神经纤维瘤病

Von Recklinghausen's Neurofibromatosis

分析了神经纤维瘤病眼部病变患者33例。其中眼眶骨病变者27例;眼睑丛状神经纤维瘤者31例,虹膜结节者20例;眼球突出者23例;合并眶内脑膜脑膨出者9例;合并青光眼者4例;33例患者均有皮肤色素斑和多发性结节。11例患者有家族遗传史。对神经纤维瘤病的发病机理做了较详细的讨论。

Thirty-three cases of Von Recklinghausen's neurofibromatosis with ophthalmic manifestation were analysed. There were 27 cases with pathologic changes in orbit bones; 31 cases with flexiform neurofibroma in eyelid; 20 cases with iridic nodes; 23 cases with exophthalmus; 9 cases complicated with meningoencephalocele in the orbit and 4 cases complicated with glaucoma. There were the manifestetion of multinode and pigment patch on skin among all the 33 cases. Eleven cases had hereditary history with the disease in their families. The pathogenesis is discussed in detail.