Doyle Aoife, Hamard Pascale, Puech Michel, Lachkar Yves
Institut du Glaucome, Fondation Hôpital St. Joseph, 185 rue Raymond Losserand, 75674 Paris, Cedex 14, France.
Graefes Arch Clin Exp Ophthalmol. 2005 Sep;243(9):955-7. doi: 10.1007/s00417-005-1141-x. Epub 2005 Apr 15.
No relationship between Marfan's syndrome and pigmentary glaucoma has previously been described in the ophthalmic literature. We describe the case of a patient with Marfan's syndrome who had bilateral pigment dispersion syndrome and asymmetrical glaucoma.
A 34-year-old man with Marfan's syndrome developed pigment dispersion bilaterally. In the right eye, elevated intra-ocular pressure was associated with marked glaucomatous excavation of the right disc and corresponding visual field loss. A localised zonular dehiscence was present at the 6 o'clock position in the right eye.
Ultrasound biomicroscopy showed marked iris concavity in the right eye, increased iridolenticular contact and superior subluxation of the right lens, resulting in increased dispersion of pigment unilaterally. This explains the asymmetric pattern of the glaucomatous damage.
Asymmetrical progression of pigmentary glaucoma is uncommon and should prompt a search for some other mechanical factor, which might increase the iridozonular contact on one side or reduce it on the other. The association between the two syndromes in this patient was most likely mechanical due to reduced fibrillin expression throughout the eye and particularly in the iris and the lens zonules, resulting in loss of iris tensile strength and marked iris concavity as well as zonular weakness and partial lens subluxation.
眼科文献中此前未描述过马凡综合征与色素性青光眼之间的关系。我们报告一例患有双侧色素播散综合征和不对称性青光眼的马凡综合征患者。
一名34岁的马凡综合征男性患者双侧出现色素播散。右眼眼压升高，伴有右眼视盘明显的青光眼性凹陷及相应的视野缺损。右眼6点钟位置存在局限性晶状体悬韧带断裂。
超声生物显微镜检查显示右眼虹膜明显凹陷，虹膜晶状体接触增加，右眼晶状体向上半脱位，导致色素单侧播散增加。这解释了青光眼损害的不对称模式。
色素性青光眼的不对称进展并不常见，应促使寻找其他一些机械因素，这些因素可能会增加一侧的虹膜晶状体接触或减少另一侧的接触。该患者两种综合征之间的关联很可能是机械性的，原因是整个眼睛尤其是虹膜和晶状体悬韧带中纤维连接蛋白表达减少，导致虹膜抗张强度丧失、虹膜明显凹陷以及悬韧带薄弱和晶状体部分半脱位。
