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Marfan´s syndrome의 二例

대한안과학회지 1959년 2권 1호 p.95 ~ 101
宋朝永 (  ) - 慶北醫大 眼科

尹正佑 (  ) - 慶北醫大 眼科

Abstract


We are Presenting of two cases of Marfans syndrome.
First case ; 22year old boy, height 262cm., weight 40.9kg. , typical skeleton, hands ,and feet. The span of the outstretched arm, from right finger to the other, is 165cm. exceeds by 3cm. than the height. Abnormal flexibility of Joints in the both Chums. Vision;
O.D. 0.1with- sph.7.0 D.
O.S. 0.lwith- sph.6.0 D.
Lens ; typically dislocated, upward and inward and inward iridodonesis is marked.
The slit lamp examination of the zonular fiber is. very attractive, In the left eye, dislocation is more severe than the other, and the zonular fiber are more elongated.
The fibers are seen very clearly, just like fine whitish silk contrast to its black background. At the 5 o´clock Position pupil margin in the left eye one of then was revered and distorted;
No significant fundus signs were seen.
His parents and four brothers all have Normal visions and skeletons Normal cardiovascular system, even x-ray picture, balium in esophagus, and normal E C G. picture.
The second case ; 5yearr old girl. Height 120cm, weight 20.06kg, typical skeleton, hands and feet.
Vision both 0.2, 0.2and 0.3with -sph.9.0 D.
Left eye ; typical dislocated lens. upward and outward. iridodonesis is also found, no significant fundus picture.
Normal cardiovascular system, even x-ray picture, baliuni in esophagus, and normal E.C.G. picture.
Father ; normal vision and skeleton.
mother ; typical skeleton, hands and feet, but normal vision, said her grandmother also had delicate bony structure and very poor vision, died Known causes.
The literature is briefly reviewed and the Post- operative complication esp. detachment of retina, emphasized.

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