Abstract
In primary corneal ectasias, noninflammatory stromal thinning causes the cornea to progressively protrude forwards. Secondary ectasia occurs as a result of thinning due to corneal surgery or inflammatory stromal melt. In both types, in the early stages, the classical clinical signs may be absent, so the diagnosis relies upon topographic findings alone.
In keratoconus the cornea has an exaggerated prolate shape with paracentral corneal steepening, which most commonly occurs inferiorly, with corneal flattening in the opposite hemisphere. This causes corneal irregularity which can only be improved by glasses in the earliest stages. Keratoconus can be classified according to the severity, location and shape of the cone.
In pellucid marginal degeneration, the thinning is further inferiorly, creating a “droopy bow tie” area of corneal steepening, associated with high orders of regular astigmatism. With further progression, the “bows” of the “tie” may meet in the inferior periphery, producing a pattern resembling a more inferior keratoconus. Keratoglobus has a more even thinning affecting the whole cornea, which expands more symmetrically, with relatively little effect on the refraction.
Topographic indices have been developed to detect very early stages of ectasia and quantify progression. These include the asymmetry and regularity indices, inferior-superior value, difference in central power, keratoconus predictability index, enhanced ectasia display and, in certain devices, neural networks. The differential diagnosis includes artefact, contact lens-induced corneal warpage and various types of corneal disease and surgery.
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References
*References Particularly Worth Reading
Rabinowitz YS, Maumenee IH, Lundergan MK. Molecular genetic analysis in autosomal dominant keratoconus. Cornea. 1992;11:302–8.
Rabinowitz YS, Garbus J, McDonnell PJ. Computer-assisted corneal topography in family members of patients with keratoconus. Arch Ophthalmol. 1990;108:365–71.
Gonzalez V, McDonnell PJ. Computer-assisted corneal topography in parents of patients with keratoconus. Arch Ophthalmol. 1992;110:1412–4.
*Rabinowitz YS, Nesburn AB, McDonnell PJ. Videokeratography of the fellow eye in unilateral keratoconus. Ophthalmology. 1993;100:181–186.
Behrens-Baumann W. Detection of keratoconus before refractive surgery [letter]. Ophthalmology. 1994;101:794–5.
Hustead JD. Detection of keratoconus before keratorefractive surgery [letter]. Ophthalmology. 1993;100:975.
*Holland DR, Maeda N, Hannush SB, Riveroll LH, Green MT, Klyce SD, Wilson SE. Unilateral keratoconus. Ophthalmology. 1997;104:1409–1413.
Eran P, Almogit A, David Z, et al. The D144E substitution in the VSX1 gene: a non-pathogenic variant or a disease causing mutation? Ophthalmic Genet. 2008;29:53–9.
Sherwin T, Brookes NH, Loh IP, et al. Cellular incursion into Bowman’s membrane in the peripheral cone of the keratoconic cornea. Exp Eye Res. 2002;74(4):473–82.
Mas-Tur V, MacGregor C, Jayaswal R, et al. A review of keratoconus: diagnosis, pathophysiology and genetics. Surv Ophthalmol. 2017;62(6):770–83.
Tuft SJ, Moodaley LC, Gregory WM, Davison CR, Buckley RJ. Prognostic factors for the progression of keratoconus. Ophthalmology. 1994;101:439–47.
Karolak JA, Kulinska K, Nowak DM, et al. Sequence variants in COL4A1 and COL4A2 genes in Ecuadorian families with keratoconus. Mol Vis. 2011;17:827–43.
Mintz-Hittner HA, Semina EV, Frishman LJ, et al. VSX1 (RINX) mutation with craniofacial anomalies, empty sella, corneal endothelial changes and abnormal retinal and auditory bipolar cells. Ophthalmology. 2004;111:828–36.
Godefrooij DA, Ardine de Wit G, Uiterwaal CS, et al. Age-specific incidence and prevalence of keratoconus: a nationwide registration study. Am J Ophthalmol. 2017;175:169–72.
*Rabinowitz YS, McDonnell PJ. Computer-assisted corneal topography in keratoconus. Refract Corneal Surg. 1989;5:400–408.
*Wilson SE, Lin DTC, Klyce SD. Corneal topography of keratoconus. Cornea. 1991;10:2–8.
de Cunha DA, Woodward EG. Measurement of corneal topography in keratoconus. Ophthal Physiol Opt. 1993;13:377–82.
Corbett MC, O’Brart DPS, Stultiens BAT, Jongsma FHM, Marshall J. Corneal topography using a new moiré image-based system. Eur J Implant Ref Surg. 1995;7:353–70.
Mok JW, Baek SJ, Joo CK, et al. VSX1 gene variants are associated with keratoconus in unrelated Korean patients. J Hum Genet. 2008;53:842–9.
Reinstein DZ, Archer TJ, Gobbe M. Corneal epithelial thickness profile in the diagnosis of keratoconus. J Refract Surg. 2009;25(7):604–10.
Maguire LJ, Bourne MW. Corneal topography of early keratoconus (reply). Am J Ophthalmol. 1989;108:747–8.
Belin MW, Khachikian SS. An introduction to understanding elevation based topography: how elevation data are displayed – a review. Clin Exp Ophthalmol. 2009;37:14–29.
Li X, Bykhovskaya Y, Canedo AL, et al. Genetic association of COL5A1 variants in keratoconus patients suggests a complex connection between corneal thinning and keratoconus. IOVS. 2013;54(4):2696–704.
Sharma A, Tovey JC, Ghosh A, et al. AAV serotype influences gene transfer in corneal stroma in vivo. Exp Eye Res. 2010;3:440–8.
Manuolio TA. Genome-wide association studies and assessment of the risk of disease. N Engl J Med. 2010;2:166–76.
*Madea N, Klyce SD, Smolek MK, Thompson HW. Automated keratoconus screening with corneal topography analysis. Invest Ophthalmol Vis Sci. 1994;35:2749–2757.
Smolek MK, Klyce SD, Maeda N. Keratoconus and contact lens-induced corneal warpage analysis using the keratomorphic diagram. Invest Ophthalmol Vis Sci. 1994;35:4192–203.
Maeda N, Klyce SD, Smolek MK. Comparison of methods for detecting keratoconus using videokeratoscopy. Arch Ophthalmol. 1995;113:870–4.
Goren MB. Comparison of methods for detecting keratoconus using videokeratography [letter]. Arch Ophthalmol. 1996;114:631.
Klyce SD, Smolek MK, Maeda N. Comparison of methods for detecting keratoconus using videokeratography [reply]. Arch Ophthalmol. 1996;114:631–2.
*Smolek MK, Klyce SD. Current keratoconus detection methods compared with a neural network approach. Invest Ophthalmol Vis Sci. 1997;38:2290–2299.
Ambrosio R Jr, Alonson RS, Luz A, et al. Corneal thickness spatial profile and corneal volume distribution: tomographic indices to detect keratoconus. J Cat Refract Surg. 2006;32(11):1851–9.
Abad JC, Rubinfeld RS, Del Valle M, et al. Vertical D: a novel topographic pattern in some keratoconus suspects. Ophthalmology. 2007;114(5):1020–6.
Khachikian SS, Belin MW. Posterior elevation in keratoconus. Ophthalmology. 2009;116(4):816e1.
Arce C. Qualitative and quantitative analysis of aspheric symmetry and asymmetry on corneal surfaces. ASCRS (American Society of Cataract and Refractive Surgeons) Conference: Boston; 2010.
Smadja D, Touboul D, Cohen A, et al. Detection of subclinical keratoconus using an automated decision tree classification. Am J Ophthalmol. 2013;156(2):237–246e1.
Maeda N, Klyce SD, Smolek MK, et al. Automated keratoconus screening with corneal topography analysis. IOVS. 1994;35(6):2749–57.
Dingeldein SA, Klyce SD, Wilson SE. Quantitative descriptors of corneal shape derived from the computer-assisted analysis of photokeratographs. Refract Corneal Surg. 1989;5:372–8.
Wilson SE, Klyce SD. Quantitative descriptors of corneal topography. A clinical study. Arch Ophthalmol. 1991;109:349–53.
Maeda M, Klyce SD, Smolek MK. Neural network classification of corneal topography. Invest Ophthalmol Vis Sci. 1995;36:1327–35.
Amsler M. Le keratocone fruste au javal. Ophthalmologica. 1938;96:77–83.
Amsler M. Keratocone classique et keratocone fruste, arguments unitaire. Ophthalmologica. 1946;111:96–101.
Belin MW, Duncan J, Ambrosio R Jr, et al. A new tomographic method of grading keratoconus: the ABCD Grading system. Int J Kerat Ect Cor Dis. 2015;4(3):85–93.
*Wilson SE, Klyce SD. Screening for corneal topographic abnormalities before refractive surgery. Ophthalmology. 1994;101:147–152.
Nesburn AB, Bahri S, Salz J, Rabinowitz YS, Maguen E, Hofbauer J, Belin M, Macy JI. Keratoconus detected by videokeratography in candidates for photorefractive keratectomy. J Refract Surg. 1995;11:194–201.
Bowman CB, Thompson KP, Stulting RD. Refractive keratotomy in keratoconus suspects. J Refract Surg. 1995;11:202–6.
Doyle SJ, Hynes E, Naroo S, Shah S. PRK in patients with a keratoconic topography picture. The concept of a physiological ‘displaced apex syndrome’. Br J Ophthalmol. 1996;80:25–8.
Colin J, Cochener B, Bobo C, Malet F, Gallinaro C, Le Floch G. Myopic photorefractive keratectomy in eyes with atypical inferior corneal steepening. J Cat Refract Surg. 1996;22:1423–6.
O’Brart DPS, Saunders DC, Corbett MC, Rosen ES. The corneal topography of keratoconus. Eur J Implant Ref Surg. 1995;7(1):20–30.
Eiferman RA, Lane L, Law M, Fields Y. Superior keratoconus [letter]. Refract Corneal Surg. 1993;9:394–5.
*McMahon TT, Robin JB, Scarpulla KM, Putz JL. The spectrum of corneal topography found in keratoconus. CLAO J. 1991;17:198–204.
*Hubbe RE, Foulks GN. The effect of poor fixation on computer-assisted topographic corneal analysis. Ophthalmology. 1994;101:1745–1748.
Silverman CM. Misalignment of videokeratoscope produces pseudo-keratoconus suspect. J Cat Refract Surg. 1994;10:468.
*Karabatsas CH, Cook SD. Topographic analysis in pellucid marginal degeneration and keratoglobus. Eye. 1996;10:451–455.
*Rabinowitz YS, Garbus JJ, Garbus C, McDonnell PJ. Contact lens selection for keratoconus using a computer assisted videokeratoscope. CLAO J. 1991;17:88–93.
Khong AM, Mannis MJ, Plotnik RD, Johnson CA. Computerised topographic analysis of the healing graft after penetrating keratoplasty for keratoconus. Am J Ophthalmol. 1993;115:209–15.
Kremer I, Eagle RC, Rapuano CJ, Laibson PR. Histological evidence of recurrent keratoconus seven years after keratoplasty. Am J Ophthalmol. 1995;199:511–2.
Bechrakis N, Blom ML, Stark WJ, Green WR. Recurrent keratoconus. Cornea. 1994;13:73–7.
Serdarevic ON, Renard GJ, Pouliquen Y. Penetrating keratoplasty for keratoconus: role of videokeratoscopy and trephine sizing. J Cataract Refract Surg. 1996;22:1165–74.
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Corbett, M., Maycock, N., Rosen, E., O’Brart, D. (2019). Corneal Ectasia. In: Corneal Topography. Springer, Cham. https://doi.org/10.1007/978-3-030-10696-6_10
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DOI: https://doi.org/10.1007/978-3-030-10696-6_10
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