Histopathologic and Immunohistochemical Studies of Keratoglobus

Meghpara, Beeran; Nakamura, Hiroshi; Vemuganti, Geeta K.; Murthy, Somasheila I; Sugar, Joel; Yue, Beatrice Y.J.T.; Edward, Deepak P.

doi:10.1001/archophthalmol.2009.184

Cited by 31 publications

(22 citation statements)

References 23 publications

(26 reference statements)

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“…4,14,27 Stromal thinning is most marked at the periphery or mid-periphery, as seen clinically. Poliquin et al 4 …”

Section: Histopathologymentioning

confidence: 95%

Keratoglobus

Wallang

Das²

2013

Eye

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Keratoglobus is a rare noninflammatory corneal thinning disorder characterised by generalised thinning and globular protrusion of the cornea. It was first described as a separate clinical entity by Verrey in 1947. Both congenital and acquired forms have been shown to occur, and may be associated with various other ocular and systemic syndromes including the connective tissue disorders. Similarities have been found with other noninflammatory thinning disorders like keratoconus that has given rise to hypotheses about the aetiopathogenesis. However, the exact genetics and pathogenesis are still unclear. Clinical presentation is characterised by progressive diminution resulting from irregular corneal topography with increased corneal fragility due to extreme thinning. Conservative and surgical management for visual rehabilitation and improved tectonic stability have been described, but remains challenging. In the absence of a definitive standard procedure for management of this disorder, various surgical procedures have been attempted in order to overcome the difficulties. This article reviews the aetiological factors, differential diagnosis, histopathology, and management options of keratoglobus.

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“…4,14,27 Stromal thinning is most marked at the periphery or mid-periphery, as seen clinically. Poliquin et al 4 …”

Section: Histopathologymentioning

confidence: 95%

Keratoglobus

Wallang

Das²

2013

Eye

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“…13 Previously described surgical techniques for keratoglobus have been adapted from those used in management of more common corneal ectasias such as keratoconus and pellucid marginal degeneration. Histopathological and immunohistochemical studies have suggested that the matrix metalloproteases responsible for disrupting the Bowman layer and causing subsequent stromal thinning accord with a similar pathogenetic element in both keratoconus and keratoglobus.…”

Section: Discussionmentioning

confidence: 99%

Use of a Novel Lamellar Keratoplasty With Pleat Technique to Address the Abnormal White-to-White Diameter in Keratoglobus

Lockington

Ramaesh

2015

Cornea

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We have demonstrated a modified anterior lamellar keratoplasty with pleat technique to achieve satisfactory visual rehabilitation in extreme keratoglobus. This pleat technique addresses the fundamental structural problem of a vertically displaced limbus in keratoglobus. We highlight the optical illusion of the white-to-white diameter in keratoglobus. We also recommend the baking analogy of muffin to cupcake as a communication aid when explaining the mechanics of surgery to the patient.

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confidence: 99%

“…5 Biochemical analyses also reveal that a decreased expression of ␣1 proteinase inhibitor and upregulation of transcription factor Sp1 are common to both. 5 Disorders of retinal development (as found in 4 of 10 EDICT patients) can also co-segregate with corneal ectasia; CRB1 mutations causing Leber congenital amaurosis (LCA) have been associated with both keratoconus and keratoglobus. 6 We wonder if the reported corneal phenotype in EDICT syndrome should fall within the keratoconus/-globus spectrum and, as such, should be considered a candidate gene for these as well as other "cornea plus" syndromes?…”

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confidence: 99%

“…Histologic examinations of both keratoconus and keratoglobus corneas have revealed disruption of Bowman's layer and Descemet's membrane, two findings that have not been identified in EDICT syndrome. 2,5,6 The histopathology of EDICT also includes several features that are not present in keratoconus or keratoglobus, such as polymorphic vacuoles within keratocytes and within and between collagen lamellae in the stroma, along with intracellular and extracellular lipid deposition in the stroma and in Bowman's layer. In addition, we observed prominent nodular protrusions of Descemet's membrane, resembling Fuchs' corneal dystrophy, and small-diameter cytokeratin-positive filaments in the endothelium with overlapping of endothelial cells, characteristic of posterior polymorphous corneal dystrophy.…”

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Corneal Thinning Phenotypes—An Alternative Perspective

Giasin

Khan

2012

Invest. Ophthalmol. Vis. Sci.

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We were very interested to read of a second mutation in the seed region of miR-184 resulting in EDICT syndrome. 1 We were unsure after reading the paper, however, of how the corneal findings should be classified and of the statement that the family "does not demonstrate a keratoconus phenotype." Keratoconus, the commonest disorder of corneal thinning and steepening, has been linked with VSX1 and SOD1 in single studies and up to 14 different genetic loci.2 It has a variable phenotype, and while few would dispute the diagnosis in classic cases with central corneal thinning, identifying variants such as forme fruste disease (where a cone may not be present) may be more challenging. A less common disorder of corneal thinning is keratoglobus, in which the thinning is global and often most pronounced in the periphery. Iris hypoplasia with keratoglobus has been described. 3 Similarly, the occurrence of keratoglobus with a corneal endothelial disease, posterior polymorphous corneal dystrophy, has also been recognized.

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Histopathologic and Immunohistochemical Studies of Keratoglobus

Cited by 31 publications

References 23 publications

Keratoglobus

Keratoglobus

Use of a Novel Lamellar Keratoplasty With Pleat Technique to Address the Abnormal White-to-White Diameter in Keratoglobus

Corneal Thinning Phenotypes—An Alternative Perspective

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