McCartney A C, Kirkness C M
Institute of Ophthalmology, London.
Eye (Lond). 1988;2 ( Pt 1):63-70. doi: 10.1038/eye.1988.14.
Corneal discs from 10 cases of posterior polymorphous dystrophy (PPD) and 20 cases of congenital hereditary endothelial dystrophy (CHED) were compared and contrasted using light and electron microscopy. Secondary epithelial changes were similar in both diseases but spheroidal degeneration of stroma was seen more commonly in dominant CHED and not at all in PPD, when band, calcific, keratopathy was commoner. Changes at the level of Descemet's membrane showing failure to regulate growth were seen in recessive CHED whereas dominant CHED and PPD were both associated with development of a fibrillary posterior collagen layer (PCL). Grotesquely banded PCL was also seen in some cases of PPD. Endothelial changes included vacuolation, development of microvilli and desmosomes in both diseases but multilayering was more common in PPD. The viscous layer of the cornea was seen by TEM in one case of PPD.
运用光学显微镜和电子显微镜对10例后弹力层多形性营养不良（PPD）患者及20例先天性遗传性内皮营养不良（CHED）患者的角膜片进行了比较和对照。两种疾病中继发性上皮改变相似，但基质的球状变性在显性CHED中更常见，而在PPD中则完全未见，PPD中带状、钙化性角膜病变更为常见。在隐性CHED中可见后弹力膜水平出现生长调节失败的改变，而显性CHED和PPD均与纤维状后胶原层（PCL）的形成有关。在部分PPD病例中还可见到奇形怪状的带状PCL。两种疾病的内皮改变均包括空泡形成、微绒毛和桥粒的出现，但多层化在PPD中更为常见。在1例PPD病例中通过透射电镜观察到了角膜的黏性层。
