Amaya Luis, Taylor David, Russell-Eggitt Isabelle, Nischal Ken K, Lengyel Dora
Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom.
Surv Ophthalmol. 2003 Mar-Apr;48(2):125-44. doi: 10.1016/s0039-6257(02)00462-9.
The morphology of congenital cataract reflects a combination of the timing and nature of the cause, the anatomy of the lens including its capsule, its development, and changes that take place with time. Morphology may variably affect prognosis, give a clue to the etiology and the age of onset and, in an isolated case, sometimes suggest heritability. The spectrum of morphological variations is enormous and can be complex. A comprehensive approach is to classify the variations according to the area of the lens involved, and sub-dividing them by a detailed description of the shape and appearance. Each specific morphological type is then analyzed determining the etiology, visual prognosis, and management. The use of gene markers has allowed many of these variations to be identified and categorized. Cataracts in childhood can involve the whole lens, in which case they are called total, Morgagnian, or disk-like. They can affect only the center of the lens: lamellar, nuclear, oil droplet, cortical, or coronary. They can be anterior: anterior polar, anterior subcapsular, or anterior lenticonus. The posterior aspect of the lens can also be affected in different fashions: Mittendorf's dot, posterior lenticonus, posterior cortical cataracts, or posterior subcapsular. There are five more forms that must be described separately: punctuate lens opacities, sutural cataracts, coralliform or crystalline, wedge-shaped, and persistent hyperplastic primary vitreous.
先天性白内障的形态反映了病因的时间和性质、晶状体的解剖结构（包括其囊膜）、其发育过程以及随时间发生的变化等多种因素的综合结果。形态可能会对预后产生不同程度的影响，为病因、发病年龄提供线索，并且在个别情况下，有时还能提示遗传性。形态学变异的范围极为广泛且可能很复杂。一种全面的方法是根据晶状体受累的区域对变异进行分类，并通过对形状和外观的详细描述进一步细分。然后对每种特定的形态学类型进行分析，以确定病因、视觉预后和治疗方法。基因标记的应用使得许多此类变异得以识别和分类。儿童期白内障可累及整个晶状体，这种情况下称为全白内障、莫尔加尼白内障或盘状白内障。它们也可能仅影响晶状体的中心：板层白内障、核性白内障、油滴状白内障、皮质性白内障或冠状白内障。白内障还可以发生在晶状体的前部：前极性白内障、前囊下白内障或前圆锥形晶状体。晶状体的后部也可能以不同方式受到影响：米滕多夫点、后圆锥形晶状体、后皮质性白内障或后囊下白内障。还有另外五种形式必须单独描述：点状晶状体混浊、缝性白内障、珊瑚状或结晶状白内障、楔形白内障以及永存原始玻璃体增生症。
