PMC3350197_01
(a) The corneal bulge between the eyelids in a 62-year-old male. (b) Slit lamp photograph showed that the right cornea had the appearance of a smooth-surfaced opacified and vascularized protrusion bulging anteriorly.
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At the first consultation we found that he could not blink because of markedly progressing corneal bulge (Figure 1(a)). Slit lamp examination showed that the right cornea had an appearance of a smooth-surfaced, opacified, and vascularized protrusion bulging anteriorly between the eyelids (Figure 1(b)).
MRI image. The inside of the bulging lesion was not occupied with a tissue mass and lens was undetected. (a) T1, (b) T2.
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Magnetic resonance imaging (MRI) demonstrated that the inside of the bulging lesion was not occupied with a tissue mass and lens was undetected (Figure 2).
(a) Gross examination of the globe showed a large corneal staphyloma and very small remnant lens. Arrow: corneal limb. Asterisk; lens. (b) Histology of the corneal protrusion with HE staining. Bar: 300 mum.
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Gross examination of the globe showed a large corneal staphyloma and very small remnant lens (Figure 3(a)).  The corneal protrusion in the right eye was thought to be a corneal staphyloma (Figure 3(b)).
The central cornea with HE staining (a). The corneal epithelium was thickened and keratinized. Bowman's membrane was absent. The stroma was consisted of irregular collagen, fibroblast-like cells, pigmented cells, blood vessels, and presumed inflammatory cells. The corneal endothelium and Descemet's membrane were absent. Immunohistochemistry for Collagen types I (b) and (c). The protruding cornea (b). The peripheral cornea (c). Collagen type I was positive strongly in the peripheral cornea (c) and weakly in protruding cornea (b). Bar: 50 mum.
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Bowman's membrane was absent (Figure 4(a)). The stroma was consisted of irregular collagen, fibroblast-like cells, pigmented cells, blood vessels, and presumed inflammatory cells (Figures 4(a) and 5(a)). Cornea lacked entire corneal endothelium (Figure 4(a)). Descemet's membrane was absent in the region of the protruding cornea (Figure 4(a)). Collagen type I was positive strongly in the peripheral cornea (Figure 4(c)) and weakly in protruding cornea (Figure 4(b)).
The corneal stroma with HE staining (a). The stroma was consisted of irregular collagen, fibroblast-like cells, pigmented cells, and presumed inflammatory cells. Immunohistochemistry for alphaSMA (b). The fibroblast-like cells labeled by antibodies against alphaSMA were scattered in corneal stroma. Posterior surface of the cornea with HE staining (c) and (d). Arrow: the end of Descemet's membrane. Bar: 10 mum.
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The end of Descemet's membrane was recognized (Figures 5(c) and 5(d)). The anterior chamber space was not formed and the iris stroma adhered to the posterior surface of the peripheral cornea (Figures 5(c) and 5(d)). The fibroblast-like cells labeled by antibodies against alphaSMA were scattered in corneal stroma (Figure 5(b)).
The remnant lens with HE staining (a). The contents of the crystalline lens were absorbed and fibrous tissue was present between anterior and posterior capsule. Immunohistochemistry for Collagen types I (b) and alphaSMA (c). Bar: 10 mum.
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The contents of the crystalline lens, that is, nucleus and lenticular fibers, were absorbed and fibrous tissue was present between anterior and posterior capsule (Figure 6(a)). The fibrous tissue in lens was labeled by antibodies against collagen type I and alphaSMA (Figures 6(b) and 6(c)).