Pletz C, Hentsch R
Augenklinik im Carl-Thiem-Klinikum Cottbus, Akademisches Lehrkrankenhaus der Charité Berlin, Thiemstrasse 111, 03048 Cottbus.
Klin Monbl Augenheilkd. 2000 Nov;217(5):284-8. doi: 10.1055/s-2000-10369.
The debate about whether megalophthalmus anterior is a diagnosis that needs to be distinguished clearly from buphthalmus or whether there are indeed connections between the two disorders is just as topical today as it was when the disorder was first described. With the aid of a genealogical examination, the symptoms of megalophthalmus anterior and the way in which they differ to those of buphthalmus will be shown.
Twelve individuals making up an age span over four generations formed the genealogical tree. In seven of the family members, the subjective refraction, the javal, the applanation values and the diameter of the cornea, both vertically and horizontally, were established, along with the length of the axis of the eye and the depth of the anterior chamber partly measured biometrically. In addition, a detailed slitlamp examination of the anterior section was carried out, together with a gonioscopy and a funduscopy. On four of these who bore the symptoms, a 30-2 cental visual field test was carried out using the Humphrey apparatus and evaluated.
The megalophthalmus anterior which used to be known by the name megalocornea globosa Kaiser-Grönholm, involves not only a megalocornea with a vertical diameter of the cornea of between 12.5-14.5 mm, but also an enlargement of the whole anterior section of the eye with iridodonesis and lentodonesis. In addition, through the existing series of examinations, the following characteristics of megalophthalmus were found: symmetrical differences between each side, a global front of the coenea with partly thining in the central region, crocodile chagrin, no sign of glaucoma (normal intaocular pressure, a practically normal angle of the iris, no excavatio papillae, no loss in the field of vision) and recessive x-chromosomal heredity.
In order to eliminate buphthalmus and to diagnose megalophthalmus anterior, the difficulty in correctly analysing the results seems to lie in the lack of glaucoma, in the findings in the anterior section and in the conditions hereditary tendency.
关于先天性大角膜是否是一种需要与先天性青光眼明确区分的诊断，或者这两种疾病之间是否确实存在关联的争论，如今和该疾病首次被描述时一样热门。借助系谱检查，将展示先天性大角膜的症状以及它们与先天性青光眼症状的不同之处。
由跨越四代的12个人组成了系谱树。在7名家庭成员中，确定了主观验光、查瓦耳（Javal）值、压平值以及角膜的垂直和水平直径，同时部分通过生物测量确定了眼轴长度和前房深度。此外，对眼前节进行了详细的裂隙灯检查，同时进行了前房角镜检查和眼底镜检查。对其中4名有症状的患者，使用汉弗莱（Humphrey）仪器进行了30-2中心视野测试并进行了评估。
过去被称为球形大角膜凯泽 - 格伦霍尔姆（Megalocornea globosa Kaiser-Grönholm）的先天性大角膜，不仅涉及角膜垂直直径在12.5 - 14.5毫米之间的大角膜，还涉及整个眼前节的扩大，伴有虹膜震颤和晶状体震颤。此外，通过现有的一系列检查，发现先天性大角膜具有以下特征：两侧对称差异、角膜整体前部在中央区域部分变薄、鳄鱼皮样改变、无青光眼迹象（眼压正常、虹膜角实际正常、无视乳头凹陷、视野无缺损）以及隐性X染色体遗传。
为了排除先天性青光眼并诊断先天性大角膜，正确分析结果的困难似乎在于缺乏青光眼表现、眼前节的检查结果以及遗传倾向情况。
