Table 1

Clinical features

CaseSexAge at presentationClinical featuresB/L or U/LSystemic featuresClinical diagnosis
HCD = horizontal corneal diameter, RE = right eye, LE = left eye, ASD = atrial septal defect, VSD = ventricular septal defect, U/L = unilateral, B/L = bilateral.
1 (KL)F3 daysDiffuse panstromal corneal clouding (see Fig 1)B/LNilCongenital glaucoma
2 (CC)M5 daysRE complete opaque cornea (see Fig 5)U/LCHARGE syndromeSclerocornea
Bilateral choanal atresia ASD, VSD, coarctation of aorta, micropenis
3 (CC)20 daysRE opaque enlarged cornea with stromal vascularisationU/LNilPeters' anomaly
? Keratolenticular adhesion
4 (KP)F4 daysB/L dense central corneal opacities with peripheral irido-corneal adhesionsB/LOrofacial digital syndromePeters' anomaly
Cleft lip and palate, brachycephaly, short femurs, brachydactyly, clinodactyly
5 (MG)M4 daysB/L corneal opacification with a central area of relative ectasia posterior embryotoxon (see Fig 3)B/LNilCorneal ectasia
6 (SH)F7 daysLE central corneal opacity with irido-corneal touchU/LNilPeters' anomaly
7 (VCN)M7 daysB/L scleralised corneas temporallyB/LNilSclerocornea
8 (EF)M5 daysOpaque corneas HCD 11 mm R and 11.5 mm LB/LNilCongenital glaucoma
9 (NS)M7.5 monthsB/L opaque corneas with evidence of scleralisationB/LNilSclerocornea
10 (ET)M6 weeksB/L diffuse opaque corneas with complete scleralisation RE and some sparing inferiorly LEB/LNilSclerocornea
11 (TH)M5 monthsB/L diffuse opaque corneas with microcornea and scleralisationB/LIntrauterine growth retardation, failure to thriveSclerocornea
12 (RM)M7 daysB/L corneal ring opacities with thinning centrallyB/LPeters' plus syndromePeters' anomaly
B/L cleft lip and palate, rhizomelia, clinodactyly, long philtrum
13 (MR)F7 daysRE central corneal opacityU/LNilPeters' anomaly
LE Axenfeld anomaly but clear cornea (see Fig 4)