Table 2

UBM and histology features

CaseUBM methodClinical featuresUBM featuresUBM DxPKPHistological featuresHistology Dx
1 (KL)EUANormal IOP under GANormal anterior segments, irregular corneal thickness with abnormal echogenecity from DM and endothelium. At its greatest corneal thickness was 2.3 mm RE and 2.2 mm LECorneal dystrophyB/LBE: vacuolation of the basal epithelium with intact Bowman's layer. Focal absence of DM with multilayering of the endothelium confirmed on EM. Immunostain positive for cytokeratin in endotheliumPosterior polymorphous dystrophy
2 (CC)AwakeShallow anterior chamber with keratolenticular adhesion and abnormal thick zonulesPeters' anomalyN/AN/AN/A
3 (CC)EUANormal IOPShallow anterior chamber with keratolenticular adhesion with cataract and aniridia. Zonules enmeshed with stretched or elongated ciliary processesPeters' anomalyN/AN/AN/A
4 (KP)EUANo evidence of glaucomaIridocorneal adhesions with a central posterior corneal defect. Hypoechoic region in the anterior stroma, not able to be explained at time of UBM. Features common to both eyesPeters' anomalyB/LLE: variable epithelial thickness with complete absence of Bowman's layer with anterior stromal oedema; complete absence of DM and endotheliumB/L Peters' anomaly
RE: Vacuolation of basal epithelium with variable thickness and irregular arrangement of the stromal lamellae. Centrally deficient DM with endothelial attenuation.
5 (MG)EUARE perforated and had emergency PKPOnly LE had UBM. Aniridia and central large posterior corneal defect in the region of which the hyperreflectivity of DM seen on UBM was absent. Ciliary processes appeared stretched. Hypoechoic region in anterior stroma similar to case 4.Peters' anomalyB/LLE: oedema of the basal epithelium, absent Bowman's layer, and irregular alignment of the stromal lamellae. Centrally marked thinning of stroma with absence of DM and endotheliumPeters' anomaly – most likely B/L but right host tissue only showed necrosis as it had perforated awaiting PKP
6 (SH)EUANo evidence of glaucomaCentral iridocorneal adhesion. Normal hyperreflectivity of DM and endothelium not seen centrally but present peripherallyPeters' anomalyU/LLE: variable epithelial thickness, thickened Bowman's layer with focal absence centrally, marked stromal disorganisation and irregular stromal thickness with a central mound of tissue in posterior cornea, over which DM extremely attenuated and focally absentPeters' anomaly
7 (VCN)EUAIridocorneal adhesions mainly central but also some peripheral with shallow AC. Hyperreflectivity normally seen at level of DM/endothelium not seen centrally. Hypoechogenic area in anterior stroma similar to that seen in cases 4 and 5.B/L Peters' anomalyB/LRE: oedema of the basal epithelium with absence of Bowman's layer. Disorganised layering of the stroma and absence of endothelium and DMB/L Peters' anomaly
LE: epithelial oedema, absent Bowman's layer, disorganised stromal layering and attenuation of endothelium with absence of DM centrally
8 (EF)EUAIOP: TonopenNo evidence of iridocorneal or keratolenticular adhesions or posterior corneal defects. Cornea thickened at 2.1 mm RE and 2.3 mm LE centrally. Normal hyperreflectivity of DM/endothelium stippled with discrete discontinuationsCorneal dystrophyB/LBE: oedema of basal epithelium and subepithelial bullae. Marked stromal scarring with irregular layering and severe endothelial attenuation with almost complete absence. Irregular fibrous thickening of Descemet's membrane, especially posterior part but complete in some areas.B/L congenital hereditary
R=37 mm HgEndothelial dystrophy
L=65 mm Hg
IOP: Perkins
R=10 mm Hg
L=10 mm Hg
9 (NS)EUANo evidence of glaucomaFormed anterior chambers with odd fragments of iris and aphakia. No evidence of posterior corneal defectAphakiaB/LRE: flattened and attenuated epithelium with absent Bowman's layer. Stroma abnormally organised, thin and vascularised. DM and endothelium could not be identified due to presence of adherent irisPrimary aphakia
Disorganised ant. segment
? Sclerocornea
LE: in addition to features above subepithelial calcification
10 (ET)EUANo evidence of glaucomaShallow ACs with posterior corneal defects with no keratolenticular adhesion but some irdiocorneal adhesions seen, centrally especially in right eye.Peters' anomalyLE: autorotational keratoplasty (elsewhere)N/AN/A
11 (TH)EUANo evidence of glaucomaFormed ACs with no corneal defect or iridocorneal or keratolenticular adhesionSclero-corneaN/AN/A
12 (RM)AwakeCentral thinning of the posterior cornea with keratolenticular and iridocorneal adhesionsPeters' anomalyB/LBE: absence of DM, endothelium and posterior stroma centrally with absent Bowman's layerPeters' anomaly
13 (MR)AwakeIOP: Perkins while feedingPeripheral iridocorneal adhesions. Centrally mild posterior corneal defect with corresponding defect in anterior capsule of lensPeters' anomalyU/LRE: centrally focal absence of DM and endotheliumPeters' anomaly
R=26 mm Hg
L=16 mm Hg