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[马方综合征的统计分析]

[Statistical analysis of Marfan's syndrome].

作者信息

Wakita M, Ryu E, Nakayasu K, Kashima K, Fujiki K, Kato K, Nakajima A, Kabasawa K

出版信息

Nippon Ganka Gakkai Zasshi. 1989 Jun;93(6):682-90.

PMID:2816576
Abstract

To evaluate the ocular complications of Marfan's syndrome which cause severe visual disturbance, we retrospectively investigated 104 patients (208 eyes) with Marfan's syndrome. Dislocation of the lens, the most frequent ocular manifestation, was detected in 151 eyes (72.6%). There were 33 eyes with a visual acuity 0.1 or less. In this group, we found retinal detachment in 12 eyes (36.4%) and glaucoma in 5 eyes (15.2%) as the causes of visual disturbance. Among 17 eyes with retinal detachment, 5 eyes (29.4%) had horizontally dislocated lens. On the other hand. The lens dislocation in a downward direction was found in 5 (22.7%) of 22 eyes with glaucoma. No lens dislocation in an upward direction was found in eyes with glaucoma. The incidence of retinal detachment in aphakic eyes (5/31, 16.1%) was higher than that in the phakic group (16/177, 9.0%). In only 2 (3.6%) of 56 eyes, did the lens dislocation become markedly worse during the observation period. From these data, we re-confirmed that retinal detachment and glaucoma are the most important ocular complications of Marfan's syndrome leading to severe visual disturbance. The decision to operate on the dislocated lens should be made cautiously.

摘要

为评估导致严重视力障碍的马方综合征眼部并发症,我们回顾性研究了104例(208只眼)马方综合征患者。晶状体脱位是最常见的眼部表现,在151只眼中被检测到(72.6%)。有33只眼视力在0.1及以下。在这组患者中,我们发现视网膜脱离12只眼(36.4%)和青光眼5只眼(15.2%)是视力障碍的原因。在17只视网膜脱离的眼中,5只眼(29.4%)有晶状体水平脱位。另一方面,在22只青光眼眼中,5只眼(22.7%)有晶状体向下脱位。青光眼患者的眼中未发现晶状体向上脱位。无晶状体眼的视网膜脱离发生率(5/31,16.1%)高于有晶状体眼组(16/177,9.0%)。在56只眼中,只有2只眼(3.6%)在观察期间晶状体脱位明显加重。从这些数据中,我们再次证实视网膜脱离和青光眼是马方综合征导致严重视力障碍的最重要眼部并发症。对于晶状体脱位手术的决定应谨慎做出。

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