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   Table of Contents      
ARTICLES
Year : 1986  |  Volume : 34  |  Issue : 1  |  Page : 37-43

Major congenital colobomatous disorders of the globe


1 Deptt. of Ophthalmology, Medical College, Gauhati, India
2 Deptt. of Ophthalmology, Medical College Silchar, Gauhati, India

Correspondence Address:
Harsha Bhattacharjee
Department of Ophthalmology, Silchar Medical College, Silchar (Assam)
India
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Source of Support: None, Conflict of Interest: None


PMID: 3127340

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How to cite this article:
Bhattacharjee H, Islam A, Deka R, Chaudhury D, Bhuyon B. Major congenital colobomatous disorders of the globe. Indian J Ophthalmol 1986;34:37-43

How to cite this URL:
Bhattacharjee H, Islam A, Deka R, Chaudhury D, Bhuyon B. Major congenital colobomatous disorders of the globe. Indian J Ophthalmol [serial online] 1986 [cited 2020 Feb 19];34:37-43. Available from: http://www.ijo.in/text.asp?1986/34/1/37/26460

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Major blinding colobomatous disorders of the globe are not treatable. Except a possibility of prevention and rehabilitation there is probably no other way to face the problem. The present survey and clinical work is an isolated attempt to get an idea about its prevalence in this locality and also to know the clinical features, heredity and different demographic dates. A search is also made regarding influence of probable biological and environmental conditions on its causations.


  Materials and methods Top


A door to door survey was conducted in four villages of Nowgong district of Assam (Titatola Bari, Dewaguri, Uttar Potacolong, and Dakshin Potacolong) and in a sample of population of Guwahati city. Students of blind school of Guwahati and Beharampur were also examined. The results of the interrogations and examinations were noted down in a pretested proforma describing the desired aspects. Special Importance was given on demographic data like full details of living, social, family, occupational history and antenatal care of the mother. Duration of study was 3 years.

A complete study in the desired aspects was possible in 43 cases of various types of microphthalmos and 22 cases of different types of coloboma of the iris and fundus of which bilateral involvement was in 19 and 1 cases respectively.

Amongst various types of microphthalmos, most prevalent variety was colobomatous type.. Gross impairment of vision was in all the affected eyes (6/60 or less). All six nanophthalmic eyes were amblyopic and refraction ranged from + 10 D to + 12 D Sph with pseudopapilloedema in the fundus. All cases of microphthalmos with cyst were associated with ectropion of the lower lid and three had bluish discolaration over the cyst. Histopathological examination was possible in one case where the cyst was found to be communicated with the cavity of the eyeball through a narrow opening at the site of the primary foetal fissure. The cyst wall was lined by collagenous tissue and it was filled with gelatineous transparent semi solid fluid.

In all cases of coloboma of the fundus, where vision was better than 6/24, a scotoma in the field of vision was detected, but its area was relatively smaller than the corres­ponding retinal defect.

In all the affected families the standard of living was observed much below than average. In four villages average per capita income ranged from Rs 90.00 to Rs. 300.00 P.M. Source of income was cultivation and most of the products they sell in the nearby market. Food habit is as usual (both veg. and non veg.). Majority of the women of the rural area and their female guardians do not like to have antenatal care from the doctor, also they prefer to supply poor diet to the expecting mother with a conception that good food to the mother will produce a bigger size baby and might produce obste­trical problem. In the area of my survey the people do not accept any modern method of contraception.


  Discussion Top


Anophthalmos and other colobomatous disorders of the globe is the fifth cause of blindness in India[1]. The prime causes i.e. cataract, glaucoma, nutritional disorders, infective ocular diseases etc. will be defini­tely citner cured, controlled or prevented with successful implementation of the pro­gramme for control of blindness in near future[2]. Blindness due to congenital abnor­malities except only in in few cases are not accessible to present day ophthalmic care. It is evident that the congenital ophthalmic disorders will be the major challenge to face by the future ophthalmologists.

Genetic as well as environmental factors were found responsible for causation of different congenital abnormalities. Mostly the occurrence of such abnormality was sporadic and definite causative factor could not be found out.

Incidence of blinding congenital ocular abnormality was more amongst children and in older age group the incidence declines. In England and Wales the overall incidence of blindness due to congenital defects 27.5% and the same incidence was highest (70%) in age group 0 -4[3]. Ghafour[4] report­ed the same incidence amongst under five population was 17.5%. 50 per cent of the children attending school for blinds in England and Wales in 1963 were found to be suffering from genetically determined eye disorders[5].

In an isolated study Singh et a1[5] found that incidence of congenital ocular abnorma­lity was 10.5 per thousand live birth. 14.3% of such infant were associated with some form of familial or maternal congenital abnormality and 19% of such cases were associated with single or multiple systemic abnormality[6],[7].

Clinical anophthalmos and microphthal­mos with or without coloboma of the uvea was not uncommon in chromosomal abnor­mality and it might occur as an outcome of intrauterine infection by rubella, toxo­plasma etc. When the condition was due to a defect in the primary optic vesicle only then it might be associated with cataract, orbital cyst etc. In isolated cases the inheri­tance is autosomal dominant, autosomal recessive or X linked.

Coloboma of the iris and fundus may also occur with other congenital deformities and are often seen in chromosomal defects. The condition is usually sporadic, but the condition can be inherited as an autosomal dominant manner with variable penetration. A small coloboma in one sibling and extreme microphthalmosi in another may be found. Teratogen like X Ray and naphthalene can produce the same condition(16).

A positive association was noted between occurrence of general malformations and certain maternal biological factors like genital bleeding in early pregnancy and history of previous spontaneous abortions. Maternal infections by various micro organis­ms [9],[10],[11] exposure of maternal pelvis by X-Rays[12] were also found teratogenic. Kapoor et [13],[14] found deficient nutrition and dura­tion of pregnancy were important factors in production of congenital abnormality of the eye.

Nikijama et al[15] reported less mother in their 20's and more mother in their 30's produce children with cataract and microphthalmos. Lilienfeld thinks these factors are not relevant.

In the present study incidence of major colobomatous defects of the globe was 2.6 per 1000 rural people, incidence of such abnormality as a causative factor of blind­ness amongst the students of blind schools of Assam was found 10.6% and in general O.P.D attendance was 10 per 1000 blind people (independent of age). The abnorma­lity was found to be more prevalent in male and in rural people. Colobomatous microph­thalmos was the most commonly occurring colobomatous defect of the globe. 13.9% cases were associated with some form extra ocular congenital abnormality and in 41.26% had more than one associated congenital ocular abnormality. Five cases had associat­ed positive family history.

Suggestive antenatal and prenatal history of the mother was elicited in few cases. Perinatal age group was found suggestive in production of clinical analphthalmos [Table - 10],[Table - 11]. Maternal nutritional status was found an important association and duration of pregnancy was found as an indepentent factor.


  Summary Top


A clinical and epidemiological study on major colobomatous defects of the globe was conducted in locality of Assam under certain specific references. The result of the study is discussed.


  Acknowledgement Top


I am thankful to Prof. L.C. Dutta and Dr. H.N. Gogoi for their guidance and necessary permission to attend the eye camps in relation to this study. The survey work of four villages were conducted along with the `Collaborative study on cataract' sponsored by I.C.M.R.

 
  References Top

1.
Kohner, E.M., 1978, Trans. Ophthalmol Soc. U.K., 98: 299.  Back to cited text no. 1
    
2.
National Programme for Control of Blindness. Report for the year 1979-80 & 1980-81. Published by Ophthalmology Section. D.G.H.S. Ministry of Health and Family Welfare, Delhi.  Back to cited text no. 2
    
3.
Sorsby, A., 1972, The incidence and causes of blindness in England: and Wales. 1963-'68. Deptt of Health and Social Security reports on Public Health and Medical Subjects, 128. London HMSO.  Back to cited text no. 3
    
4.
Ghafour, I.M., 1983, Brit. J. Ophthalmol, 67: 209-213.  Back to cited text no. 4
    
5.
Fraser, G.R. & Friedmann, A.I., 1967, The Causes of Blindness in Childhood. Baltimore, Johns Hopkins Press.  Back to cited text no. 5
    
6.
Singh, V.P. Gupta. S.L., Jain, 1.S., Gupta, A., Bbakoo, O.N., Bulletin, P.G.I. Chandigarh, 1979, 13. 4. p 216,121.  Back to cited text no. 6
    
7.
Neel, J.V; 1938, Amer. J. Human, Genet.; 10: 398.  Back to cited text no. 7
    
8.
Nishimura, H., 1969, Congenital Malformations. ed. Franser, C.F. & Mckuick, VA., Excel pta Medica. Amstradaum. Princeton. P-2.18.  Back to cited text no. 8
    
9.
Ingalls, T.M., Tedeschi, C.G., and Helpenn, M.M., 1952, Amer J. Ophthalmol, 35: 311.  Back to cited text no. 9
    
10.
Goldstein, J.H., Hutt, A.E., 1972, Amer J. Ophthalmol, 73 : 333-335.  Back to cited text no. 10
    
11.
Roy, H.F. : Duiscl, R.A., 1966, Amer. J. Ophthalmol, 62: 237.  Back to cited text no. 11
    
12.
Masket, S.; Golioto, F.M., Best. M., 1970, Amer. J. Ophthalmol 70: 382.  Back to cited text no. 12
    
13.
Kapoor, S. and Kapoor, M., 1979, J. Paedia­tric Ophthalmol, 14: 382.  Back to cited text no. 13
    
14.
Stevenson, 1960, Ciba Symposium on Conge­nital Malformations. London. 241.  Back to cited text no. 14
    
15.
Nikijama, A. Fujiki, K., Tanka, U. and Yasuda, N , 1979, Amer. J. Ophthalmol. 88: 461.  Back to cited text no. 15
    



 
 
    Tables

  [Table - 1], [Table - 2], [Table - 3], [Table - 4], [Table - 5], [Table - 6], [Table - 7], [Table - 8], [Table - 9], [Table - 10], [Table - 11], [Table - 12]



 

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