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Neurological Surgery No Shinkei Geka Volume 20, Issue 12 （December 1992）

Neurological Surgery 脳神経外科 20巻12号（1992年12月発行）

Japanese English

研究

部分的脳梁欠損を伴った大脳半球間裂内Choroidal Epithelial Cystの1例—症例および文献的考察 Interhemispheric Choroidal Epithelial Cyst Associated with Partial Agenesis of the corpus callosum: Case report and review of the literature 稲垣裕敬 ¹ , 黒崎雅道 ¹ , 堀智勝 ¹ , 小枝達也 ² , 大浜栄作 ³ Hirotaka INAGAKI ¹ , Masamichi KUROSAKI ¹ , Tomokatsu HORI ¹ , Tatsuya KOEDA ² , Eisaku OHAMA ³ ¹鳥取大学脳研神経外科 ²鳥取大学脳研神経小児科 ³鳥取大学脳研脳神経病理 ¹Divisions of Neurosurgery, Institute of Neurological Sciences, Faculty of Medicine, Divisions of Tottori University ²Divisions of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University ³Divisions of Neuropathology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University キーワード： Interhemispheric choroidal epithelial cyst , Agenesis of the corpus callosum , MRI , Light microscopy , Electron microscopy Keyword: Interhemispheric choroidal epithelial cyst , Agenesis of the corpus callosum , MRI , Light microscopy , Electron microscopy pp.1301-1306

発行日 1992年12月10日 Published Date 1992/12/10

DOI https://doi.org/10.11477/mf.1436900576

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Abstract 文献概要
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I．はじめに

　頭蓋内には種々の嚢胞性病変が発生するが単層の上皮細胞が嚢胞壁を形成する場合，従来，その大部分は光顕上ependymal cystあるいは（neuro）epithelial cystと包括的に診断されることが多く，また発生部位によりcolloid cyst， Rathkels cleft cystなどの名称も用いられ，若干の混乱を招いていたと思われる．これら単層の上皮性嚢胞の組織起源は様々であり，電顕による微細構造の検討によりこれらの細胞起源に関する報告がいくつか見られる^{3,5,7,10,11,13,15,18,20）}．

　今回われわれは大脳半球問裂に発生し，脳梁の部分欠損を伴った多房性の嚢胞であり，詳細な電顕的検索によりchoroidal epithelial cystと診断しえた1乳児例を経験した．症例を呈示するとともにこれら上皮細胞の起源に関して若干の文献的考察を加える．

　A case of interhemispheric choroidal epithelial cyst is reported.

　The patient is a 9-month-old female who was transadmitted to our hospital for further examination because of the enlargement of her head. She had no neurological deficits nor symptoms of increased intracranial pressure. CT scanning performed on admission showed multiple cystic lesions in the right frontoparietal interhemispheric space, whose circumference was partially enhanced with contrast medium. Metrizamide CT cisternography demonstrated no communication between the lesions and the ventricular system. The signal intensity of the cysts was higher than that of cerebrospinal fluid on both T1-weighted and T2-weighted MR images. Sagittal T1-weighted images showed partial agenesis of the corpus callosum.

　The surgical exploration was performed via interhemispheric approach. The cyst wall was found to be white, relatively rich in vascular components, and was removed as much as possible. The examination of the cyst fluid showed total protein levels of 1250 to 3440mg/dl, and sugar contents of 43 to 99mg/dl. Callosal agenesis was confirmed at operation.

　The light microscopic examination revealed that the cyst wall was composed of a single layer of columnar or cuboidal epithelium with occasional papillary configuration and thick collagenous connective tissue. The epithelial cells contained PAS-positive granules in the cytoplasm. Electron microscopy showed numerous clubshaped microvilli with no coating materials, continuous basement membrane, tight junction, interdigitation, andmultiple fenestrations of endothelium of stromal vessels.

　On the basis of these findings, the lesion was diagnosed as choroidal epithelial cyst. In the literature, interhemispheric choroidal epithelial cyst associated with partial callosal agenesis, confirmed ultrastructually, has not, to our knowledge, been reported.