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Sclerocorneaを呈した3歳児の一眼に全層角膜移植術を施行し,摘出した角膜と角膜に癒着した虹彩様組織を組織学的に検討した。従来報告のあるボーマン膜の欠如,実質の膠原線維束配列の乱れと血管侵入,デスメ膜と内皮細胞の欠如という角膜所見に加え,角膜に癒着した虹彩に相当する組織は実質形成不全,および色素上皮細胞層と無色素上皮細胞層の2層からなる上皮,水晶体側に色素上皮細胞を含む粗性結合組織膜の存在を認めた。本症例は組織学的には,anterior chamber cleavage syndromeに含まれる症例の1例であり,間葉組織のみならず神経堤細胞・外胚葉系の発生異常も生じていると考えられた。
A 3-year-old male child with sclerocornea under-went penetrating keratoplasty. The removed cor-nea and the adherent iris were subjected to his-topathological observations. We observed absence of Bowman's and Descemet's membranes. The en-dothelial cells were also absent. The cornealstroma showed irregularity in distribution and diameter of collagen fibrils in addition to neovas-cularization. The adherent iris showed an epithelial layer with pigmented and nonpigmented cells, hypo-plasia of iris stroma and membraneous connective tissue with pigmented epithelial cells. The findings seemed to suggest that this eye manifested an anterior chamber cleavage syndrome involving, additionally, the mesenchymal and neural crest cells.
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