|Year : 1966 | Volume
| Issue : 6 | Page : 262-263
Marfan's syndrome with bilateral megalocornea and subluxated cataractous lenses
BN Consul, AS Mehrotra, GB Mathur
S. M. S. Medical College and Hospital, Jaipur, Rajasthan, India
|Date of Web Publication||17-Jan-2008|
B N Consul
S. M. S. Medical College and Hospital, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
|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
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|| |
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|| |
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.
| References|| |
Bellows. J. G. (1944): Cataract and Anamolies of the lens, Henry Kimpton. London. P. 266.
Consul, S. N.. Kulshrestha O. P. and Kasliwal R. M., (1960) Journal of Indian Med. Asso., 35: 218.
Derrick Veil (1931): Megalocornea sine glaucoma. (Quoted by Stephenson)
Duke-Elder, S. (1964): System of Ophthalmology, Vol. III, Pt. II, Henry Kimpton, London. P. 1104.
Mathur, K. N. and Saxena R. B. (1965): Orient Arch. Ophth. 3: 325.
Stephenson, W. V. (1945): Anterior Megalophthalmos and arachnodactyly. Am. J. Ophth. 28: 315.
[Figure - 1], [Figure - 2]