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Year : 1988  |  Volume : 36  |  Issue : 2  |  Page : 100-101

Microphthalmos with upper eye lid cyst-A case report

Deptt of Ophthalmology, Rajendra Medical College & Hospital, Ranch, Bihar, India

Correspondence Address:
Satyendra Thakur
Deptt of Ophthalmology, Rajendra Medical College & Hospital, Ranch, Bihar
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Source of Support: None, Conflict of Interest: None

PMID: 3235157

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One case of microphthalmos with upper eye lid cyst in the right eye and malformed left eye is presented. The cyst with microphthalmos was excised which demonstrated a nearly normal anterior segment but disorganized and folded retina and choroid with thin sclera.

How to cite this article:
Thakur S, Kashyap B P. Microphthalmos with upper eye lid cyst-A case report. Indian J Ophthalmol 1988;36:100-1

How to cite this URL:
Thakur S, Kashyap B P. Microphthalmos with upper eye lid cyst-A case report. Indian J Ophthalmol [serial online] 1988 [cited 2021 Jun 17];36:100-1. Available from: https://www.ijo.in/text.asp?1988/36/2/100/26153

  Introduction Top

Microphthalmos with upper eye lid cyst is a rare congenital anomaly which is caused by some defect in the closure of the fetal fissure and invagination of the optic vesicle. This abnormality arises in humans bet­ween the 5 and 14 mm stages of gestation.

In this paper, an interesting aspect of this anomaly is presented.

  Case report Top

M S. a 15 year old male Muslim was admitted in the Eye Depai tiiient of this Medical College, with complaints of no vision and restriction of movement in the right eye since his childhood and repeated attacks of pain with increasing swelling of the same eye for the last few years. For the last 15 days he had constant pain, watering and discharge from the same eye. In the left eye there was diminution of vision.

There was no significant family history.

General examination revealed no abnormalities. Exa­mination of the right eye [Figure - 1] : There was a cystic round swelling measuring about 8 cm in diameter protruding from the upper eye lid like a balloon with glistening skin and engorged blood vessels over it raised temperature, transilluminant immobile upper eye lid and slit like palpabral aperture. There was lacrimation and discharge present on the eye lid margin Nothing resembling eye was seen. There was no light perception.

Examination of the left eye

The eye ball was small with horizontal nystagmus, small cornea measuring about 8 mm in diameter, typical coloboma of the iris and a small dot shaped lenticular opacity in the lower part Vision reduced to finger count at about 3 meters distance. Ophthalmoscopically there was a typical coloboma of the choroid.

INVESTIGATIONS : Routine blood, urine and stool test­ing yielded normal results.

SURGERY After controlling the infection, the cyst was excised completely under generl anaesthesia, the cyst ruptured during dissection. It contained yellowish fluid. The stalk of the cyst was attached to the postero- inferior aspect of the globe. A very small eye ball was attached to the lower part of the cyst and was removed


Gross [Figure - 2] The specimen consisted of small eye ball measuring about 15 x 13 x 15 mm The cornea was about 6 mm in diameter with opacity in the centre and haziness on the periphery. A large cyst measuring 48 x 42 mm was present The cyst was unilocular, thin walled with inner trebeculations and the lumen cont­ained yellowish fluid.

Microscopic [Figure - 3] : Cornea showed avascular scarring Descemet's membrane and the endothelium were not clearly viewed. The angle of the anterior chamber, iris and ciliary body appeared normal. The retina was composed off disorganized glial tissues with folds Disorganised choroidal tissue was seen at some places. Sclera was thin. The cyst consisted of an outer fibrovas­cular layer and inner thick neuroglial tissue layer [Figure - 4].

DIAGNOSIS : Right eye : Microphthalmos with upper eye lid cyst Left eye - Microphthalmos with typical uveal tissue coloboma and dot shaped lenticular opacity.

  Discussion Top

Microphthalmos may be unilateral or bilateral, when unilateral the other eye is frequently malformed [1],[2] In this reported case there was a typical coloboma of the uveal tissues in the left eye which was also malformed.

In humans, the embryonic fissure forms during the invagination of the optic vesicle at the 5-8 mm stage and is completed at 14 mm of gestation [2],[3]. Typical colobo­matous abnormalities and microphthalmos with cyst occur at this time. The embvonic fissure begins to close centrally at about the 8 mm stage and fuses both anteriorly and posteriorly by the 20 mm stage [2],[3]. This inner layer of the optic cup grows and develops faster than the outer layer which causes eversion of the margin of the fissure.

Microphthalmos with or without cyst is due to inherited cause [1]

The microphthalmic eye is often obscured within the orbit by conjunctival folds or by a large cyst [5]. The size of the cyst varies. It may be small enough to escape clinical detection or it may be enlarged, probably because of an increase in fluid filling the cavity' the cause is unknown. Since the cyst grows after birth, it distends the lower lid frequently because the embryonic fissure from which it develops is located inferiorly [2] Occasionally the cyst is present beneath the upper eye lid [2],[6],[7], either because it is large enough to extend around the globe superiorly or because it arose from an atypical superior coloboma In this reported patient the cyst distened the upper eye lid like a baloon - which is a rare presentation.

The anterior segment of the eye ball may appear nearly normal or may display marked derangement [8]. The retina may show focal presentation of its architecture, but is usually disorganized, gliotic, detached and folded [1]. In the present case the anterior segment was nearly normal. Choroid and retina were disorganized and folded Sclera was thin.

Histopathologically the eye is always malformed [1]. The cyst was two layered structure, inner layer is composed of neurologial tissue and outer layer consists of connec­tive tissue.

  Acknowledgements Top

I am thankful to Dr. P.V.R. Sinha, Prof & Head of Pathology, Rajendra Medical College, Ranchi for his guidance in the pathological study of this case.

  References Top

Mal/eu, TA Jr., Battle, m., survey of Ophthal, 13: 200-206,1969.   Back to cited text no. 1
Duke Elder, S., System of Ophthalmology, Vol III, Part IL Henry Kimpton, London, 451-495, 1972.  Back to cited text no. 2
Mann, L, Developmental abnormalities of the eye, Ed. 2, J.K Lippincott Philadalphia, 79-94: 1957.  Back to cited text no. 3
Weyman, M.F., Am J. Ophthal, 8 : 214-216, 1925,   Back to cited text no. 4
Waring G.O. et at Am J. Ophthal, 82: 714, 1976.   Back to cited text no. 5
May, CH., et al, Arch Ophthal, 35.424,-1906.  Back to cited text no. 6
Dollfus, M.A, et al., Am J. Ophthal, 66: 504, 1968.   Back to cited text no. 7
Arstikaitis, M., Arch Ophthal, 82 : 480, 1969.  Back to cited text no. 8


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]


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