Abstract
The crystalline lens is the second-most powerful refractive structure of the eye after the cornea. Cataract, opacity of the crystalline lens, is the most common cause of treatable vision loss. Cataract may develop within the anterior subcapsular, posterior subcapsular, cortical, and nuclear locations within the lens. Aberrant development of the lens can result in congenital lens opacities. Toxic exposure, metabolic disease, inflammatory conditions, and hereditary factors contribute to cataractogenesis. Defects in the lens zonular apparatus can result in lens subluxation or dislocation. Cataract surgery is the only therapeutic option to improve visually significant lens opacity.
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Duncan G, Wormstone IM, Davies PD. The aging human lens: structure, growth, and physiological behaviour. Br J Ophthalmol. 2006;81:818–23.
Strenk SA, Strenk LM, Koretz JF. The mechanism of presbyopia. Prog Retin Eye Res. 2005;24:379–93.
Hogan M, Alvarado JA, Weddell JE. Histology of the human eye. Philadelphia: WB Saunders; 1971.
Ling CA, Weiter JJ, Buzney SM, Lashkari K. Competing theories of cataractogenesis after pars plana vitrectomy and the nutrient theory of cataractogenesis: a function of altered aqueous fluid dynamics. Int Ophthalmol Clin. 2005;45:173–98.
Yurchenco P, Schittny J. Molecular architecture of basement membranes. FASEB J. 1990;4:1577.
Sakai L, Keene D, Engvall E. Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibris. J Cell Biol. 1986;103:2499.
Streeten B. Anatomy of the zonular apparatus. In: Tasman W, Jaeger E, editors. Duane’s foundation of clinical ophthalmology. Philadelphia: JB Lippincott; 1992. p. 1–27.
Maisel H, Harding CV, Alcala JR. The morphology of the lens. In: Bloemendal H, editor. Molecular and cellular biology of the eye lens. New York: Wiley; 1981.
Rafferty M, Goosen W. Cytoplasmic filaments in the crystalline lens of various species: functional correlations. Exp Eye Res. 1978;26:177.
Kuszak J, Ennesser CA, Umlas J, et al. The ultrastructure of fiber cells in primate lenses: a model for studying membrane senescence. J Ultrastruct Mol Struct Res. 1988;100:60.
de Jong W. Evolution of lens and crystallins. In: Boenerdal H, editor. Molecular and cellular biology of the eye lens. New York: Wiley; 1981. p. 221–78.
West S, Duncan DD, Munoz B, et al. Sunlight exposure and risk of lens opacities in a population-based study: the Salisbury eye evaluation project. JAMA. 1998;280:714–8.
Taylor H, West SK, Rosenthal FS, et al. Effect of ultraviolet light on cataract formation. N Engl J Med. 1988;319:1429–33.
Schein O, West S, Munroz B, et al. Cortical lenticular opacification: distribution and location in a longitudinal study. Invest Ophthalmol Vis Sci. 1994;35:363.
Graziosi P, Rosmini F, Bonacini M, et al. Location and severity of cortical cataracts in different regions of the lens in age-related cataract. Invest Ophthalmol Vis Sci. 1996; 37:1698–1703.
Bron A, Brown N. Lens structure and forms of cataract. In: Duncan G, editor. The lens transparency and cataract: Eurage, Rijswijk (South Holland, Netherlands); 1985. p. 3–11.
Vrensen G, Willekens B. Biomicroscopy and scanning electron microscopy of early opacities in the aging human lens. Invest Ophthalmol Vis Sci. 1990;31:1582.
Brown N, Harris ML, Shun-Shin GA, et al. Is cortical spoke cataract due to lens fibre breaks? The relationship between fibre folds, fibre breaks, waterclefts and spoke cataract. Eye. 1993;7:672.
Zimmerman L, Johnson F. Calcium oxalate crystals within ocular tissues. Arch Ophthalmol. 1958;60:372.
Johnson F, Karuna P. Histochemical identification of calcium oxalate. Arch Pathol. 1962;74:347.
Anonymous. Risk factors associated with age-related nuclear and cortical cataract: a case-control study in the Age-Related Eye Disease Study-AREDS report no. 5. Ophthalmology. 2001;108:1400–8.
Wong Y, Klein BE, Klein R, et al. Refractive errors and incident cataracts: the beaver dam eye study. Invest Ophthalmol Vis Sci. 2001;42:1449–54.
Harocopos GJ, Shui Y, McKinnon M, et al. Importance of vitreous liquefaction in age-related cataract. Invest Ophthalmol Vis Sci. 2004;45:77–85.
Klein B, Klein R, Lee KE, Grady LM. Statin use and incident nuclear cataract. JAMA. 2006;295:2752–8.
Lerman S. Chemical and physical properties of the normal and aging lens: spectroscopic (UV, fluorescence phosphorescence, and NMR) analysis. Am J Optom Physiol Optic. 1987;64:11.
Andley U, Clark P. Photoreactions of human lens monomeric crystallins. Biochem Biophys Acta. 1989;997:284.
Al-Ghoul K, Costello M. Fiber cell morphology and cytoplasmic texture in cataractous and normal human lens nuclei. Curr Eye Res. 1996;15:1996.
Fan X, Monner VM, Whitson J. Lens glutathione homeostasis: discrepancies and gaps in knowledge standing in the way of novel therapeutic approaches. Exp Eye Res. 2017;156:103–11.
Clark J. Development and maintenance of cell transparency. In: Albert D, Jakobiec F, editors. Principles and practice of ophthalmology: basic sciences. Philadelphia: WB Saunders; 1994.
Moreau KL, King JA. Protein misfolding and aggregation in cataract disease and prospects for prevention. Trends Mol Med. 2012;18:273–82.
Boyle D, Takemoto L. EM immunolocalization of alpha crystallins: association with the plasma membrane from normal and cataractous human lenses. Curr Eye Res. 1996;15:577.
Hightower K. The role of the lens epithelium in development of UV cataract. Curr Eye Res. 1995;14:71.
Li W-C, Kuzak JR, Dunn K, et al. Lens epithelial cell apoptosis appears to be a common cellular basis for non-congenital cataract development in humans and animals. J Cataract Refract Surg. 1995;130:169.
Kerr F, Wyllie A, Currie A. Apoptosis: a basic biological phenomenon with wide-ranging implications in tissue kinetics. Br J Cancer. 1972;26:239.
Schwartz L. OsborneB: programmed cell death, apoptosis and killer genes. Immunol Today. 1993;14:528.
Golstein P. Controlling cell death. Science. 1997;275:1081.
Worgul B, Rothstein H. Radiation cataracts and mitosis. Ophthalmic Res. 1975;7:21.
Streeten B, Eshaghian J. Human posterior subcapsular cataract: a gross and flat preparation study. Arch Ophthalmol. 1978;96:1653.
Worgul B, Merriam GR, Szechter A. Lens epithelium and radiation cataract. I. Preliminary studies. Arch Ophthalmol. 1976;94:996.
Greiner J, Chylack L. Posterior subcapsular cataracts: histopathologic study of steroid-associated cataracts. Arch Ophthalmol. 1979;97:135.
Eshaghian J, Streeten B. Human posterior subcapsular cataract: an ultrastructural study of the posteriorly migrating cells. Arch Ophthalmol. 1980;98:134.
Wedl C. Atlas der pathologischen Histologie des Auges. Liepzig: George Wigand Verlag; 1860.
Schlotzer-Schrehardt U, Naumann GO. Ocular and systemic pseudoexfoliation syndrome. Am J Ophthalmol. 2006;141:921–37.
Schlotzer-Schrehardt U. Molecular pathology of pseudoexfoliation syndrome/glaucoma – new insights from LOXL1 gene associations. Exp Eye Res. 2008;88:776–85.
Thorleifsson G, et al. Common sequence variants in the LOXL1 gene confer susceptibility to exfoliation glaucoma. Science. 2007;317:1397–400.
Schlotzer-Schrehardt U. Pseudoexfoliation syndrome: the puzzle continues. J Ophthalmic Vis Res. 2012;7:187–9.
Streeten B, Dark A. Pseudoexfoliation syndrome. In: Garner A, Klintworth G, editors. Pathobiology of ocular disease, a dynamic approach. New York: Marcel Dekker; 1994. p. 591–629.
Forsius H. Exfoliation syndrome in various ethnic populations. Acta Ophthalmol. 1988;184(Suppl):71.
Dark A, Streeten B. Precapsular film on the aging human lens: precursor of pseudoexfoliation? Br J Ophthalmol. 1990;74:717.
Tetsumoto K, Schlotzer-Schrehardt U, Kuchle M, Dorfler S. Precapsular layer of the anterior lens capsule in early pseudoexfoliation syndrome. Graefes Arch Clin Exp Ophthalmol. 1992;230:252.
Mizuno K, Murois S. Cycloscopy of pseudoexfoliation. Am J Ophthalmol. 1979;87:513.
Betelson T, Drablos P, Flood P. The so-called senile exfoliation (pseudoexfoliation) of the lens capsule, a product of lens epithelium. Acta Ophthalmol. 1964;42:1096.
Ashton N, Shakib M, Collyer R, Blach R. Electron microscopic study of pseudo-exfoliation of the lens capsule. Lens capsule and zonule fibers. Invest Ophthalmol Vis Sci. 1965;4:141.
Bertelsen T, Seland J. Flat whole-mount preparations of the lens capsule in fibrillopathia epitheliocapsularis. Acta Ophthalmol. 1972;49:938.
Ringvold A. On the occurrence of pseudo-exfoliation material in extra-bulbar tissue from patients with pseudo-exfoliation syndrome of the eye. Acta Ophthalmol. 1973, 51:411.
Eagle R, Font R, Fine B. The basement membrane exfoliation syndrome. Arch Ophthalmol. 1979;97:510.
Streeten B, Dark AJ, Wallace RN, et al. Pseudoexfoliative fibrillopathy in the skin of patients with ocular pseudoexfoliation. Arch Ophthalmol. 1990;110:490.
Streeten B. Aberrant synthesis and aggregation of elastic components in pseudoexfoliative fibrillopathy: a unifying concept. New Trends Ophthalmol. 1993;18:187.
Schlotzer-Schrehardt U, von der Mark K, Sakai LY, Nauman GO. Increased extracellular deposition of fibrillin-containing fibrils in pseudoexfoliation syndrome. Invest Ophthalmol Vis Sci. 1997;38:1997.
Prince A, Streeten BW, Ritch R, et al. Preclinical diagnosis of pseudoexfoliation syndrome. Arch Ophthalmol. 1987;105:1076.
Gifford HJ. A clinical and pathologic study of exfoliation of the lens capsule. Am J Ophthalmol. 1958;46:508.
Bartholomew R. Lenses displacement associated with pseudocapsular exfoliation. Br J Ophthalmol. 1970;54:744.
Dark A. Cataract extraction complicated by capsular glaucoma. Br J Ophthalmol. 1979;63:465.
Vazquez-Ferreiro P, Carrra-Hueso FJ, Poquet Jornet JE, Fikri-Benbrahi N, Diaz-Rey M, Sanjuan-Cervero R. Intraoperative complications of phacoemulsification in pseudoexfoliation: metaanalysis. J Cataract Refract Surg. 2016;41:1666–75.
Fontana L, Coassin M, Ioveieno A, Moramarco A, Cimino L. Cataract surgery in patients with pseudoexfoliation syndrome: current updates. Clin Ophthalmol. 2017;11:1377–83.
Cashwell LJ, Holleman IL, Weaver RG, van Rens GH. Idiopathic true exfoliation of the lens capsule. Ophthalmology. 1989;96:348.
Callahan A, Klein B. Thermal detachment of the anterior lamellae of the anterior lens capsule: a clinical and histologic study. Arch Ophthalmol. 1958;59:73.
Burde R, Bresnick G, Uhrhammer J. True exfoliation of the lens capsule: an electron microscopic study. Arch Ophthalmol. 1969;82:651.
Henkind P, Prose P. Anterior polar-cataract: electron microscopic evidence of collagen. Am J Ophthalmol. 1967;63:768.
Font R, Brownstein S. A light and electron microscopic study of anterior subcapsular cataracts. Am J Ophthalmol. 1974;78:972.
Peng Y, et al. Morphologic and immunohistochemical studies of anterior subcapsular cataract. Invest Ophthalmol Vis Sci. 1990;31(Suppl):445.
Vogt A. Neue Falle von Linsenkapselglaukome (Glaucoma Capsulare). Klin Monatsbl Augenheilkd. 1930;84:1.
Duke-Elder S, MacFaul P. Injuries. Part I. Mechanical injuries. In: Duke-Elder S, editor. System of ophthalmology, vol. 1969. St. Louis: CV Mosby. p. 121–41.
Fraunfelder F, Hanna C. Electric cataracts. I. Sequential changes, unusual and prognostic findings. Arch Ophthalmol. 1972;87:179.
Hanna C, Fraunfelder F. Electric cataracts. II. Ultrastructural lens changes. Arch Ophthalmol. 1972;78:184.
Duke-Elder S. Diseases of the lens and vitreous. Glaucoma and hypotony. In: Duke-Elder S, editor. System of ophthalmology. St. Louis: CV Mosby; 1969.
McDonnell P, Zarbin M, WR G. Posterior capsule opacification in pseudophakic eyes. Ophthalmology. 1983;90:1548.
Apple D, Mamalis N, Loftfield K, et al. Complications of intraocular lenses: a historical and histopathologic review. Surv Ophthalmol. 1984;29:1.
Kappelhof J, Vrensen G, de Jong P, et al. An ultrastructural study of Elschnig’s pearls in the pseudophakic eye. Am J Ophthalmol. 1986;101:58.
Jongebloed W, Kijk F, Kruis J. Soemmering’s ring, an aspect of secondary cataract: a morphological description by SEM. Doc Ophthalmol. 1988;70:165.
Elschnig A. Klinisch-anatomischen Beitrag zur Kenntris des Nachstaares. Klin Monatsbl Augenheilkd. 1911;49:444.
Hiles D, Watson B. Complications of implant surgery in children. Am Intraocular Implant Soc J. 1979;5:24.
Hiles D, Johnson B. The role of the crystalline lens epithelium in post-pseudophakos membrane formation. Am Intraocular Implant Soc J. 1980;6:141.
Saika S. Relationship between posterior capsule opacification and intraocular lens biocompatibility. Prog Retinal Eye Res. 2004;23:283–305.
Cogan D, Donaldson D, Reese A. Clinical and pathological characteristics of radiation cataract. Arch Ophthalmol. 1952;47:55.
Kamari F, Hallaj S, Dorosti F, Alinezhad F, Taleschian-Tabrizi N, Farhadi F, Aslani H. Phototoxicity of environmental radiations in human lens: revisiting the pathogenesis of UV-induced cataract. Graefes Arch Clin Exp Ophthalmol. 2019;257(10):2065–2077. [Epub ahead of print].
McCanna R, et al. Argon-laser-induced cataract as a complication of retinal photocoagulation. Arch Ophthalmol. 1982;100:1071.
Appleton B, McCrossan G. Microwave lens effects in humans. Arch Ophthalmol. 1972;88:259.
Hirsch S, Appleton B, Fine B, Brown P. Effects of repeated microwave irradiations on the albino rabbit eye. Invest Ophthalmol Vis Sci. 1977;16:315.
Vannas A, Hogan M, Golbus W. Lens changes in a galactosemic fetus. Am J Ophthalmol. 1975;80:726.
Cumming R, Mitchell P, Leeder S. Use of inhaled corticosteroids and the risk of cataracts. N Engl J Med. 1997;1997:8.
Williamson J, Paterson R, Fine B, et al. Posterior subcapsular cataracts and glaucoma associated with long-term corticosteroid therapy in patients with rheumatoid arthritis and related conditions. Br J Ophthalmol. 1969;53:361.
Sabates N, Tolentino F, Arroyo M. The complications of perfluoropropane gases used in complex retinal detachments. Retina. 1996;16:7.
Siddall J. The ocular toxic findings with prolonged and high dosage chlorpromazine intake. Arch Ophthalmol. 1965;74:460.
Stern R. Ocular lens toxic findings in patients treated with PUVA: photochemotherapy follow up study. J Invest Dermatol. 1994;103:534.
Tso M, Fine B. Kayser-Fleischer ring and associated cataract in Wilson’s disease. Am J Ophthalmol. 1975;79:479.
Cairns J, Williams H, Walshe J. ‘Sunflower cataract’ in Wilson’s disease. Br Med J. 1969;3:95.
Friedman B, Rotth A. Argyrosis cornea. Am J Ophthalmol. 1930;13:1050.
Roberts W, Wolter J. Ocular chrysiasis. Arch Ophthalmol. 1956;56:48.
Lowenfeld I, Thompson H. Fuchs’ heterochromic cyclitis: a critical review of the literature. I. Clinical characteristics of the syndrome. Surv Ophthalmol. 1973;17:394.
Karkinen-Jaaskelainen M, Saxen L, Vaheri A, et al. Rubella cataract in vitro: sensitive period of the developing human lens. J Exp Med. 1975;141:1238–48.
Meisler D, Mandelbaum S. Propionibacterium-associated endophthalmitis after extracapsular cataract extraction: review of reported cases. Ophthalmology. 1989;96:54–61.
Duke-Elder S, editor. Diseases of the uveal tract. Duke-Elders, editor. System of ophthalmology. St Louis: CV Mosby; 1966.
Flocks M, Littwin C, Zimmerman L. Phacolytic glaucoma: a clinicopathologic study of 138 cases of glaucoma associated with hypermature cataract. Arch Ophthalmol. 1955;54:37.
Rahi A, Misra R, Morgan G. Immunopathology of the lens. II. Humoral and cellular immune responses to autologous lens antigens and their roles in ocular inflammation. Br J Ophthalmol. 1977;61:164.
Easom H, Zimmerman L. Sympathetic ophthalmia and bilateral phacoanaphylaxis: a clinicopathologic correlation of the sympathogenic and sympathizing eyes. Arch Ophthalmol. 1964;72:9.
Marak GJ, Font R, Alepa F. Experimental lens-induced granulomatous endophthalmitis: passive transfer with serum. Ophthalmic Res. 1976;8:117.
Mann I. The development of the human eye. New York: Grune & Stratton; 1964.
Coulombre A. Experimental embryology of the vertebrate eye. Invest Ophthalmol Vis Sci. 1965;4:411.
Font R, Yanoff M, Zimmerman L. Intraocular adipose tissue and persistent hyperplastic primary vitreous. Arch Ophthalmol. 1969;82:43.
Jakobiec FA, Ma L, Wolkow N, Cameron JD, Maltry AC. Osseous and adipocyte differentiations in the intraocular lens and vitreous. Am J Ophthalmol. 2018;186:77–88.
Streeten B, Karpik A, Spitzer K. Posterior keratoconus associated with systemic abnormalities. Arch Ophthalmol. 1983;101:616.
Zimmerman L. Phakjomatous choristoma of the eyelid: a tumor of lenticular anlagen. Am J Ophthalmol. 1971;71:169.
McMahon R, Font R, McLean I. Phakomatous choristoma of the eyelid: electron microscopic confirmation of lenticular derivation. Arch Ophthalmol. 1976;94:1778.
Zimmerman L, Font R. Congenital malformations of the eye. JAMA. 1966;196:684.
Curtin V, Joyce E, Ballin H. Ocular pathology of the oculo-cerebral-renal syndrome of Lowe. Am J Ophthalmol. 1964;64:533.
Tripathy R, Cibis G, Tripathy B. Pathogenesis of cataracts in patients with Lowe’s syndrome. Ophthalmology. 1986;93:1046.
Streeten B, Robinson M, Wallace R, Jones D. Lens capsule abnormalities in Alport’s syndrome. Arch Ophthalmol. 1987;105:1693.
Hinglais N, Grunfeld J-P, Bois E. Characteristic ultrastructural lesion of the glomerular basement membrane in progressive hereditary nephritis (Alport’s syndrome). Lab Investig. 1972;27:473.
Pollard Z. Familial bilateral posterior lenticonus. Arch Ophthalmol. 1983;101:1238.
Graw J. Congenital hereditary cataracts. Int J Dev Biol. 2004;48:1031–44.
Francois J. Congenital cataracts. Assen: Royal Van Gorcum; 1963.
Garner A, Klintworth G. The causes and morphology of cataracts. In: Garner A, Klintworth G, editors. Pathobiology of ocular disease: a dynamic approach, vol. 481. New York: Marcel Dekker; 1994.
Heckenlively J. The frequency of posterior subcapsular cataract in the hereditary retinal degenerations. Am J Ophthalmol. 1982;93:733.
Kaiser-Kupfer M, Kuwabara T, Uga S. Cataract in gyrate atrophy: clinical and morphologic studies. Invest Ophthalmol Vis Sci. 1982;24:432.
Font R, Fine B. Ocular pathology in Fabry’s disease: histochemical and electron microscopic observations. Am J Ophthalmol. 1972;73:418.
Robb R, Kuwabara T. The ocular pathology of type A Niemann-Pick disease: a light and electron microscopic study. Invest Ophthalmol Vis Sci. 1973;12:366.
Dark A, Streeten B. Ultrastructural study of cataract in myotonia dystrophica. Am J Ophthalmol. 1977;84:666.
Magenis R, Maslen C, Smith L, et al. Localization of fibrillin (FBN) gene to chromosome 15 and band 21.1. Genomics. 1991;11:346.
Nirankari M, Chaddah R. Displaced lenses. Am J Ophthalmol. 1967;63:1719.
Dietz H, Cutting G, Pyeritz R, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991;352:337.
Maumenee I. The eye in Marfan syndrome. Trans Am Ophthalmol Soc. 1981;79:684.
Ramsey M, Fine B, Shields J. The Marfan syndrome: a histopathologic study of ocular findings. Am J Ophthalmol. 1973;76:102.
Francois J. Hereditary in ophthalmology. St Louis: CV Mosby; 1961. p. 161–4.
Luebbers J, Goldberg M, Herbst R, et al. Iris transillumination and variable expression in ectopia lentis et pupillae. Am J Ophthalmol. 1977;83:337.
Henkind P, Ashton N. Ocular pathology in homocystinuria. Trans Ophthalmol Soc UK. 1965;85:21.
Ramsey M, Yanoff M, Fine B. The ocular histopathology of homocystinuria. Am J Ophthalmol. 1972;74:377.
Jensen A, Cross H, Paton D. Ocular complications in the Weill-Marchesani syndrome. Am J Ophthalmol. 1974;77:261.
Shih V, Abroms I, Johnson J, et al. Sulfite oxides deficiency: biochemical and clinical investigations of a hereditary metabolic disorder in sulfur metabolism. N Engl J Med. 1977;297:1022.
Smith T, Holland M, Woody M. Ocular manifestations of familial hyperlysinemia. Trans Am Acad Ophthalmol Otolaryngol. 1971;75:355.
Berliner ML. Biomicroscopy of the eye: slit lamp microscopy of the living eye. New York: Paul B. Hoeber; 1949.
Klintworth GK, Landers MBIII. The Eye: structure and function in disease. Baltimore: Williams & Wilkins; 1976.
Barber AN. Embryology of the human eye. CV Mosby: St Louis; 1955.
Naumann GOH, Apple DJ, Domarus D, et al. Pathologie des Auges. Berlin: Springer; 1980. 3.
Teekhasaenee C, Nimmanit S, Wutthiphan S, et al. Posterior polymorphous dystrophy and Alport’s syndrome. Ophthalmology. 1991;98:1207–15.
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Maltry, A.C., Cameron, J.D. (2022). Pathology of the Lens. In: Albert, D.M., Miller, J.W., Azar, D.T., Young, L.H. (eds) Albert and Jakobiec's Principles and Practice of Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-030-42634-7_137
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