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2歳女児の右眼窩のみに原発したjuve—nile fibromatosisの1例を経験した。初診時,右眼の眼球陥凹と上転,下転,内転の眼球運動障害があった。前眼部,中間透光体,眼底に異常所見はなかった。眼窩CT撮影で眼球の後上外側に直径約1cmの高吸収域が認められ,右眼窩腫瘍が疑われた。開頭による腫瘍摘出術を施行し,以後5か月間再発をみていない。摘出された腫瘍は病理組織学的にjuvenile fibromatosisと診断された。本症が眼窩に原発することはきわめて稀であり,特に本例のように眼窩のみに原発した報告はわれわれが調べた限りではなかった。本例ではfibromatosisが眼球の後上外側に発生したため,上直筋,外直筋をまき込んで眼球運動障害を生じ,また周囲組織と強く癒着し,眼球を後方に牽引したために眼球が陥凹したと思われた。
A 2-year-old girl presented with enophthalmos and impaired eye movement in her right eye. Weakness of upward, downward and medial gaze was present. Orbital CT scan showed a high-den-sity mass measuring 1 cm in diameter located supe-rior-posterior-lateral to the right eye with adjacent bone destruction. The tumor was surgically removed. Pathological examination showed fibrob-lastic proliferation invading the muscle, optic nerve and the adipose tissue. Malignant cells were absent. We diagnosed the condition as juvenile fi-bromatosis of the orbit. The impaired eye move-ment seemed to be due to invasion of fibrous tissue into the muscles. Enophthalmos seemed to be caused by adhesion and traction of the eyeball by the tumor tissue. There are two reports in whole literature dealing with juvenile fibromatosis of the orbit with systemic involvements. Our case is unique in manifesting orbital involvement only.
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