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      A Case of Rathke’s Cleft Cyst Inflammation Presenting with Diabetes Insipidus

      case-report

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          Abstract

          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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          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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              Transsphenoidal management of 28 symptomatic Rathke's cleft cysts, with special reference to visual and hormonal recovery.

              This is a retrospective study of a series of 28 Rathke's cleft cysts operated on transsphenoidally that was undertaken for the following reasons: 1) to study the presentation, with particular reference to hormonal and visual disturbances; 2) to study postoperative improvements in endocrine function and vision; 3) to record postoperative complications; and 4) to assess the efficacy of a minor modification of the standard transsphenoidal surgical technique, whereby the cyst is allowed to drain directly into the sphenoid air sinus without fossa floor repair when there is no intraoperative cerebrospinal fluid leak. A retrospective study was made of case notes, radiological findings, and operative notes in the series. There were 16 female and 12 male patients, with a mean age of 45 years. Endocrine disturbance was the most common presentation (50%), followed by headaches (32.1%) and visual impairment (14.3%). Neuroophthalmological assessment showed central visual acuity and field deficits in 32.1 and 44.6% of eyes, respectively. Biochemically, 85.7% of patients showed hormonal disturbances. Magnetic resonance imaging was used for 84.7% of cases, and 60.7% of cases showed suprasellar extension. Four magnetic resonance imaging patterns were noted. All cases were operated on transsphenoidally. Postoperative complications included cerebrospinal fluid rhinorrhea (7.1%), diabetes insipidus (3.6%), and meningitis (3.6%). Recovery of visual acuity and field was seen in 66.6 and 68% of eyes, respectively. Postoperative prolactin levels declined to normal or nearly normal values in 62.5% of cases, 20% of cases with low preoperative gonadotrophin levels achieved normal levels, and 15.4% of cases with preoperative pan-hypopituitarism achieved normal serum cortisol levels postoperatively. Within the study period there were no recurrences; although a relatively short time interval is presented for this series, a patient with a similar lesion operated on by the same method 7 years previously remains well, without radiological evidence of recurrence. Although comparatively rare and benign lesions, Rathke's cleft cysts are an important cause of hormonal and visual disturbances. Transsphenoidal surgery is safe and efficacious and leads to excellent improvement of function in the majority of cases. The surgical modification described seems safe and satisfactory and is extremely easy to perform.
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                Author and article information

                Journal
                Korean J Intern Med
                Korean J. Intern. Med
                KJIM
                The Korean Journal of Internal Medicine
                Korean Association of Internal Medicine
                1226-3303
                2005-6648
                June 2001
                : 16
                : 2
                : 132-135
                Affiliations
                [* ]Division of Nephrology, Department of Internal Medicine, College of Medicine, Hallym University, Chunchon, Korea
                Division of Nephrology, Department of Internal Medicine, Institute of Renal Disease, College of Medicine, Korea University, Seoul, Korea
                Author notes
                Address reprint requests to : Won Yong Cho, MD, Division of Nephrology, Department of Internal medicine, Institute of Renal disease, College of Medicine, Korea University Hospital, 126-1 Anam-dong, Sungbuk-gu, Seoul, Korea
                Article
                kjim-16-2-132-14
                10.3904/kjim.2001.16.2.132
                4531716
                11590900
                70fae637-8f5e-4991-b9d9-2d1e8e415df3
                Copyright © 2001 The Korean Association of Internal Medicine

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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                Categories
                Case Report

                Internal medicine
                rathke’s cleft cyst,diabetes insipidus
                Internal medicine
                rathke’s cleft cyst, diabetes insipidus

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