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Review
. 2023 Nov 23;13(4):461-466.
doi: 10.4103/tjo.TJO-D-23-00070. eCollection 2023 Oct-Dec.

Overview of sclerocornea

Affiliations
Review

Overview of sclerocornea

Rama Rajagopal et al. Taiwan J Ophthalmol. .

Abstract

Sclerocornea is a rare non-progressive, non-inflammatory usually bilateral congenital corneal opacity that can be associated with both ocular and systemic abnormalities. It could be inherited in 50% of cases. Ill-defined limbal architecture and vascularization in association with ocular comorbidities results in poor outcomes with corneal transplantation. This narrative review summarizes the current literature on etiology and clinical presentation in sclerocornea. With regards to keratoplasty, it focusses on key elements in decision making, highlights the role of investigations and discusses practical surgical pearls to enhance outcome of keratoplasty in these eyes.

Keywords: Cornea plana; investigations; penetrating keratoplasty; sclerocornea; systemic associations.

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Conflict of interest statement

The authors declare that there are no conflicts of interests of this paper.

Figures

Figure 1
Figure 1
Slit-lamp image of sclerocornea with peripheral corneal opacification and vascularization and a central corneal haze
Figure 2
Figure 2
Slit-lamp image of sclerocornea with corneal opacity involving the entire cornea
Figure 3
Figure 3
Slit-lamp examination (slit view) of sclerocornea showing cornea plana
Figure 4
Figure 4
(a) Microphotograph of the corneal button showing multilayered stratified squamous epithelium, loss of Bowman’s membrane along with thick collagen fibers with scattered fibroblasts, below corneal collagen fibers. (×100, H and E), (b) Microphotograph in higher magnification of scleral collagen fibers with scattered fibrocytes and vascular channels (×300, H and E)
Figure 5
Figure 5
Anterior segment optical coherence tomography image of sclerocornea to estimate corneal diameter and demonstrating increased corneal thickness
Figure 6
Figure 6
Ultrasound biomicroscopy image of sclerocornea showing thickened cornea, shallow anterior chamber, and peripheral anterior synechiae
Figure 7
Figure 7
Intraoperative image showing the retraction of the conjunctiva for adequate limbal visualization and sizing of the graft
Figure 8
Figure 8
Postoperative image of a patient with sclerocornea who underwent penetrating keratoplasty showing persistent epithelial defect

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