|Year : 1966 | Volume
| Issue : 6 | Page : 258-259
Microphthalmos with cyst
DK Sen, A Thomas
Schell Eye Hospital, Christian Medical College, Vellore, India
|Date of Web Publication||17-Jan-2008|
D K Sen
Schell Eye Hospital, Christian Medical College, Vellore
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sen D K, Thomas A. Microphthalmos with cyst. Indian J Ophthalmol 1966;14:258-9
A portion of retinal tissue may protrude into the surrounding tissue in the region of the foetal cleft. The knuckle of retina may be so large that a cystic protrusion is formed of dimensions larger than the eye itself. It is probable that the essential cause of such protrusions is an active poliferation of the ectodermal elements at the lip of the fisure (Baurmann, 1923). The eyeball itself is invariably small and frequently grossly illformed in such cases.
Such a condition was first accurately discribed by Arlt (1858) who associated it with a defective closure of the foetal cleft.
There are two other hypothesis about this condition, (1) by Talko who believed that development of the eyeball was prevented secondarily by the presence of a cyst in the lower lid which was not in connection with the eyeball at all and (2) by Cusset (1877) who thought that they were dermoid cysts developed in relation to the branchial cleft.
Pathological investigations have favoured Arlt's hypothesis (1858).
The condition is so rare as to warrant mention. Only four cases were seen in such a large Hospital as the Schell Eye Hospital, Vellore, during the seven years from 1959 to 1965. There was nothing particular in the antenatal history in all the cases. Delivery was also normal.
| Case Report|| |
Case No. 1.
K., 10 days old, female, Mohammedan child, was brought on 26-5-1964 as the child was not opening the eye since birth. On examination, the palpebral apertures were found to be narrow on both sides but more so on the right. The lids on both the sides were relatively immobile. On the right side there was a swelling (1.5 cm. x 1.5 cm.) of the lower lid. On opening the lids an orbito palpebral cyst was found. No eye ball could be visible clinically. However, it could be palpated through the lids. The cyst could be transilluminated and a dark shadow was observed on one side of the cyst. No cyst could be made out on the left side but the eye was microphthalmic. There was no evidence of any other congenial abnormality elsewhere in the body. There was nothing particular in the family history.
Case No. 2.
This case attended the hospital on 2-9-1965. This was a male Hindu child, 21 days old. Here the picture was similar to the first one. An orbitopalpebral cyst was causing a swelling of the lower lid on the right side. No eyeball could be visible clinically. Palpation, however, revealed its presence. Left eye was microphthalmic without any deformity. There was nothing particular in the family history.
Case No. 3
A new-born female child, 2 days old, was brought to the Hospital on 19-11-1965 by her relatives as the eyes of the child appeared abnormal to them. On examination both the sides were found to be microphthalmic.
However, the child had no orbito-palpebral cyst on either side.
Mother of this child was reported to be normal.
Case No. 4.
The father of case No. 3, aged 45 years, when examined, was found to have microphthalmia with orbitopalpebral cyst on the right side which was embeded deep in the lower lid. On opening the lids the eyeball was found to be very much smaller, grossly abnormal and situated deep in the socket. On the left side the size of the eyeball was normal but there was a congenital coloboma of the iris at 6 o'clock position. No other congenital abnormality could be detected. Fundus could not be seen because of the presence of advanced senile cataract on this side.
| Comments|| |
Congenital microphthalmos with cyst may be sporadic or hereditary. Some sporadic cases are secondary to exogenous factors, such as infections (German measles, syphilis etc.) in the mother and embryo during early pregnancy. Most sporadic cases however, are undoubtedly due to mutations and other random factors not well understood. Here, there is no family history of microphthalmos for several generations back. However, the most interesting cases of congenital microphthalmos are inherited genetically.
| Summary|| |
Three cases of Microphthalmos with orbito-palbebral cyst are reported. In one of them, the father of the child also had microphthalmos with an orbito-palpebral cyst.
Four cases of this condition have been briefly described. Three of them have been described in babies 2 to 21 days old and one in the father of one of these babies.
| References|| |
Baurmann (1923): K. M. Aug., LXXI, 227.
Cosset (1877): Cited by Duke-Elder (1939).
Duke-Elder (1939): Text-Book of Ophthalmology, Vol. II, P. 1268, Henry Kimpton, London.