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ARTICLES
Year : 1985  |  Volume : 33  |  Issue : 3  |  Page : 201-202

Bilateral coloboma of lens in Marfan's syndrome


Department of New Teaching Hospital, Jodhpur, India

Correspondence Address:
A S Mehrotra
New Teaching Hospital, Jodhpur
India
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Source of Support: None, Conflict of Interest: None


PMID: 3841870

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How to cite this article:
Mehrotra A S, Solanki N, Sabharwal K K. Bilateral coloboma of lens in Marfan's syndrome. Indian J Ophthalmol 1985;33:201-2

How to cite this URL:
Mehrotra A S, Solanki N, Sabharwal K K. Bilateral coloboma of lens in Marfan's syndrome. Indian J Ophthalmol [serial online] 1985 [cited 2019 Oct 16];33:201-2. Available from: http://www.ijo.in/text.asp?1985/33/3/201/30823

The presence of ectopia lentis with colo­boma of lens is among the very rare occu­rence. Only few cases had been reported in literature. One such case has been recently reported by Angra[1]. Here we are adding one more case to the literature, of this rare con­genital anomaly.


  Case report Top


G. K. 27 yrs. male attended the eye O.P.D. for problem of Headach & eye strain. The external examination of eyes showed relatively deep AC and iridodonesis. Visual acuity recorded was R-6/9, L-6/12, no improvement with pin hole. Both pupils were dilated for retinoscopy & fundus examination. There was subluxation of lens in up and out quadrent with coloboma of lens. The coloboma was in lower circumference of the lens. On slit lamp examination all findings were confirmed. The zonules were present but scanty in this region.

The fundus examination revealed no ab­normality.

Systemic examination showed, height-180 cm. with long and thin extremeties (Dolicho­stenomelia). The finger bones were long. Positive wrist sign., high arched palate, defe­ctive dentation and pigeon shaped chest were other findings.

No Cardiovascular abnormality detected. ECG was normal. Other systems were normal


  Discussion Top


Coloboma of lens is usually unilateral but in some cases bilateral and double coloboma may be present. It usually presents with other coloboma of the eye but isolated colo­boma of lens also is seen. Coloboma of iris, retina, choroid and optic disc are due to defective closure and development of the optic vesicle. But coloboma of lens can't be explained on this basis as lens develop from surface ectoderm as lens vesicle.

The zonules may be maldeveloped in colo­boma of lens which may be due to either of reasons, Firstly typically incomplete closure of the optic vesicle, Secondly it may be due to persistant remnants of fibrovascular sheath of the lens interfering with development of zonules. (Waardewburg)[2].

In our case maldevelopment of zonules is not typically present so the coloboma of lens is possibly an isolated presentation.


  Summary Top


A rare case of coloboma lens, both eyes in Marfan's syndrome is reported.

 
  References Top

1.
Angra S. K., 1584, Ind. J. Ophthalmol. 32: 21.  Back to cited text no. 1
    
2.
Waardewburg, P. J., 1961, Genetics and Ophthalmology Royal Van Gorcum Ltd. Assen Nether­lands, 954.  Back to cited text no. 2
    


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