Elsevier

Ophthalmology

Volume 107, Issue 3, March 2000, Pages 511-520
Ophthalmology

Original Articles
Visual acuity in children with coloboma1: Clinical features and a new phenotypic classification system,

https://doi.org/10.1016/S0161-6420(99)00140-2Get rights and content

Abstract

Objective

The aims of this study were to describe the clinical features and biometric findings in the eyes of children with coloboma and to develop a classification of coloboma that correlates with visual function.

Design

Retrospective observational case series.

Participants

One hundred thirteen children and young adults (48 female, 65 male) aged 0 to 20 years with 196 eyes having coloboma.

Methods

Children with coloboma were recruited from schools for the blind, integrated education programs, schools for the mentally handicapped, community-based rehabilitation services, and hospital clinics in Andhra Pradesh, India, between January 1998 and January 1999. Visual function was assessed, including distance and near visual acuity (VA), and navigational vision. The corneal diameter and axial length of eyes were measured wherever possible.

Main outcome measures

Anatomic site of coloboma, association with microcornea and/or microphthalmos, VA, presence of navigational vision and reading vision.

Results

Of 196 eyes with colobomatous malformations, 11 had microphthalmos with cyst, and 185 eyes had coloboma (associated with microcornea in 155 eyes and with a normal corneal diameter in 30). Microphthalmos was present in 72 of the 185 eyes with coloboma, of which 71 of 72 also had microcornea. The prognosis for vision depended on the phenotype of the better eye. Microphthalmos with cyst had the worst prognosis (VA < 3/60, 100%; reading and navigational vision, 0%). Microcornea with microphthalmos had a worse prognosis than microcornea without microphthalmos (VA < 3/60: 66.7% vs. 23.3%; unable to read N10: 66.7% vs. 34.1%; no navigational vision: 30.6% vs. 6.73%). Simple coloboma (no microcornea or microphthalmos) had the best prognosis (VA < 3/60: 6.7%; able to read N10: 93.3%; navigational vision: 100%). A corneal diameter <6 mm had a poor visual prognosis, whereas a corneal diameter >10 mm had a good prognosis.

Conclusions

A phenotypic classification of coloboma is proposed, which in this study showed a good correlation with visual acuity, reading, and navigational vision. Microphthalmos with cyst had the worst prognosis, coloboma with microcornea and microphthalmos a poor prognosis, coloboma with only microcornea had an intermediate prognosis, and simple coloboma had the best prognosis.

Section snippets

Materials and methods

Children were recruited from special schools for the blind, integrated education programs, schools for the mentally handicapped, hospital clinic records and from a community based rehabilitation program in the Indian state of Andhra Pradesh between January 1998 and January 1999. Every child identified with coloboma was examined by use of the same methods by one general ophthalmologist (SJH) and an optometrist (SA). Examinations were performed in the schools or in nearby eye clinics. The ethics

Results

Colobomatous congenital malformations of the globe were found in 196 eyes of 113 individuals, 48 of whom were female (42.5%) and 65 male (57.5%). The remaining 30 eyes were either normal (n = 6) or had a noncolobomatous abnormality (n = 24). The age range was from 9 months to 20 years, with a mean of 11 years. Clinical findings are presented for individual eyes, and the results of VA and visual function are presented for the individual.

Discussion

The high proportion of children in this series with posterior segment colobomata, and the low number with purely unilateral disease, probably reflects recruitment bias in favor of those with lesions causing severe visual loss. Three quarters of the children (83 of 113) had bilateral signs with some form of coloboma in each eye, although asymmetry was common. Involvement of the macula by the posterior segment coloboma was associated with a poorer VA as shown in previous studies.21 Localizing

Conclusion

Measurement of corneal diameter and axial length is a useful adjunct to clinical examination in assessing the prognosis for vision in a baby or child with coloboma. The clinical classification system proposed in this article divides colobomatous disorders into four clinical groups. Eyes with microphthalmos and cyst and those with corneal diameters of 5 mm or less have very poor prognosis for vision. Colobomata associated with normal corneal size have a good prognosis apart from the risk of

Acknowledgements

The authors thank the dedicated field team of M. Ferozuddin, S. M. Fahrath, and B. Raju. Dr Xiaoling Liang translated reference 17 from Chinese into English.

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    Supported by grants from the British Council for the Prevention of Blindness (London, England), the Tjissen Foundation (The Netherlands), Dark and Light (The Netherlands), and Christoffel Blinden Mission (Bensheim, Germany).

    1

    The authors have no proprietary interest in any materials used in this study.

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