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   Table of Contents      
CASE REPORT
Year : 1993  |  Volume : 41  |  Issue : 3  |  Page : 130-132

Congenital microcoria : A study in three generations


Department of Ophthalmology, King George's Medical College, Lucknow, India

Correspondence Address:
Sandeep Saxena
M.S., G-19 River Bank Colony, Lucknow, 226018
India
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Source of Support: None, Conflict of Interest: None


PMID: 8125547

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How to cite this article:
Saxena S, Saxena R C. Congenital microcoria : A study in three generations. Indian J Ophthalmol 1993;41:130-2

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Saxena S, Saxena R C. Congenital microcoria : A study in three generations. Indian J Ophthalmol [serial online] 1993 [cited 2018 Nov 17];41:130-2. Available from: http://www.ijo.in/text.asp?1993/41/3/130/25603



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Congenital microcoria is a developmental ocular abnormality characterised by a pupil less than 2 mm in diameter in daylight, and non-responsive to light or accommodation. [1],[2] This condition was first noted by Wide. [3] It occurs sporadically or as a hereditary trait in association with other ocular abnormalities including myopia, pupillary membrane, microphakia, 'spherophakia, ectopic lens, or congenital glaucoma with gonio-dysgenesis. [1],[2],[4],[5] As of now, the precise cause of congenital microcoria is unknown. Previous studies using light microscopy have demonstrated defective development of the iris dilator muscle. [1],[2],[4],[5] Recent electron microscopic observations have shown a specific defect in the differentiation of intermediate filaments, with apparent failure of development of functional myofilaments and functional pupil dilator muscle. [6]

We herewith, report hereditarily transmitted congenital microcoria in 16 of the 33 members of three generations belonging to Hindu vaishya family.


  Observations Top


Thirty-three members of three generations belonging to a Hindu vaishya family were examined. Of these, 16 members (10 men and 6 women) were identified as having congenital microcoria, as per the definition of the disease [Table - 1], [Figure - 1]. The age of the patients ranged from 5 to 75 years. All 16 patients had bilateral involvement. The irides of all the patients were brown and showed poor development of collarettes and crypts. The pupils were less than 2 mm in diameter and there was no reaction to light or accommodation. Mydriatics such as 1% atropine, 1% cyclopentolate hydrochloride, 1 % tropicamide and 10% phenylephrine hydrochloride failed to dilate the pupils.

Axial length of 21 eyes of 11 patients was measured. (BioPhysic Medical Paxial Biometer-­Pachymeter, France). Of these 11 patients, 8 were men and 3 were women. Their age ranged from 5 to 65 years with a mean (+ Standard deviation) of 28.09 ( 20.49) years [Table - 2]. One eye with total retinal detachment was excluded from the study. Axial length of the eyes with congenital microcoria ranged from 20.53 to 30.50 mm, with a mean ( S.D.) axial length of 25.60 (+ 3.19)mm [Table - 3]. Coefficient of correlation was calculated to be 0.55.


  Discussion Top


Sporadic occurrence [1],[2],[5],[6],[7],[8] as well as hereditarily transmitted [4],[7] cases of congenital microcoria have been reported. In our present study, we found 16 patients with congenital microcoria among 33 members of three generations belonging to a Hindu vaishya family, the largest ever family pedigree studied.

Though there have been earlier reports of association of myopia with congenital microcoria; [7],[8] to the best of our knowledge, ocular axial length in congenital microcoria has been measured for the first time. The mean axial length of the eye was found to be increased. The coefficient of correlation showed a positive association between age and axial length of the eye.



 
  References Top

1.
Coulon G, Do lbosc B, Jeffredo Y, Viennet G, Oppermann A, and Royer J. Congenital microcoria: A case report with histopathological study.) Fr Ophthalmol. 9: 35-39, 1986.  Back to cited text no. 1
    
2.
Hyams SW and Neumann E. Congenital glaucoma and combined mechanism glaucoma. Am J Ophthalmol. 68: 326-327, 1969.  Back to cited text no. 2
    
3.
Wilde. Malformations and Congenital diseases of the organ of sight. London. 1862.  Back to cited text no. 3
    
4.
Tawara A, and Inomata H. Familial cases of Congenital microcoria associated with late onset congenital glaucoma and goniodysgenesis. Jpn J Ophthalmol. 27: 63-72, 1983.  Back to cited text no. 4
    
5.
Holtman HW. Ein Fall von isolierter beiderseitiger Micro­coria and Linsenektopie. Klin Monatsbl Aegen heild. 166: 98-101. 1975.  Back to cited text no. 5
    
6.
Simpson WA, and Parsons MA. The ultrastructural pathological features of Congenital Microcoria: A case report. Arch Ophthalmol. 107: 99-102. 1989.  Back to cited text no. 6
    
7.
Duke-Elder S. System of Ophthalmology. Vol 3 Part II. Congenital deformities. Henry Kimpton. London. pp 590­591, 1964.  Back to cited text no. 7
    
8.
Butler JM, Raviola G, Miller CD, and Friedman Al Fine structural defects in a case of congenital microcoria. Graefes Arch Clin Exp Ophthalmol. 227: 88-94, 1989.  Back to cited text no. 8
    


    Figures

  [Figure - 1]
 
 
    Tables

  [Table - 1], [Table - 2], [Table - 3]


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