Year : 1990 | Volume
: 38 | Issue : 4 | Page : 205--206
Congenital cystic eyeball
VP Gupta1, K Uma Chaturvedi2, DK Sen3, KK Govekar3,
1 Department of Opthalmology, University College of Medical Sciences and Guru Teg Bahadur Hospital, Shahdara, Delhi - 110 095, India
2 Department of Pathology, Maulana Azad Medical College, New Delhi 110002, India
3 Gruru Nanak Eye Centre, Maulana Azad Medical College, New Delhi 110002., India
V P Gupta
University College of Medical Sciences and Guru Teg Bahadur Hospital, Shahdara, Delhi 110 095
A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye«SQ»s predilection for the left eye has been pointed out for the first time in this article.
|How to cite this article:|
Gupta V P, Chaturvedi K U, Sen D K, Govekar K K. Congenital cystic eyeball.Indian J Ophthalmol 1990;38:205-206
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Gupta V P, Chaturvedi K U, Sen D K, Govekar K K. Congenital cystic eyeball. Indian J Ophthalmol [serial online] 1990 [cited 2020 May 30 ];38:205-206
Available from: http://www.ijo.in/text.asp?1990/38/4/205/24495
Congenital cystic eyeball, a term introduced by Ida Mann  in 1937, is a rare malformation. The condition has also been called as "Anophthalmos with cyst". The congenital cystic eyeball develops when the. primary optic vesicle fails to invaginate. The orbit is filled by a cystic mass; there is no rudimentary eyeball. The lids and conjunctival sac are fully developed. Two previous case reports from India did not document the histopathological findings of congenital cystic eye ,. The purpose of this article is to report unusually large congenital cystic eyeball with histopathological observations. Its predilection for the left eye has also been hinted for the first time in this article.
A one day old female infant was admitted in Guru Nanak Eye Centre, Maulana Azad Medical College and LNJPN Hospital, New Delhi, on 10th March 1988, because of a large swelling protruding between the left eyelids since birth. The delivery was an uneventful full term pregnancy with normal vaginal delivery.She was the first child born to her parents.
Examination revealed a 2.5kg. healthy female baby without any systemic abnormality. Ocular examination revealed a large pinkish swelling about the size of an egg, bulging forward and stretching the upperlid. [Figure 1]. The swelling was translucent, cystic in consistency, non-tender, non-reducible and non-pulsatile. It was covered by conjunctiva from which slight oozing of blood was seen; crusts of dried blood on the surface of conjunctiva were noted. The lowerlid was hidden underneath the cyst; the upperlid was markedly distended displacing the eyebrow. [Figure 1]. Clinically there was no trace of a rudimentary eyeball. The right eye was essentially normal.
Roentgenograms of the skull showed microcephaly and symmetrical enlargement of the left orbit with a soft tissue shadow. No radiological evidence of encephalocele was present. A-scan ultrasonography substantiated the cystic nature of the mass with clear contents within and absence of a rudimentary eyeball.
At the age of 13 days the cyst was removed. A curvilinear incision along the inferior orbital margin was made. The cyst (approximate size 4.5x3.5x2cm.) was removed in toto after separating it from the conjunctiva and surrounding tissues. There was no optic stalk for the cyst. Complete absence of the rudimentary eyeball was confirmed at surgery.
Histopathological examination of the mass revealed a unilocular cyst with an outer wall composed of fibrous tissue into which were inserted the rudimentary tendons of the extraocular muscles. The inner wall of the cys was constituted by neural tissue in a disorganized man ner [Figure 2], however, focal areas of retinal differentiatior [Figure 3] could be seen. In another area the retinal pig ment epithelium [Figure 4] could be identified. There was however, no evidence of cornea, lens, uveal tissue anc vitreous. Absence of optic stalk was also confirmed.
Congenital cystic eyeball is an extremely rare anomaly. It develops as a result of partial or complete arrest in the invagination of the primary optic vesicle between the 2mm. and 7mm. stages of fetal development .
The exact aetiology is not known. But it is likely that environmental factors are operative. Evidences of inflammatory cells in the cyst favours an inflammatory cause .
Congenital cystic eyeball is present since birth; it replaces the normal eyeball. Typically it is a large orbital mass which causes the upperlid to bulge forward. All these features were seen in this newborn. In majority of the cases the cyst is centrally placed in the orbit or may bulge more towards the upperlid as in this case, however Dash et al reported a case of congenital ectoptic encysted eyeball in the lowerlid .
The cyst may be single or may have two or more loculations. The size of the the cyst may to vary. The patency of the stalk is believed to influence the size of the cyst. If the optic stalk remains patent the excess of the fluid could go back to the ventricles of the brain and the cyst remains small . If the lumen of the stalk is blocked the cystic eye becomes large. Collins and Johnson reported the largest cystic eyeball with no trace of the optic stalk . The absence of optic stalk in our case thus explains the occurrence of a very large cyst. Normal or anomalous extraocular muscles may be attached to the cystic eye. Rudimentary tendons of the extraocular muscles were seen in this patient. Absence of the lens in this case favours the concept that the abnormality occurred before the 7mm.stage.
X-rays skull in this patient revealed microcephaly and the affected orbit was larger in size. Dollfus et al  and Baghdassarian et al  also reported symmetrical enlargement of the orbit containing the cystic eyeball, whereas Helveston et al  reported a slightly smaller orbit on the affected side. The cysts reported by Dollfus et al  and Baghdassarian et al  and in the present case were unusually large. The presence of a large cystic eyeball in the orbit in utero for a sufficient period may cause the bones of the orbit to enlarge.
The malformation affects only one eye. Duke-Elder made mention of 16 such cases in his text , however, no predilection for a particular eye has been hinted at. The case reports published thereafter i.e. five case reports ,,,, have been reviewed by us. In all these case reports as well as in the present case, interestingly, only the left eye was involved. Why only the left eye is affected is yet to be explained ? Whether this is just a coincidence or it has got some embryological basis is to be investigated further.
|1||Mann I: (1957) :Development Abnormalities of the eye. Philadelphia, JB Lippincott Co., pp 66-69.|
|2||Dash RG. Boparai MS & Pai P (1984): Congenital ectopic encysted eyeball. Ind. J. Ophthalmol 34:247-248.|
|3||Shukla Y, Kulshrestha OP & Bajaj K (1984): Congenital cystic eye - A case report. Ind.J. Ophthalmology 34: 249-250.|
|4||Duke-Elder, S. (1964): System of Ophthalmology Vol.111, Congenital Deformities. Part II, Henry Kimpton London, pp 451 & 481.|
|5||Dollfus M.A. et al 1968): Congenital cystic eyeball. Amer J.Ophthalmol 66: 504-509.|
|6||Baghdassarian SA, Tabbara KF, Matta CS (1973): Congenital cystic eye Amer J.Ophthalmol 76: 269.|
|7||Helveston EM, Malone E & Lashment MH (1970): Congenital cystic eye. Arch. Ophthalmol 84: 622-624.|