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| CASE REPORT |
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| Year : 1984 | Volume
: 32
| Issue : 1 | Page : 21-22 |
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Coloboma of lens
SK Angra, S Gupta, VK Dada, AK Gupta
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
Correspondence Address:
S K Angra
For Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 6500659 
How to cite this article:
Angra S K, Gupta S, Dada V K, Gupta A K. Coloboma of lens. Indian J Ophthalmol 1984;32:21-2 |
Coloboma of lens in Marfan's syndrome was described by Mckusick[1] and was observed in one patient by Wachtel[2] while studying the ocular pathology of Marfan's syndrome. This is the one of the very rare fully documented report of bilateral lens coloboma in Marfan's syndrome.
| Case report | |  |
I.P., 19 years old male was examined for diminished vision in right eye. The family history was non-contributory. The patient was -thin built, tall with arachnodactyly and had hand span more than the height. He had high arched palate. He had no cardiac, pulmonary, renal, central nervous system or glossal anomalies.
Ocular examination revealed a vision of counting fingers at 1 metre in Right Eye and 6/60 (P) .in Left Eye improving to 6/60 with15,00 D Sph. (R) and 6/6 with -1.0 D. Sph. (L). 15° Rt. divergent squint was present. There were bilateral colobomata of the ectopic lenses. In left eye it was in the lower quadrant and in right eye, there were two, one in the lower quadrant. The zonules were seen attached to the coloboma areas and uveal pigment was seen deposited on the zonules. The fundus showed myopic changes in both eyes. [Figure - 1][Figure - 2].
| Discussion | | |
The unusual features of this case of Marfan's syndrome is association of bilateral coloboma of the lens. This association has not so far been described clinically with full documentation.
Coloboma of lens is a condition with autosomal dominant heredity, occurs at about 4th month of development. It is usually associated with other colobomatous conditions of the globe. It was sporadic in occurrences and there was no other coloboma present It typically occurs in the downward position but may occur anywhere along the circumference of the lens. It is usually unilateral and single but may be bilateral and double. Shape and size of coloboma vary. Frequently the lens is cataractous, the opacity being nuclear or localised to colobomatous area but never involving the foetal or embryonic nuclei. In the region of coloboma the zonules are either absent or maldeveloped. The maldevelopment of zonules may be because of two causes.
In cases of typical colobomas it may be due to incomplete closure and development of the optic vesicles and these are associated with other colobomas in the eye. Since coloboma of the iris, retina, choroid, macula and optic nerve have all been described in Marfan's syndrome. The coloboma of the lens may not have a similar etiological basis because the lens vesicle is formed independent of closure of foetal fissure. In our case it was isolated anomaly and no other coloboma was preset in any of the other tissues.
The persistent remnants of fibro vascular sheath of the lens may act by mechanically interfering with the development of zonules or by actual pressure on the lens itself. But in our case no such apparent effect was seen on the iris or pupil itself nor remnants of persistent pupillary membrane were there.
The poor development of zonules appears to be the primary defect in our case and hence its association in Marfan's syndrome can be explained.
| Summary | | |
A rare association of bilateral coloboma of the lens with Marfan's syndrome is reported.[4]
| References | | |
| 1. | McKusick, N.A. 1955, Circulation 11.:321.  |
| 2. | Wachtel, J.G., 1966, Arch. Ophthalmol. 76:512. |
| 3. | Angra S.K. and Mohan, M., 1974, East. Arch. Ophthalmol. 2:231. |
| 4. | Duke Elder, S., 1964: System of Ophthalmology Vol. III, part II, p. 1102, 706, Henry Kimpton-1964. |
[Figure - 1], [Figure - 2]
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