|Year : 1973 | Volume
| Issue : 3 | Page : 134-135
Gurbax Singh, S Choudhry
Department of Ophthalmology, Maulana Azad Medical College, New Delhi, India
Department of Ophthalmology, Maulana Azad Medical College, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Singh G, Choudhry S. Ocular neurofibromatosis. Indian J Ophthalmol 1973;21:134-5
Neurofibromatosis is one of the phakomatoses with hereditary background. It is a complex, multiple tumor syndrome characterised by diffuse proliferation of the Schwann cells of the peripheral, cranial and visceral nerves. It presents in the form of cutaneous, osseous, ocular and neurological manifestations.
ALLENDE  laid down the order of frequency of involvement in eye as eyelids, optic nerve, retina, iris, cornea, tarsal conjunctiva and bulbar conjunctiva. Occasionally, choroid, episclera, sclera and nerves supplying extra ocular muscles may be affected. In fact any part of the eye may be involved in neurofibromatosis. Hence in literature case reports have appeared from time to time describing some of the unusual ocular manifestations and presentations in cases of neurofibromatosis. We had the opportunity to study one such case which presented with unusual findings which have not been reported so far.
| Case Report|| |
A 40 years Hindu male presented with the history of acute pain in the right eye radiating to the right forehead of one day duration. This was accompanied with perception of collored haloes and marked diminution of vision. In the past patient complained of occasional transitory attacks of blurred vision and headache.
General examination revealed presence of subcutaneous nodules all over the body. Biopsy taken from one of these nodules established the diagnosis of neurofibromatosis [Figure - 1].
No abnormal skin pigmentation was seen.
Local examination of the right eye:- Vision was finger counting from one foot. The perception and projection of light was normal. Eye was congested with prominent blood vessels over the conjunctiva. Cornea was slightly hazy with prominent corneal nerves. Anterior chamber was shallow and irregular. There were anterior synechiae present just beyond the collarette. Iris was attached to the back of cornea by means of fine pillar like outgrowths. These pillar like structures were gray in color and symmetrically distributed all around the collarette. Iris showed presence of fine pigmented spots distributed all over its surface. The pupil was moderately dilated and oval, not reacting to light. Intraocular pressure was 59.1 mm Hg. by Schiotz tonometery. Fundus showed presence of hyperaemia of disc and dilatation of veins.
Local examination of left eye:Vision was 6/6 part. There was a pigmented patch in the lower part of the cornea near the limbus. Iris showed presence of fine pigmented spots. No nodules were seen.
Treatment and Follow Up:- The patient was treated on lines of acute congestive glaucoma with Diamox and intensive miotic therapy. Tension was controlled and vision improved to 6/18 in the right eye. A 2 mm corneoscleral trephine was performed in the right eye, following which the tension remained controlled. Fields showed presence of peripheral constriction and sickle shaped enlargement of the blind spot in the right eye.
X-ray skull, orbit and optic foramen revealed nothing abnormal.
| Discussion|| |
The interesting feature in this case is presence of pillar like outgrowths arising from iris just beyond the collarette attaching themselves on the back of cornea. This finding has not been described previously in literature. The pillar like outgrowths were quite distinct from the iris nodules described previously and were arranged in a symmetrical manner just beyond the collarette. The cause of glaucoma in this particular case could be due to extensive angle changes but the angle could not be examined due to extensive anterior synechiae and pillar like projections.
The patient responded very well to the conventional medical and surgical treatment of glaucoma.
| Summary|| |
An interesting case of ocular involvement in generalised neurofibromatosis has been discussed.
| References|| |
Allende, F. P. (1945): Arch. Ophthal.
[Figure - 1]