1/101. Rathke's cleft cyst as a cause of growth hormone deficiency and micropenis.Rathke's cleft cyst has rarely been reported in pediatric patients, and such cysts are usually found by chance, in 2-33% of routine necropsies, as they have not interfered with pituitary function. In general, they are intrasellar with a single layer of ciliated cuboidal or columnar epithelium containing mucoid material. The age range in which symptomatic Rathke's cleft cysts occur is between 30 and 60 years. This paper reports an 8.1-year-old boy presenting with growth hormone deficiency and micropenis attributable to hypogonadotropic hypogonadism (HH), implying altered pituitary function since intrauterine life. At this age (before puberty) the diagnosis of HH can be made by means of the LHRH agonist stimulation test, since conventional LHRH is not able to discriminate HH from a normal prepubertal child. To our knowledge, this is the first case of micropenis caused by Rathke's cleft cyst interfering with gonadotropin and growth hormone secretion since intrauterine life.- - - - - - - - - - ranking = 1keywords = cleft cyst, cyst, cleft (Clic here for more details about this article) |
2/101. Aplasia of right internal carotid artery and hypopituitarism.BACKGROUND: The pathogenesis of congenital hypopituitarism is unknown in many cases. OBJECTIVE: We report a case of congenital pan-anterior hypopituitarism in association with a complex vascular abnormality involving the central nervous system, nasal pyriform aperture stenosis, and a single central maxillary incisor. MATERIALS AND methods: MRI and MRA were used to define this patient's complex vascular anomaly. RESULTS: The vascular abnormality consists of absence of the right common carotid artery, the right internal carotid artery, the A1 segment of the right anterior cerebral artery, the anterior communicating artery, and partial absence of the M1 segment of the right middle cerebral artery. CONCLUSION: This unusual vascular anomaly may contribute to the pathogenesis of some cases of congenital hypopituitarism and related midline abnormalities, or may result from a common defect that causes pituitary insufficiency.- - - - - - - - - - ranking = 0.0030084330664666keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/101. 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.- - - - - - - - - - ranking = 1.1525434096183keywords = cleft cyst, suprasellar, cyst, cleft (Clic here for more details about this article) |
4/101. Panhypopituitarism associated with diabetes insipidus in a girl with a suprasellar arachnoid cyst.We report on a female patient with a large suprasellar arachnoid cyst (3.5 x 2.5 cm) combined with right optic nerve hypoplasia. She developed growth hormone deficiency and hypothyroidism at the age of 8.5 years, adrenal insufficiency at the age of 11 years, diabetes insipidus and hypogonadotropic hypogonadism at the age of 15 years. When last seen at the age of 19 years she was extremely obese ( 5.9 BMI SDS). The endocrine picture suggests that arachnoid cysts might be involved in far more complex hypothalamic-pituitary disturbances than previously thought.- - - - - - - - - - ranking = 0.37982638029872keywords = suprasellar, cyst (Clic here for more details about this article) |
5/101. Cystic lymphocytic hypophysitis, visual field defects and hypopituitarism.A 45-year-old man presented with anterior pituitary failure, requiring thyroxine, hydrocortisone and androgen replacement. An MRI scan revealed a large cystic pituitary mass and thickening of the pituitary stalk. Over three years, diabetes insipidus and bitemporal hemianopia developed and the cystic mass had enlarged on MR scanning. Transphenoidal resection was performed with normalisation of the visual fields. histology revealed lymphocytic hypophysitis, which is rare in men. The presentation with cystic enlargement is unique.- - - - - - - - - - ranking = 0.0030642874938046keywords = cyst (Clic here for more details about this article) |
6/101. Neonatal craniopharyngioma. Case report.The authors report a case of craniopharyngioma in a newborn infant. Marked hydrocephalus of the fetal head was recognized in the pelvimetric roentgenograms of the mother and massive suprasellar calcification was seen in the plain skull films.- - - - - - - - - - ranking = 0.074739561062223keywords = suprasellar (Clic here for more details about this article) |
7/101. Repeated hemorrhage in ciliated craniopharyngioma--case report.A 49-year-old female presented with a ciliated craniopharyngioma manifesting as repeated intratumoral hemorrhage. Histological examination suggested that the hemorrhage originated from the many thin blood vessels in the cyst wall stroma associated with inflammation. Symptomatic hemorrhage in cystic craniopharyngioma may mimic pituitary apoplexy but the etiology is quite different. Minor hemorrhage may recur unless the cyst wall is totally removed.- - - - - - - - - - ranking = 0.0030642874938046keywords = cyst (Clic here for more details about this article) |
8/101. 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.- - - - - - - - - - ranking = 0.85626734643034keywords = cleft cyst, cyst, cleft (Clic here for more details about this article) |
9/101. Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl--a case report.Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincidence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.- - - - - - - - - - ranking = 0.37369780531111keywords = suprasellar (Clic here for more details about this article) |
10/101. Panhypopituitarism associated with severe retroperitoneal fibrosis.A 43-year-old man, with a history of central diabetes insipidus diagnosed 3 years previously, complained about reduced libido. An MRI scan showed a suprasellar lesion just below the supraoptic recess of the third ventricle. A stereotactically guided biopsy revealed fibrous glia, but no other specific tissue and no inflammatory cells. Two months later the patient presented with fatigue and muscular weakness. Tertiary adrenal failure and hypothyroidism were diagnosed by endocrine function tests and therapy with levothyroxine and hydrocortisone was started. Another 2 months later the patient was admitted with giddiness, nausea, peripheral oedema and oliguria. Radiological imaging and an open transperitoneal kidney exploration showed severe fibrosis around both ureters. Histological examination confirmed the diagnosis of idiopathic retroperitoneal fibrosis. Presumably the suprasellar tumour was the first manifestation of retroperitoneal fibrosis. Once the diagnosis 'idiopathic retroperitoneal fibrosis' is confirmed, fibrotic manifestations and complications involving extra-retroperitoneal tissues including the endocrine system, should be sought.- - - - - - - - - - ranking = 0.14947912212445keywords = suprasellar (Clic here for more details about this article) |
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