Lapp T, Birnbaum F, Evert M, Reinhard T, Auw-Hädrich C
Division of Infection and Immunity, University College London, Cruciform Building, Gower Street, London WC1E 6BT, UK.
Ophthalmologe. 2012 Feb;109(2):155-60. doi: 10.1007/s00347-011-2443-5.
A 57-year-old male patient with Marfan syndrome presented at our clinic with a whitish gelatinous corneal tumour in the right eye. The initial examination revealed pronounced corneal oedema, bullous keratopathy, as well as an iris-fixed anterior chamber lens implanted 7 years previously. After the tumour was removed, the anterior chamber lens was explanted and keratoplasty was explanted and a two stage keratoplasty was performed. Histological analysis of the tumour and the cornea revealed vimentin and a number of smooth muscle actin (SMA)-positive tumour cells. The cornea below the tumour displayed a partially absent Bowman's layer and extensive pannus tissue. The characteristics of the corneal tumour and the subjacent cornea as described above are typical of secondary corneal myxoma. The influence of Marfan syndrome, a systemic connective tissue disorder present in the patient, on the etiopathogenesis of the corneal myxoma could not be fully determined.
一名57岁患有马凡综合征的男性患者因右眼出现白色胶冻状角膜肿瘤前来我院就诊。初步检查发现角膜明显水肿、大泡性角膜病变,以及7年前植入的虹膜固定前房型人工晶状体。肿瘤切除后,取出前房型人工晶状体,进行角膜移植并分两期进行角膜移植术。对肿瘤和角膜的组织学分析显示波形蛋白和一些平滑肌肌动蛋白(SMA)阳性肿瘤细胞。肿瘤下方的角膜显示Bowman层部分缺失和广泛的血管翳组织。上述角膜肿瘤及下方角膜的特征是继发性角膜黏液瘤的典型表现。患者所患的系统性结缔组织疾病马凡综合征对角膜黏液瘤发病机制的影响尚不能完全确定。