Kampik A, Lund O E, Hälbig W
Klin Monbl Augenheilkd. 1986 Mar;188(3):188-92. doi: 10.1055/s-2008-1050611.
The clinical course and histopathological findings in 17 cases of congenital corneal opacity treated by penetrating keratoplasty are described. On the basis of the histopathological findings 9 of the cases of corneal opacity were diagnosed as Peter's anomaly, 5 cases as sclerocornea or mesenchymal dysgenesis, 2 as choristoma, and 1 case as leukoma. Only 2 of the corneal grafts performed in these infants remained clear. The underlying disease in the 2 cases where surgery was successful was Peter's anomaly, with a clear corneal periphery. From the analysis of the corneal buttons the authors deduce that graft failure in most such cases is due to severe structural changes of the host cornea. Three findings represented consistently unfavorable signs: (1) absence of Bowman's layer; (2) absence of Descemet's membrane and (3) corneal vascularization at the cut edge of the button. Immunoreaction or infection of the graft are rare causes of graft failure. Despite the extremely poor prognosis of bilateral congenital corneal opacities, penetrating keratoplasty should be attempted. The histopathological findings may be used as prognostic criteria for graft survival or the extent of anterior segment dysgenesis.
本文描述了17例先天性角膜混浊患者接受穿透性角膜移植术的临床过程及组织病理学发现。根据组织病理学发现,9例角膜混浊患者被诊断为彼得异常,5例为巩膜角膜或间充质发育异常,2例为迷芽瘤,1例为角膜白斑。这些婴儿中仅2例角膜移植术后植片保持透明。手术成功的2例患者潜在疾病为彼得异常,角膜周边清晰。通过对角膜植片的分析,作者推断大多数此类病例移植失败是由于宿主角膜严重的结构改变。有三个发现始终代表不利迹象:(1)Bowman层缺失;(2)Descemet膜缺失;(3)植片切缘处角膜血管化。移植免疫反应或感染是移植失败的罕见原因。尽管双侧先天性角膜混浊预后极差,但仍应尝试进行穿透性角膜移植术。组织病理学发现可作为移植存活或前段发育异常程度的预后标准。