Pouliquen Y, Dhermy P, Espinasse M A, Savoldelli M
J Fr Ophtalmol. 1985;8(1):43-54.
Keratoglobus is a bilateral corneal disease characterised by thinning and protrusion of the entire corneal surface. The cornea is of normal size and usually transparent. Acute corneal edema due to rupture of Descemet's membrane and perforation even from minimal trauma are the most frequent complications. Keratoglobus may be associated with blue sclera, hyperextensibility of the joints or auditory problems. Most often it is a congenital disease with autosomal recessive transmission, but it may be secondarily acquired to an advanced keratopathy (keratoconus), to trauma or to exophthalmos. We report 5 cases of keratoglobus. 4 patients underwent penetrating keratoplasty. The anatomical abnormalities of congenital keratoglobus (3 cases) are constants : an absent Bowman's membrane, a disorganized stroma containing granular material, a thickened, striated Descemet's membrane with breaks, folds and verrucosities. On the other hand, acquired keratoglobus (1 case) shows localised breaks in Bowman's membrane resembling those of keratoconus. The anatomopathological differences are discussed.
球形角膜是一种双侧性角膜疾病,其特征为整个角膜表面变薄并向前突出。角膜大小正常,通常透明。由于后弹力层破裂导致的急性角膜水肿甚至因轻微外伤引起的穿孔是最常见的并发症。球形角膜可能与蓝色巩膜、关节过度伸展或听觉问题有关。它最常为常染色体隐性遗传的先天性疾病,但也可能继发于晚期角膜病变(圆锥角膜)、外伤或眼球突出。我们报告5例球形角膜病例。4例患者接受了穿透性角膜移植术。先天性球形角膜(3例)的解剖学异常是恒定的:Bowman膜缺失、基质含有颗粒物质且结构紊乱、后弹力层增厚、有条纹且有破裂、褶皱和疣状改变。另一方面,后天性球形角膜(1例)显示Bowman膜有类似圆锥角膜的局限性破裂。对解剖病理学差异进行了讨论。