Indian Journal of Ophthalmology

ARTICLE
Year
: 1967  |  Volume : 15  |  Issue : 6  |  Page : 241--242

Bilateral congenital anophthalmos


DL Maria, SK Srivastava, PS Sankholkar 
 Medical College, Aurangabad, India

Correspondence Address:
D L Maria
Medical College, Aurangabad
India




How to cite this article:
Maria D L, Srivastava S K, Sankholkar P S. Bilateral congenital anophthalmos.Indian J Ophthalmol 1967;15:241-242


How to cite this URL:
Maria D L, Srivastava S K, Sankholkar P S. Bilateral congenital anophthalmos. Indian J Ophthalmol [serial online] 1967 [cited 2020 Apr 2 ];15:241-242
Available from: http://www.ijo.in/text.asp?1967/15/6/241/38818


Full Text

Anophthalmos is a rare congenital anomaly. It is defined as a condition in which there is complete absence of one or both eyes. It is of great interest to embryologists, anatomists and oph­thalmologists. The condition was first noted in 1557, but it was reliably re­ported by Bartholin in 1657 (SORSBY 1934). A few cases have been report­ed since (MANN and COLLINS 1887) Van HIPPEL 1900, WARFEL 1961, MICHAELS and ZUGSMITH 1963, IMAM 1964, MARIA and SHUKLA 1965, MEHRA 1965, INGLE, TAL­WALKAR and MARIA 1966. Be­cause of the rarity of this anomaly and few reports from the country, this case of bilateral congenital anophthalmos is being reported.

 Case Report



A two days old male child was referred to the ophthalmic Department from the Obstetric and Gynaecology department on 15-2-67 for examination of eyes. This child was the third son, the eldest one was male aged seven years, alive and the second was a female child, who died at two years of age. The last delivery was three years back. Both previous deliveries were full-term and normal. There was no congenital deformity.

 Examination



The subject was a premature (32 weeks) male child weighing 1.75 Kg. Both the orbits were small, larger in the transverse diameter than the verti­cal. Both eye-lids and eye-lashes were present. The palpebral fissures were narrow but equal on both sides and the lacrimal puncta were present. The conjunctival sac was reduced in size.

The eye-ball could not be felt on pal­pation but movements of the conjunc­tival sac were noticed which must be due to the presence of extraocular mus­cles. The lids were kept closed. No other congenital abnormality was seen in the child. The orbits measured 25 mm in transverse diameter and 14 mm in vertical diameter, giving an orbital index of 56, normal value for the col­oured being 84. The palpebral fissures measured 10 mm on both sides. The anterior and posterior fontanelle were normal. Other systems did not reveal any abnormality. Photos I and II.

 Discussion



According to MANN (1937), there are three types of anophthalmos.

Primary Anophthalmos: There is a failure of formation of the optic pits which appear at 2 mm stage. The de­fect is restricted to the optic pits only, since the rest of the brain develops nor­mally. Usually, the condition is bila­teral and sporadic. The orbit and the other mesodermal structures develop normally though they may be mal­formed.

Secondary Anophthalmos: This is due to absence or abnormal develop­ment of the fore-brain as a whole. It usually results in nonviable monsters.

Degenerative anophthalmos: This is due to the subsequent degeneration of a primarily formed optic vesicle. The etiology is suggested by the presence of a small fibrous nodule or rudimen­tary lens structure. There is usually no gross anomaly of the brain. There does not appear to be any particular sex incidence, nor is there any evidence of parental transmission except in rare cases. van DUNSE (1899) postulated an inconvincing theory of suppression of optic vesicle by thickened amnion.

Germinal influence is present in cases associated with other congenital ano­malies. Genetic and chromosomal ab­normalities may be responsible in few cases. The sporadic and isolated occur­rence of anophthalmos with no history of disease or consanguinity suggests an environmental cause. This is sup­ported by experimental work wherein suppression of the optic vesicles in animals has been produced by tera­togens like X-rays, deficiency or ex­cess of vitamin A etc. It may be con­cluded that there is no one cause but multiple factors responsible in the eti­ology of anophthalmos.

 Summary



A case of bilateral anophthalmos of the primary type in a third male off­spring of a family has been reported. The subject is reviewed in brief.[12]

References

1BARTHOLIN (1657) Hist. anatomica­rum rariorum Amsterdam quoted in 3.
2COLLINS (1887) Roy Lond. Ophthal. Hosp. Rep. 11, 429 quoted in 3.
3DUKE ELDER, S. (1964) System of Ophthalmology Vol. III Part 2 Henry Kimpton, London P. 416.
4INGLE V. N., TALWALKAR B., MARIA D. L. 1966 - Ind. J. Pediat, 33; 149.
5IMAM S. N. (1964) Patna J. of Med. ophthalmic special 242-243.
6MANN IDA (1937) "Developmental Abnormalities of Eye" Cambridge P. 65-72.
7MARIA D. L. and SHUKLA S. P. (1965) J. All India opthalmic Soc. 13, 121.
8MEHRA, K. S. (1965) Anophthalmos amer. J. Ophthal. 60, 341.
9MICHAELS D. D. and ZUGSMITH G. S. (1963) Amer. J. Ophthal. 50, 1256.
10SORSBY. A. (1934) Brit. J. Ophtha. 18, 469.
11VAN DUYSE (1899) Arch. Ophthal. (Paris) 19412.
12WARFEL J. J. (1961) Amer. J. Oph­thal. 51, 698.