Marfan's Syndrome.
- Author:
Cho Young SONG
;
Chong Woo YOON
- Publication Type:Case Report
- MeSH:
Arm;
Cardiovascular System;
Child, Preschool;
Dislocations;
Esophagus;
Fathers;
Female;
Fingers;
Foot;
Hand;
Humans;
Joints;
Male;
Marfan Syndrome*;
Mothers;
Parents;
Pliability;
Pupil;
Retina;
Siblings;
Silk;
Skeleton;
Young Adult
- From:Journal of the Korean Ophthalmological Society
1959;2(1):95-101
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
We are Presenting of two cases of Marfans syndrome. First acse; 22 year old boy, height 262 cm, weight 40.9 kg, typical skeleton, hands and feet. The span of the outstreched arm, from right finger to the other, is 165 cm, exceeds by 3 cm than the height. Abnormal flexibility of Joints in the both thums. Vision; O.D. 0.1 with-sph.7.0 D., O.S. 0.0 with-sph.6.0 D. Lens; typically dislocated, upward and inwad 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 th left eye, one of them 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-raypicture, balium in in esophagus, and normal E.C.G. picture. The second sase; 5 year old girl, Height 120 cm, weight 20.6 kg, typical skeletor, hands and feet. Vision both 0.2, 0.2 and 0.3 with -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, balium 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.
