type. Incidentally found in the pars intermedia in 13% to 33% of routine autopsies, they are discovered in growing numbers on CT and MRI scans. From 1991 to 1999, 9 cases of Rathke's cleft cysts were encountered by the senior authors, 4 of which were treated surgically (3 by using a transsphenoidal approach and one by using a transcranial subfrontal approach). Of the 3 patients treated with a transsphenoidal approach, 2 presented with symptoms mimicking sphenoid sinusitis and were initially referred for otolaryngologic evaluation. The clinical, pathologic, and radiologic features, as well as management and follow-up of those 2 patients, are reviewed. There has been only one previous case report of Rathke's cleft cysts presenting as sinusitis. Nevertheless, the otolaryngologist should be aware of this condition because it may present with other significant symptomatic extracranial extensions.
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8/30. Tentorial enhancement on MR images is a sign of cavernous sinus involvement in patients with sellar tumors.

    BACKGROUND AND PURPOSE: This study was undertaken to analyze enhancement patterns of the dura around sellar tumors and to compare the results with tumor invasion or compression of the cavernous sinuses. Postoperative enhancement patterns on MR images were compared with preoperative findings. methods: Contrast-enhanced coronal and sagittal MR images were examined prospectively in 96 patients with sellar tumors (65 macroadenomas, 15 microadenomas, 14 Rathke cleft cysts, and two chordomas at the sella). All patients underwent surgical treatment, and pre- and postsurgical features on MR images were compared. RESULTS: Presurgical MR images showed dural enhancement in 36.5% of the patients: asymmetric tentorial enhancement in 24 patients, symmetric tentorial enhancement in seven, and sphenoidal ridge or clivus enhancement in four. Asymmetric tentorial enhancement disappeared after surgical decompression in seven patients. For evaluation of cavernous sinus invasion ipsilateral to the enhancement, sensitivity and specificity of the asymmetric tentorial enhancement sign were 81.3% and 86.3%, respectively. sensitivity and specificity of the sign were 42.9% and 93.6% for cavernous sinus involvement, including compression and invasion. CONCLUSION: Asymmetric tentorial enhancement is a useful sign in the diagnosis of invasion or severe compression of the cavernous sinus by sellar tumor. The sign may represent venous congestion or collateral flow in the tentorium due to obstructed flow in the medial portion of the cavernous sinus.
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keywords = cleft
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9/30. An entirely suprasellar Rathke's cleft cyst: case report and review of the literature.

    Rathke's cleft cysts usually present with an intrasellar or a combined intrasellar and suprasellar localization. In this report we present a 58-year-old man with a Rathke's cleft cysts in an entirely suprasellar location with intact diaphragm and intact sella. Although this presentation of a Rathke's cleft cyst is extremely rare, it belongs in the differential diagnosis of entirely suprasellar lesions. The relevant literature is discussed.
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10/30. 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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keywords = cleft
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