|Year : 1980 | Volume
| Issue : 4 | Page : 229-230
Crouzon's disease with bilateral luxation of globes
G David Chandran, TA Alexander, Anna Thomas
Department of Ophthalmology, Christian Medical College, Vellore, India
G David Chandran
Schell Eye Hospital Vellore 632001
|How to cite this article:|
Chandran G D, Alexander T A, Thomas A. Crouzon's disease with bilateral luxation of globes. Indian J Ophthalmol 1980;28:229-30
|How to cite this URL:|
Chandran G D, Alexander T A, Thomas A. Crouzon's disease with bilateral luxation of globes. Indian J Ophthalmol [serial online] 1980 [cited 2013 May 26];28:229-30. Available from: http://www.ijo.in/text.asp?1980/28/4/229/28265
This syndrome initially described by Crouzon in 1912, has following clinical features namely frontal bossing, prognathism, exophthalmos, hypertelorism, exotropia, optic atrophy, maxillary hypoplasia, psittacorhina (parrot beak) and high arched palate The premature synostosis has been blamed to be secondary to an inflammatory process occurring around the sutures or as a true developmental anomaly of germinal origin, where there is a defect in the mesoderm which goes to form the bones of base of skull.
In this article we are reporting a case of Crouzon's disease with bilateral luxation of the globes.
| Case report|| |
29 year old female was brought with complaints of forward protrusion of the eye balls since birth. On examination visual acuity in right eye was counting fingers 50 cms and left eye has hand movements. The left eye had divergent squint of 35°. Fundus examination revealed bilateral primary optic atrophy. As she was developing exposure keratitis, lateral tarsorrhaphy was done in both eyes and was advised orbital decompression. The patient was unwilling for surgery and hence was discharged. 9 months later the patient was brought to us with bilateral luxation of the globes and severe exposure keratitis. Perception of light was absent in both eyes. We admitted the case and did enucleation in both eyes as they were blind and painful. X-ray studies of Skull showed oxycephaly.
| Discussion|| |
Ocular lesions in Crouzon's disease are common but luxation of the globe is unusual. Bilateral proptosis is believed to be due to shallowness of the orbit and oedema of the tissues around. The orbits are improperly developed with a divergent axis. The orbital changes lead to proptosis and exposure keratitis, which sometimes is very severe. Luxation of the globe occurs following prolonged crying, after trauma, or spontaneously. In this case it is interesting that the luxation developed following a bout of severe fits. As far we could trace, this seems to be the first time bilateral luxation of the globe is being reported in Crouzon's disease.
The inevitable severe complication of this condition can be prevented by neurosurgical interference as early as the disease is recognised, around the age of 6 months. The chief indications for surgery are progressive exophthalmos, progressive optic atrophy, progressive mental retardation and raised intracranial pressure. From the ocular point of view, orbital decompression, strengthening of Orbicularis oculi and correction of squint are advised, which could not be done in our case as the eyes were blind.
| Summary|| |
A case of Crouzon's disease with bilateral luxation of the globes is being reported.
| References|| |
|1.||Duke-Elder, S., 1967, Systerm of Ophthalmology Vol. III, Part If, p. 1943, Henry Kimpton, London. |
|2.||Mount, L. A., 1947, New York State J. Med. 47 ; 20 |
[Figure - 1], [Figure - 2], [Figure - 3]