1/8. Devastating complications from alcohol cauterization of recurrent Rathke cleft cyst. Case report.Rathke cleft cysts are commonly found on autopsy but are seldom symptomatic. Conventional treatment of these lesions consists of transsphenoidal drainage with partial excision of the cyst, and recurrence is rare. Alternatively, the instillation of absolute alcohol into the cyst has been described in several reports, with no complications. The authors report on a woman with Rathke cleft cyst that recurred three times after the initial treatment; the lesion was treated with alcohol cauterization on the final recurrence with devastating complications. This 51-year-old woman presented in 1992 with headaches and visual disturbances. Admission magnetic resonance imaging revealed a sellar/suprasellar lesion that was treated with conventional surgery and was subsequently confirmed to be a Rathke cleft cyst. The patient again presented with recurrence of the cyst at 22, 26, and 31 months after the initial presentation. On the final recurrence the cyst was treated with alcohol cauterization. Postoperatively, the patient awoke blind and suffered a seizure from leakage of the alcohol. The patient remains completely blind in both eyes and is also anosmic and has left lid ptosis and exotropia, which signify damage to the first through third cranial nerves. Rathke cleft cysts have been known to recur after primary surgery; however, this is the first report of a single cyst recurring three times despite conventional surgical treatment. Additionally, this is the first report in which devastating complications from alcohol cauterization of the cyst have been described. The authors therefore advocate caution when attempting alcohol cauterization and advise that meticulous care be taken to ensure the patency of the cyst.- - - - - - - - - - ranking = 1keywords = cleft cyst, cyst, cleft (Clic here for more details about this article) |
2/8. A case of cystic lymphocytic hypophysitis with cacosmia and hypopituitarism.Lymphocytic hypophysitis is a rare inflammatory disease of the pituitary gland that is being increasingly recognized as a cause of hypopituitarism. This condition may be due to an autoimmune pituitary destruction which usually occurs in young women during pregnancy or in the immediate postpartum period. We describe a case of cystic pituitary mass in a thirty-eight year-old woman presenting with nausea, vomiting, cold intolerance, blurring of vision and the presence of disagreeable odors for a one-month period. She had secondary amenorrhea and galactorrhea for three months. Combined anterior pituitary stimulation test confirmed the diagnosis of hypopituitarism. magnetic resonance imaging scan with enhancement showed a huge cystic sellar mass with suprasellar extension and thickening of the pituitary stalk. Transsphenoidal exploration was performed with preoperative diagnosis of pituitary macroadenoma with cystic necrosis. Histological examination revealed lymphocytic hypophysitis characteristic of diffuse, dense lymphocytes and plasma cells infiltration with surrounding interstitial reactive fibrosis. Postoperatively, the patient's olfactory function returned to normal but combined anterior pituitary stimulation test showed persistence of hypopituitarism with mild hyperprolactinemia. prednisolone, thyroxine and estrogen replacements were started and clinical symptoms were much improved. In summary, we report an extremely rare case of a woman with cystic lymphocytic hypophysitis with cacosmia and hypopituitarism, confirmed by histological examination.- - - - - - - - - - ranking = 0.031313679306874keywords = cyst (Clic here for more details about this article) |
3/8. Anosmia in association with occupational use of a waterproof coating chemical.A case of acute permanent anosmia is described in a renovation worker during exposure to a waterproof coating chemical. The chemical consisted of several substances of which four (acetone, acrylates, butyl acetate and carbon disulfide) has been previously reported to induce hyposmia or anosmia in workers. Other aetiologies were clinically excluded but a large arachnoidea cyst in the frontal part of the left temporobasal fossa with possible compression of the left entorhinal cortex. The toxic aetiology of anosmia is supported by the acute onset and the temporal relationship with occupational exposure. The silent cyst as the cause of anosmia is improbable, but it may have had some contributory role. Our case illustrates both the challenges when clinically examining patients with work-related olfactory impairment and the importance of multi-disciplinary approach to such patients.- - - - - - - - - - ranking = 0.0078284198267184keywords = cyst (Clic here for more details about this article) |
4/8. Idiopathic hypogonadotrophic hypogonadism associated with arachnoid cyst of the middle fossa and forebrain anomalies: presentation of an unusual case.We report a 22-yr-old male patient with hypogonadotrophic hypogonadism (HH) associated with a giant middle fossa arachnoid cyst (AC) diagnosed by magnetic resonance imaging (MRI). He presented with pubertal and growth delay. He also had learning disabilities and anosmia. Laboratory investigation revealed pre-pubertal levels of testosterone and normal results of the combined test of anterior pituitary function, except for in GnRH acute and prolonged test. Cranial MRI showed an AC in left middle fossa with expansion to suprasellar cisterna and several abnormalities like left temporal lobe hypoplasia, left optic tract and bilateral olfactory bulb hypoplasia and left hypothalamic hypoplasia.- - - - - - - - - - ranking = 0.019571049566796keywords = cyst (Clic here for more details about this article) |
5/8. Olfactory neuropathy in severe acute respiratory syndrome: report of A case.This case was a 27 years old female with severe acute respiratory syndrome (SARS). She suffered from typical symptoms of SARS. Although she got almost complete recovery from most symptoms after treatment, she noted acute onset complete anosmia 3 weeks after the onset of her first symptom. Her brain MRI examination did not show definite lesion except an incidental finding of left temporal epidermoid cyst. Her anosmia persisted for more than 2 years during following up. Peripheral neuropathy and myopathy have been reported as a concomitant problem during the convalescent stage of SARS, while the sequel of permanent ansomia in SARS was not reported before. Olfactory neuropathy, which rarely occurred in typical peripheral neuropathy, could be a special type of neuropathy induced by corona virus infection in SARS. Olfactory function test should be taken into routine check-up for patients with SARS. The pathophysiology and therapeutic strategy of this special type of permanent olfactory dysfunction deserve further investigation.- - - - - - - - - - ranking = 0.0039142099133592keywords = cyst (Clic here for more details about this article) |
6/8. Paediatric phenotype of kallmann syndrome due to mutations of fibroblast growth factor receptor 1 (FGFR1).kallmann syndrome characterised by hypogonadotropic hypogonadism (HH) and anosmia is genetically heterogeneous with X-linked, autosomal dominant and autosomal recessive forms. The autosomal dominant form due to loss of function mutation in the fibroblast growth factor receptor 1 (FGFR1) accounts for about 10% of cases. We report here three paediatric cases of kallmann syndrome with unusual phenotype in two unrelated patients with severe ear anomalies (hypoplasia or agenesis of external ear) associated with classical features, such as cleft palate, dental agenesis, syndactylia, micropenis and cryptorchidism. We found de novo mutation in these two patients (Cys178Ser and Arg622Gly, respectively), and one inherited Arg622Gln mutation with intrafamilial variable phenotype. These genotype-phenotype correlations indicate that paediatric phenotypic expression of FGFR1 loss of function mutations is highly variable, the severity of the oro-facial malformations at birth does not predict gonadotropic function at the puberty and that de novo mutations of FGFR1 are relatively frequent.- - - - - - - - - - ranking = 0.00083277759142608keywords = cleft (Clic here for more details about this article) |
7/8. A newly recognized neuroectodermal syndrome of familial alopecia, anosmia, deafness, and hypogonadism.We describe a large, three generation kindred in which 16 individuals were affected with alopecia, hyposmia or anosmia, conductive deafness associated with protruding ears, microtia, and/or atresia of the external auditory canal, hypogonadotropic hypogonadism due to LH/FSH deficiency, and a greater than normal tendency to dental caries. Variable manifestations include mild facial asymmetry, mental retardation, congenital heart defect, and cleft palate. This seems to be a previously undescribed pleiotropic autosomal dominant trait with variable expressivity. The manifestations can be explained on the basis of involvement of the ectoderm and neuroectoderm of the first and second branchial arches, of Rathke's pouch, and of the diencephalon.- - - - - - - - - - ranking = 0.00083277759142608keywords = cleft (Clic here for more details about this article) |
8/8. smell impairment. Can it be reversed?patients who have lost the sense of smell usually come to a doctor on their own, reporting loss of the sense of taste. inflammation (often due to allergy), viral infection, and head trauma are common causes of olfactory disturbance. history taking may provide clues to these and other problems (eg, toxin exposure, congenital dysosmia). Workup should not begin until a standardized test has been given that established impairment of the sense of smell. The only truly reversible cause is inflammation, which is confirmed when smell returns after a course of corticosteroid. Sinus computed tomography is necessary to view the olfactory cleft; lack of obstruction indicates that smell impairment is nonreversible. patients deserve an explanation for their disorder and a prognosis. If restoration of their sense of smell is unlikely, patients should be cautioned to take steps to ensure safety in regard to such dangers as gas leaks, smoke, and spoiled foods.- - - - - - - - - - ranking = 0.00083277759142608keywords = cleft (Clic here for more details about this article) |