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      Alterações visuais decorrentes do cisto da fenda de Rathke: relato de caso Translated title: Visual alterations due to Rathke's cleft cyst: case report

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          Abstract

          Os cistos da fenda de Rathke são lesões para-selares, geralmente assintomáticas, presentes em 12 a 33% das autópsias de pacientes com hipófise normal. Ocasionalmente, os cistos podem aumentar de volume a ponto de comprimir estruturas supra-selares e intra-selares, levando ao aparecimento dos sintomas. Clinicamente, os pacientes referem escurecimento visual, além de apresentarem defeitos campimétricos. M.A.S.R., 47 anos, sexo feminino, branca, natural de Pelotas/RS com queixa de embaçamento progressivo da visão do olho direito há 2 meses. Ao exame, constatou-se baixa visual e hemianopsia bitemporal. A tomografia computadorizada evidenciou imagem hipodensa arredondada, de bordos nítidos, em topografia selar, determinando remodelação e alargamento da sela túrcica. A ressonância magnética mostrou lesão expansiva cística localizada na sela túrcica com crescimento supra-selar. A referida lesão apresenta obliteração da cisterna supra-selar e importante compressão sobre o quiasma óptico. A abordagem cirúrgica confirmou a presença de lesão expansiva cística comprimindo o quiasma óptico e o exame de anatomia patológica do material diagnosticou cisto da fenda de Rathke. O exame oftalmológico, três meses após a cirurgia, mostrou melhora da acuidade visual e recuperação total dos defeitos do campo visual. O cisto da fenda de Rathke deve ser incluído no diagnóstico diferencial dos tumores para-selares passíveis de causar compressão da via óptica nervosa e ressalta-se a importância do diagnóstico e tratamento precoce no intuito de prevenir danos estruturais com perdas irreversíveis da função visual, bem como os distúrbios endócrinos.

          Translated abstract

          Rathke's cleft cysts are parasellar lesions, which are usually asymptomatic and found in 12 to 33% of autopsies done on patients with normal pituitary gland. Occasionally, the cysts can swell up to the point of putting pressure on the suprasellar and intrasellar structures, which provoke the symptoms. Clinically, the patients complain about blurred vision and they also present hemianopsia deficiency. M.A.S.R., 47 years old, female, white, born in Pelotas/RS, complained about progressive vision reduction in the right eye for 2 months. During the examination, loss of vision and bitemporal hemianopsia were discovered. Computerized tomography highlighted a rounded hypodense image with clear borders, in sellar topography, determining remodeling and enlarging of the sella turcica. Magnetic resonance showed expansive cystic injury located in the sella turcica with suprasellar growth. The mentioned injury presented obliteration in the suprasellar cistern and an important compression on the optic chiasm. The surgical approach confirmed the presence of extensive cystic injury compressing the optic chiasm, and the anatomicopathological examination of the material diagnosed Rathke's cleft cyst. The ophthalmic examination three months after the surgery showed improvement in visual accuracy and total recovery of defects of the visual field. Rathke's cleft cyst must be included in the differential diagnosis of parasellar tumors which may cause compression of optical pathway, and the importance of the diagnosis and immediate treatment with the purpose of avoiding structural damages with unreversible losses of visual function, as well as endocrine disorders are emphasized.

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          Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts.

          The clinical, radiographic, and pathological findings in 155 patients with symptomatic Rathke's cleft cysts are discussed. Eight patients were treated by the authors and 147 were collected in a review of the literature. This lesion occurred more often in female than male patients by a 2:1 margin, and the mean age at presentation was 38 years. The average patient had been symptomatic for nearly 3 years at the time of treatment, with the most common symptoms and signs being pituitary dysfunction, visual disturbances, and headaches. Affected children generally were pituitary dwarfs. The sella was enlarged in 80% of cases, and the cyst was situated in both an intrasellar and a suprasellar location in 71%. Computerized tomography revealed a low-density cystic mass with capsular enhancement in one-half of the cases. A variable appearance was seen with magnetic resonance imaging. Partial excision and drainage of the cyst by the transsphenoidal approach is the recommended treatment, as the recurrence rate is low. Most symptoms and signs improved or resolved following surgery with the exception of hypopituitarism and diabetes insipidus. The cyst lining was usually composed of ciliated cuboidal or columnar epithelium. Theories as to the origin of Rathke's cleft cysts are also discussed.
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            Radiologic characteristics and results of surgical management of Rathke's cysts in 43 patients.

            Although Rathke's cysts are a relatively common autopsy finding, rarely have they been reported as a clinical entity. Because of recent improvements in neuroradiological imaging, cystic intrasellar and suprasellar lesions are discovered often, leading to questions about proper management. Against this background, we reviewed the data from 43 patients with Rathke's cysts treated by one neurosurgeon over a 13-year period, and present the results here. The 43 patients had a mean age of 34 years, and 77% were female. Headache was the most common symptom, followed by galactorrhea, visual field loss, and hypopituitarism. Computed tomographic (CT) scans were reviewed in 20 cases, magnetic resonance (MR) images were reviewed in 15, and both CT and MR studies were reviewed in 5 cases. Although all Rathke's cysts were discrete and well-defined by both CT and MR imaging, the diversity of locations, CT attenuations, and MR signal intensity make it difficult to establish the diagnosis by radiological criteria. Forty patients underwent transsphenoidal surgery and three underwent craniotomy. There was one recurrence at 25 months requiring a second operation, and the mean follow-up period was 62 months. Seven patients had persistent headaches. For symptomatic lesions suspected to be Rathke's cysts, the recommended treatment is simple drainage of the cyst with biopsy of the wall, when this can be done safely. Follow-up imaging should be minimal for asymptomatic patients, and radiation therapy is not indicated.
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              Symptomatic Rathke's cleft cyst. Morphological study with light and electron microscopy and tissue culture.

              A rare case is reported in which a symptomatic Rathke's cleft cyst was studied by light and electron microscopy and tissue culture. The findings are compared with those of a craniopharyngioma studied in the same way. The patient was a 26-year-old woman presenting with headache, chiasmatic syndrome, and hypopituitarism. A cyst containing a mural nodule was partially removed and an Ommaya reservoir placed in the operative site for further treatment. The cyst wall was composed of connective tissue and three kinds of epithelial cells: non-ciliated squamous, ciliated columnar, and mucous-secreting cells. The morphology of these cells in vitro was similar to prickle cells seen in craniopharyngioma and the epidermis. It is concluded that both Rathke's left cyst and craniopharyngioma originate in remnants of Rathke's pouch, but at times may show some histological differences.
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                Author and article information

                Journal
                abo
                Arquivos Brasileiros de Oftalmologia
                Arq. Bras. Oftalmol.
                Conselho Brasileiro de Oftalmologia (São Paulo, SP, Brazil )
                0004-2749
                1678-2925
                December 2004
                : 67
                : 6
                : 965-968
                Affiliations
                [01] orgnameHospital Banco de Olhos de Porto Alegre
                [02] orgnameUniversidade Católica de Pelotas orgdiv1Faculdade de Medicina
                [03] orgnameUniversidade Católica de Pelotas orgdiv1Faculdade de Medicina orgdiv2Departamento de Oftalmologia
                [04] orgnameUniversidade Católica de Pelotas orgdiv1Faculdade de Medicina orgdiv2Departamento de Neurologia
                Article
                S0004-27492004000600024 S0004-2749(04)06700624
                ad7d0498-d0d8-4169-8ee6-acc1cbd1f07c

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 28 April 2004
                : 07 July 2004
                : 24 October 2003
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 17, Pages: 4
                Product

                SciELO Brazil

                Categories
                Relatos de Casos

                Doenças hipofisárias,Magnetic resonance imaging,Tissue cultures,Pituitary diseases,Sella turcica,Cysts,Visual field,Hemianopsia,Imagem por ressonância magnética,Cultivo de tecido,Sela túrcica,Cistos,Campo visual

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