CLINICAL SCIENCE

Clinicopathological correlation of congenital corneal opacification using ultrasound biomicroscopy

K K Nischal¹, J Naor², V Jay³, L D MacKeen⁴ and D S Rootman^2,4

¹ Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, London, and The Institute of Child Health, London UK
² Department of Ophthalmology, Toronto General Hospital, University of Toronto, Toronto, Canada
³ Department of Pathology, The Hospital for Sick Children, Toronto
⁴ Department of Ophthalmology, Hospital for Sick Children, Toronto, Canada

Correspondence to:
Correspondence to:
K K Nischal, Department of Ophthalmology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK;
kkn{at}btinternet.com

Aim: To investigate the correlation between clinical, high frequency ultrasound biomicroscopy (UBM) and, where possible, histological findings in cases of congenital corneal opacification presenting to the departments of ophthalmology, Great Ormond Street Hospital for Children, London, and the Hospital for Sick Children, Toronto, Canada.

Method: 22 eyes of 13 children (age range 3–225 days) with congenitally opaque corneas were examined. UBM was performed using the ultrasound biomicroscope (Allergan-Humphrey). All eyes underwent penetrating keratoplasties (PKP) except five. The host corneas were all sent for histological examination.

Results: The final diagnosis in our series was Peters' anomaly in nine cases (70%), corneal dystrophy in two cases (15%), and sclerocornea in two cases (15%). The UBM findings changed the clinical diagnosis in five cases (38%). In these five cases histology was available in four and confirmed the UBM diagnosis in each case. In no case of the 13 where histology was available did it contradict the UBM findings. In two cases a hypoechoic region in the anterior stroma was seen on UBM which correlated histologically with absent Bowman's layer and oedema. In two cases UBM revealed aniridia and in one, congenital aphakia, which was not apparent clinically.

Conclusion: UBM examination is not only very useful in evaluating the clinical diagnosis in congenital corneal opacification, it also acts as a preoperative guide in cases undergoing PKP by detecting keratolenticular and iridocorneal adhesions and other ocular abnormalities such as aniridia and congenital aphakia. In all cases where PKP was performed the UBM diagnosis was confirmed histologically. The clinical diagnosis was incorrect in five cases. This has important implications in studies of phenotype/genotype correlation of congenital corneal opacification.

Keywords: congenital corneal opacification; ultrasound biomicroscopy

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Zhang, M, Chen, J, Liang, L, Laties, A M, Liu, Z (2006). Ultrasound biomicroscopy of Chinese eyes with iridocorneal endothelial syndrome. Br. J. Ophthalmol. 90: 64-69 [Abstract] [Full Text]