|Year : 1957 | Volume
| Issue : 4 | Page : 112-114
A case of bilateral epibulbar dermoid tumours with unilateral coloboma of lid, hare lip etc
Department of Ophthalmology R. G. Kar Medical College, Calcutta, India
|Date of Web Publication||9-May-2008|
Department of Ophthalmology R. G. Kar Medical College, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bose J. A case of bilateral epibulbar dermoid tumours with unilateral coloboma of lid, hare lip etc. Indian J Ophthalmol 1957;5:112-4
True corneal dermoids are rare. They airse from an aberration of mesoblast lying between the surface ectodorm and the optic cup. The resultant deformity varies with the time at which the anomaly appears. (Duke-Elder, 1943).
1. If at 4.5 mm. stage of the embryo, the corneal tissue is replaced by a fibrofatty growth, the eye becoming grossly microphthalmic without the formation of lens or iris
2. If after 4.5 mm. stage, an ill-formed lens and iris are formed.
3. Localised patches of dermoid are commonly seen at the infero-lateral quadrant of the limbus. Recently a case was admitted under me, apparently simulating bilateral corneal staphyloma but really a case of bilateral epibulbar dermoid associated with coloboma of the right upper lid and harelip.
| Case Report|| |
Master N.K.D. Hindu, aged 17, gave history of something growing on the white of both eyes since childhood and gradual diminution of vision since early childhood. The father of the patient said that at the beginning there were two small growths about the size of a pea, one in each eye and attached to the infero-lateral aspects of both eye balls. The growths had gradually increased in size and ultimately encroached the pupillary zone of both the corneae resulting in marked loss of vision.
Patient always used to lower his chin down and tried to look through the upper part of his eyes and walked w hit his head swinging rhythmically from side to side. On examination a coloboma of the right upper lid, involving half its length and exposing the supero-nasal part of the right bulb, was found. The growth, the size of a large bean and approximately 12 mm X 6 mm. in dimensions, had its long axis horizontal. Its colour simulated skin colour and had a few hair over it. It covered almost the whole of cornea except a crescentic area of 4 m.m width from 12-o'clock to 5-o'clock. This strip of cornea and adjoining conjunctiva (very small in area due to the absence of superior fornix) were dry, lustreless and xerotic probably due to exposure. There were sub-conjunctival bands underneath the conjunctiva simulating trachomatous fibrosis. Near the lateral canthus, a big sub-conjuctival growth could easily be demonstrated (Lacrymal gland ?). Though the anterior chamber, iris and pupil could not be identified and the corneal transparency was not ideal, still the patient could count fingers at 1 metre. This much vision was unexpected and surprising not only to me but also to other senior colleagues and Prof. Karl Lindner of Vienna, who was shown this case during his visit to our Hospital. Right eye ball seemed to be smaller in size with symblepharon near the upper formix. Its movements were restricted on all sides.
The left eye was almost normal in shape and size. The coloboma in the upper lid was not very marked and the whole of the corneal surface, except the upper part was covered by the growth (Dermoid with hair). The upper uncovered part of the cornea was very clear and almost of normal transparency, delimited inferiorly by a horizontal line. Through it the upper part of the iris, (though not the pupil) could be seen. With the left lid raised and the patient looking downward the vision came up to finger counting at 1 metre. There was defective closure of both lids of both the eyes but more marked in the right eye due to the pressure of pedunculated dermoids. The eyeballs did not show any tenderness or pressure.
Facial region showed broad nasal bridge, deviated nasal septum and a highly arched palate. A hare lip at the right side of the upper lip and a few super-numerary auricles were also found.
Findings of the department of Oto-rhino-laryangology :-Nasal Septum deviated to the right and mal-development of facial and nasal bones.
Findings of the Dept. of Radiology- . A calcified shadow in the occipital region. There was deficiency in bone. Its orbital distance appeared to be increased. Sella Turcica and bony orbits were normal.
After being admitted in the in-patients' department in the first week of August 1955, the growth in the left eye was first shaved off from the cornea on 22nd August 1955 under local anaesthesia and the raw bed was covered by a conjunctival hood. 50,000 units of Penicillin was pushed sub-conjunctivally after the operation. On the 3rd day after operation the conjunctival hood could not be separated from the cornea due to firm fixation.
On a subsequent sitting a mucous graft from the patients' lower lip was taken and placed on the bulbar area of the right eye, adjoining the superior one third of the limbal ring, as it was in this area that the superior fornix was deficient and the conjunctiva and cornea were xerotic, dry and lustreless. The graft took very nicely at its nasal and temporal parts but was somewhat loose at the central part, thus making a loose hood or fold, which subsequently used to hang over the only functioning part of the cornea, thus obstructing patient's vision. The depth of the sueprior fornix, also could not be imporved.
On a third sitting, the dermoid of the right eye was similarly shaved off from the cornea and the exposed rough corneal surface was simply cauterised by heat cautery. At the time of operation and a few weeks following the operation, the visual acuity of the patient improved by this removal, but subsequently the muco-conjuctival hood used to cover the cornea from the superior side and the patient had to lift up his right lid manually in order to see more clearly. The corneal dryness and haziness, still remained and the cause was thought to be exposure keratitis due to colobomatus lid but in March 1958, when he visited the hospital we found the cronea fairly clear, brighter and more transparent. So the idea of repairing the coloboma is still subjudice.
The growths were sent to the pathologist, Eye Infirmary, Medical College and the section of the left growth showed stratiform epithelium with true characteristics of epidermis. Underneath the stratiform epitheleium was a dense layer of fibrous tissue containing sebacous glands, hair follicles, and elastic fibres [Figure - 3],[Figure - 4].
These growths were in all probability corneal and most probably were very small when the patient was born but because they had grown later on, they covered almost the half or more of the corneal surface and protruded through the palpebral fissure thus becoming pedunculated.
The peculiar swinging movement of the eyes with the lowered position of the head, proves that the patient had been using the upper part of the cornea from his infancy otherwise this type of co-ordination between head, eyes and locomotor systems would not have been possible. Luckily for the surgeon, the growths were superficial and the corneae were not pierced duiing the removal of any of the dermoids.
Other abnormalities in association with the dermoid happen in 30 per cent of all cases (Duke Elder, 1943). Cohen's (1942) case is analogous. About the aetiology, Herrenschwand (1916) pointed out that an organic adhesion of the lid margin to the surface of the globe might be the cause. Some of the adhesions might be the residue of amniotic bands. But it is difficult to surmise whether the anomaly is due to any genetic or environmental factor. In this particular case, there was antipartum haemmorrhage at 3rd month of pregnancy in the patient's mother.
A case of bilateral dermoid with coloboma of the upper lids, extra auricles and hare-lip is described. The patient used to make a swinging head movement during locomotion.
I am grateful to the Superintendent, R. G. Kar Medical College for his kind permission to publish this case and also to the respective heads of the E. N. T. and Radiology Dept. for their kind co-operation in investigating this case. I am also indebted to Prof. K. Sen for his kind help in making of sections of the growths.
| References|| |
Cohen, (1942) Med. J. Australia, ii,36o, 1942.
Herrenschwand, (1916) K. M. Aug./vi, 471.
Duke Elder, Sir W. S., Text book of Ophthalmology Vol. II p. 1407-1408. Henry Kimpton-London 1943.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]