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ARTICLE
Year : 1966  |  Volume : 14  |  Issue : 6  |  Page : 262-263

Marfan's syndrome with bilateral megalocornea and subluxated cataractous lenses


S. M. S. Medical College and Hospital, Jaipur, Rajasthan, India

Date of Web Publication17-Jan-2008

Correspondence Address:
B N Consul
S. M. S. Medical College and Hospital, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Consul B N, Mehrotra A S, Mathur G B. Marfan's syndrome with bilateral megalocornea and subluxated cataractous lenses. Indian J Ophthalmol 1966;14:262-3

How to cite this URL:
Consul B N, Mehrotra A S, Mathur G B. Marfan's syndrome with bilateral megalocornea and subluxated cataractous lenses. Indian J Ophthalmol [serial online] 1966 [cited 2020 Dec 2];14:262-3. Available from: https://www.ijo.in/text.asp?1966/14/6/262/38670

It has been estimated that 50 per­cent of all cases of arachnodactyly arc associated with ectopia lentis (Rados, 1941; Consul, 1960; Mathur, 1965). The ectopic lens is frequently reduced in size. In majority of cases the lens remains clear. Damianos* found 10 opaque lenses out of 46 cases, while "Ringelhan and Elschnig report 28 opaque lenses out of 44 cases.

In all cases of anterior megaloph­thalmos the corneal diameters are en­larged and may even reach 18 mm. (Stephenson, 1945) but in addition to the enlarged corneal diameters, there are other characteristics (Derick Vail, 1931). It is hereditary, always bila­teral, and limited to the male sex ex­cept in cases of consanguinity, ab­sence of all signs of glaucoma, deep­ning of the anterior chamber, irido­donesis, atrophy of stroma of the iris, dislocated or tremulous lens etc.

A case of Marfan's syndrome with Megalocornea and cataractous octopic lenses is reported, a very rare combi­nation of the two eye conditions.


  Case Report Top


M.R. aged 14 years male was ad­mitted to the eye ward of this hospital on 16-3-65 with a perforating injury of the right eye with a piece of wood while playing. There was total loss of vision after the trauma. There was also marked diminution of vision in the left eye and partial deafness.

His vision was very defective since early childhood, as stated by his father. Some operation was done in his right eye about 5 years back (pro­bably needling), after which he got some vision in this eye, but the left eye was having no useful vision. After an injury 6 years back, he became partially deaf.

Family history, No abnormality was noticed in all his brothers sisters and parents.

General examination: Patient was tall (5 ft 4 inches) thin built with long hands and fingers [Figure - 1]. His weight was 82 lbs. His cardio-vascular, pul­monary and C. N. S. examinations showed no abnormality.

Local condition: Right eye cornea was large in diameter and very thin and was pushed in the anterior cham­ber. There was perforation in the upper half. The anterior chamber was absent and there was lot of blood be­hind the cornea. Lens was not found (Probably needling was done earlier). Eye was very soft.

Left eye-Megalocornea: (Vertical diameter 14.2 mm horizontal 14.6 mm) [Figure - 2]. The chamber was deep; the pupil regular, round, sluggish in reaction. The lens was opaque and subluxated. The vision was reduced to finger counting at 2 m. with good projection of light. The fundus could not be seen due to lenticular opacity.

Investigations:- Total leucocytes: 7,100/cmm. Differential count: P. 70%, L. 30%.

Kahn test was negative.

Skiagrams---Nothing abnormal was detected, except the long fingers and toes.

Nothing else abnormal was detect­ed in his cardio-vascular system.

Treatment: In the right eye, corneal suturing was tried on the same day, just to improve the shape of the eye for cosmetic reasons. Cornea was pa­pery thin, so that there was great difficulty in suturing. Five weeks later this eye was enucleated as tension was not getting built up, there was no vision, the iris was pulled up in the section and the pain and photophobia in the eye was not relieved. Left eye: Curette evacuation of the lens was done in this eye.


  Summary Top


A rare case of Marfan's syndrome with cataractous subluxated lens and bilateral megalocornea is reported.

Re-check up of left eye (in March 66)

Vision with glasses 6/18 fundus could not be seen due to corneal degenerative changes and corneal curvature irregularities.[6]

 
  References Top

1.
Bellows. J. G. (1944): Cataract and Anamolies of the lens, Henry Kimpton. London. P. 266.  Back to cited text no. 1
    
2.
Consul, S. N.. Kulshrestha O. P. and Kasliwal R. M., (1960) Journal of Indian Med. Asso., 35: 218.  Back to cited text no. 2
    
3.
Derrick Veil (1931): Megalocornea sine glaucoma. (Quoted by Stephenson)  Back to cited text no. 3
    
4.
Duke-Elder, S. (1964): System of Ophthalmology, Vol. III, Pt. II, Henry Kimpton, London. P. 1104.  Back to cited text no. 4
    
5.
Mathur, K. N. and Saxena R. B. (1965): Orient Arch. Ophth. 3: 325.  Back to cited text no. 5
    
6.
Stephenson, W. V. (1945): Anterior Megalophthalmos and arachnodactyly. Am. J. Ophth. 28: 315.  Back to cited text no. 6
    


    Figures

  [Figure - 1], [Figure - 2]



 

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