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Year : 1966  |  Volume : 14  |  Issue : 6  |  Page : 258-259

Microphthalmos with cyst

Schell Eye Hospital, Christian Medical College, Vellore, India

Date of Web Publication17-Jan-2008

Correspondence Address:
D K Sen
Schell Eye Hospital, Christian Medical College, Vellore
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Sen D K, Thomas A. Microphthalmos with cyst. Indian J Ophthalmol 1966;14:258-9

How to cite this URL:
Sen D K, Thomas A. Microphthalmos with cyst. Indian J Ophthalmol [serial online] 1966 [cited 2023 Dec 8];14:258-9. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1966/14/6/258/38668

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 Top

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 Top

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 Top

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.[3]

  References Top

Baurmann (1923): K. M. Aug., LXXI, 227.  Back to cited text no. 1
Cosset (1877): Cited by Duke-Elder (1939).  Back to cited text no. 2
Duke-Elder (1939): Text-Book of Ophthalmology, Vol. II, P. 1268, Henry Kimpton, London.  Back to cited text no. 3


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