Reis-Bucklers角膜营养不良的组织病理学研究

Histopathologic changes of Reis-Bücklers corneal dystrophy

目的:探讨Reis-Bucklers角膜营养不良的组织病理学改变。方法:取1家系中3例3眼Reis-Bucklers角膜营养不良患者角膜移植术后的病变角膜,3眼的1/2角膜组织行HE、PAS、刚果红、Masson、阿辛蓝染色后光学显微镜观察。以2例2眼正常角膜组织(眼库捐献角膜)作为对照。结果:Reis-Bucklers角膜营养不良患者前弹力层消失,被异常组织代替。HE染色证实病变主要累及角膜前弹力层,PAS、刚果红、Masson染色均为阳性,阿辛蓝染色为阴性。结论:该Reis-Bucklers角膜营养不良家系为前弹力层地图型病变,角膜异常沉着物性质为细胞外淀粉样纤维蛋白沉着。

AIM： To explore the histopathologic changes of Reis-Bücklers corneal dystrophy（RBCD）.METHODS： Cornea buttons were obtained from patients in 1 pedigree who underwent lamella keratoplasty. Sections with HE and special staining which included symplectic blue staining and Masson staining and Congo red staining were observed under light microscope. Two normal cornea specimens（donated corneas from eye bank）were used as control.RESULTS： In those patients, the bowman＇s membrane disappeared. The main lesion was in bowman＇s membrane through HE staining, positive through PAS Congo red staining and Masson staining, negative through symplectic blue staining.CONCLUSION： The Reis - Bücklers corneal dystrophy is characterized as geographic map-like lesion of bowman＇s membrane, and the abnormal extracellular deposit is amyloid protein fiber.