The Sclera pp 277-297 | Cite as

Noninflammatory Diseases of the Sclera

  • Maite Sainz de la Maza
  • Joseph Tauber
  • C. Stephen Foster


Like scleritis and episcleritis, noninflammatory diseases of the sclera cannot be properly understood as isolated entities but must be seen in relation to the larger picture of a patient´s general health. Noninflammatory affections of the sclera may be manifestations of ocular diseases, such as the connective tissue abnormalities, degenerations, or tumors. They also may be signs of systemic diseases, such as metabolic disorders, connective tissue abnormalities, or hematologic disorders. In many cases, the characteristics of the scleral abnormality (deposit, thinning, thickening, or mass) are sufficiently distinctive that the ocular or systemic diagnosis is first suspected by the ocular presentation. Ophthalmologists must be competent enough to recognize this interconnection and therefore, promptly diagnose and, if possible, treat the underlying ocular or systemic disease.


Osteogenesis Imperfecta Porphyria Cutanea Tarda Homogentisic Acid Angioid Streak Pseudoxanthoma Elasticum 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Altiparmak UE, Oflu Y, Kocaoglu FA, Katircioglu YA, Duman S. Ocular complications in 2 cases with porphyria. Cornea. 2008;27(9):1093–6.PubMedGoogle Scholar
  2. 2.
    Sevel D, Burger D. Ocular involvement in cutaneous porphyria. A clinical and histological report. Arch Ophthalmol. 1971;85:581.Google Scholar
  3. 3.
    Takamura N, Kurihara K, Yamashita S, Kondo M. Need for measurement of porphyrins in teardrops in patients with congenital erythropoietic porphyria. Br J Ophthalmol. 2002;86(10):1188.PubMedGoogle Scholar
  4. 4.
    Venkatesh P, Garg SP, Kumaran E, Tewari HK. Congenital porphyria with necrotizing scleritis in a 9-year-old child. Clin Experiment Ophthalmol. 2000;28(4):314–8.PubMedGoogle Scholar
  5. 5.
    Salmon JF, Strauss PC, Todd G, Murray AD. Acute scleritis in porphyria cutanea tarda. Am J Ophthalmol. 1990;109(4):400–6.PubMedGoogle Scholar
  6. 6.
    Freedman J. Scleral involvement in porphyria. Am J Ophthalmol. 1978;85(3):427.PubMedGoogle Scholar
  7. 7.
    Bandyopadhyay R, Bhaduri G, Banerjee A, Dasgupta A, Bandyopadhyay M, Purkait S, Singh M. Bilateral scleromalacia in a case of congenital erythropoietic porphyria. J Indian Med Assoc. 2006;104(7):406–7.PubMedGoogle Scholar
  8. 8.
    Kurihara K, Takamura N, Imaizumi S, Yamashita S, Kondo M. Ocular involvement caused by the accumulation of porphyrins in a patient with congenital erythropoietic porphyria. Br J Ophthalmol. 2001;85(10):1265–6.PubMedGoogle Scholar
  9. 9.
    Tanigawa K, Takamura N, Nakata K, Nagataki S, Yamashita S. Ocular involvement in congenital erythropoetic porphyria. Ophthalmologica. 1996;210(3):183–5.PubMedGoogle Scholar
  10. 10.
    Chumbley LC. Scleral involvement in symptomatic porphyria. Am J Ophthalmol. 1977;84:729.PubMedGoogle Scholar
  11. 11.
    Aguarde JP, Mascaro JM, Galy-Mascaro C, et al. Some little known cutaneous and ocular manifestations of porphyria. Ann Dermatol Syphiligr. 1969;96:265.Google Scholar
  12. 12.
    Goldman H, Scriver CR, Aaron K, et al. Adolescent cystinosis: comparisons with infantile and adult forms. Pediatrics. 1971;47:979.PubMedGoogle Scholar
  13. 13.
    Gahl WA, Thoene JG, Schneider JA. Cystinosis. N Engl J Med. 2002;347(2):111–21.PubMedGoogle Scholar
  14. 14.
    Kleta R, Kaskel F, Dohil R, Goodyer P, Guay-Woodford LM, Harms E, Ingelfinger JR, Koch VH, Langman CB, Leonard MB, Mannon RB, Sarwal M, Schneider JA, Skovby F, Sonies BC, Thoene JG, Trauner DA, Gahl WA. First NIH/office of rare diseases conference on cystinosis: past, present, and future. NIH office of rare diseases. Pediatr Nephrol. 2005;20(4):452–4.PubMedGoogle Scholar
  15. 15.
    Tsilou E, Zhou M, Gahl W, Sieving PC, Chan CC. Ophthalmic manifestations and histopathology of infantile nephropathic cystinosis: report of a case and review of the literature. Surv Ophthalmol. 2007;52(1):97–105.PubMedGoogle Scholar
  16. 16.
    Lietman PS, Frazier PD, Wong VG, et al. Adult cystinosis: a benign disorder. Am J Med. 1966;40:511.PubMedGoogle Scholar
  17. 17.
    Zimmerman TJ, Hood I, Gasset AR. “Adolescent” cystinosis: a case presentation and review of the recent literature. Arch Ophthalmol. 1974;92:265.PubMedGoogle Scholar
  18. 18.
    Kenyon KR, Sensenbrenner JA. Electron microscopy of cornea and conjunctiva in chilhood cystinosis. Am J Ophthalmol. 1974;78:68.PubMedGoogle Scholar
  19. 19.
    Sanderson PO, Kuwabara T, Stark WJ, et al. Cystinosis: a clinical, histopathologic, and ultrastructural study. Arch Ophthalmol. 1974;91:270.PubMedGoogle Scholar
  20. 20.
    Wong VG, Lietman PE, Seegmiller JE. Alterations of pigment epithelium in cystinosis. Arch Ophthalmol. 1967;77:361.PubMedGoogle Scholar
  21. 21.
    Wong VG. Ocular manifestations in cystinosis. Birth Defects Orig Artic Ser. 1976;12(3):181.PubMedGoogle Scholar
  22. 22.
    Wong VG, Schulman JD, Seegmiller JE. Conjunctival biopsy for the biochemical diagnosis of cystinosis. Am J Ophthalmol. 1970;70:278.PubMedGoogle Scholar
  23. 23.
    Gahl WA, Reed GF, Thoene JG, et al. Cysteamine therapy for children with nephropathic cystinosis. N Engl J Med. 1987;316:971.PubMedGoogle Scholar
  24. 24.
    Kaiser-Kupfer MI, Fujikawa L, Kuwabara T, et al. Removal of corneal crystals by topical cysteamine in nephropathic cystinosis. N Engl J Med. 1987;316:775.PubMedGoogle Scholar
  25. 25.
    Khan AO, Latimer B. Successful use of topical cysteamine formulated from the oral preparation in a child with keratopathy secondary to cystinosis. Am J Ophthalmol. 2004;138(4):674–5.PubMedGoogle Scholar
  26. 26.
    Van Offel JF, De Clerck LS, Francx LM, Stevens WJ. The clinical manifestations of ochronosis: a review. Acta Clin Belg. 1995;50(6):358–62.PubMedGoogle Scholar
  27. 27.
    Keller JM, Macaulay W, Nercessian OA, Jaffe IA. New developments in ochronosis: review of the literature. Rheumatol Int. 2005;25(2):81–5.PubMedGoogle Scholar
  28. 28.
    Smith JW. Ochronosis of the sclera and cornea complicating alkaptonuria: review of the literature and report of four cases. JAMA. 1942;120:1282.Google Scholar
  29. 29.
    Cheskes J, Buettner H. Ocular manifestations of alkaptonuric ochronosis. Arch Ophthalmol. 2000;118(5):724–5.PubMedGoogle Scholar
  30. 30.
    Wirtschafter JD. The eye in alkaptonuria. Birth Defects Orig Artic Ser. 1976;12(3):279.PubMedGoogle Scholar
  31. 31.
    Kampik A, Sani JN, Green WR. Ocular ochronosis. Clinicopathological, histochemical, and ultrastructural studies. Arch Ophthalmol. 1980;98(8):1441–7.PubMedGoogle Scholar
  32. 32.
    Varga J, Wohlgethan JR. The clinical and biochemical spectrum of hereditary amyloidosis. Semin Arthritis Rheum. 1988;18(1):14–28.PubMedGoogle Scholar
  33. 33.
    Paton D, Duke JR. Primary familial amyloidosis. Ocular manifestations with histopathological observations. Am J Ophthalmol. 1966;61:736.PubMedGoogle Scholar
  34. 34.
    Brownstein MH, Elliot R, Helwig EB. Ophthalmologic aspects of amyloidosis. Am J Ophthalmol. 1970;69:423.PubMedGoogle Scholar
  35. 35.
    Gorevic PD, Rodriguez MM. Ocular amyloidosis. Am J Ophthalmol. 1994;117(4):529–32.PubMedGoogle Scholar
  36. 36.
    Schwartz MF, Green WR, Michels RG, et al. An unusual case of ocular involvement in primary systemic nonfamilial amyloidosis. Ophthalmology. 1982;89:394.PubMedGoogle Scholar
  37. 37.
    Wright JR, Calkins E, Breen WJ, et al. Relationship of amyloid to aging. Medicine. 1969;48:39.PubMedGoogle Scholar
  38. 38.
    Liew SC, McCluskey PJ, Parker G, Taylor RF. Bilateral uveal effusion associated with scleral thickening due to amyloidosis. Arch Ophthalmol. 2000;118(9):1293–5.PubMedGoogle Scholar
  39. 39.
    Coats G. Hyperplasia, with colloid and amyloid degeneration, of the episcleral and circumdural fibrous tissue. Trans Ophthalmol Soc UK. 1915;35:257.Google Scholar
  40. 40.
    Wayman LL, Margo CE. Xanthoma disseminatum with bilateral epibulbar involvement. Am J Ophthalmol. 2005;139(3):557–9.PubMedGoogle Scholar
  41. 41.
    Broekhuyse RM, Kuhlmann ED. Lipids in tissues of the eye. VI. Sphingomyelins and cholesterol esters in human sclera. Exp Eye Res. 1972;14:111.PubMedGoogle Scholar
  42. 42.
    Broekhuyse RM. The lipid composition of aging sclera and cornea. Ophthalmologica. 1975;171:82.PubMedGoogle Scholar
  43. 43.
    Kenyon KR, Topping TM, Green WR, et al. Ocular pathology of the Maroteaux-Lamy syndrome (systemic mucopolysaccaridosis Type VI); Histologic and ultrastructural report of two cases. Am J Ophthalmol. 1972;73:718.PubMedGoogle Scholar
  44. 44.
    Conn H, Green WR, de la Cruz ZC, et al. Scleropachynsis maculopathy: a clinicopathologic case report. Arch Ophthalmol. 1977;95:497.Google Scholar
  45. 45.
    Winterbotham CTC, Torczynski E, Horwitz AL, et al. Unusual mucopolysaccharide disorder with corneal and scleral involvement. Am J Ophthalmol. 1990;109:544.PubMedGoogle Scholar
  46. 46.
    Iwamoto M, Nawa Y, Maumenee IH, Young-Ramsaran J, Matalon R, Green WR. Ocular histopathology and ultrastructure of Morquio syndrome (systemic mucopolysaccharidosis IV A). Graefes Arch Clin Exp Ophthalmol. 1990;228(4):342–9.PubMedGoogle Scholar
  47. 47.
    Spellacy E, Bankes JL, Crow J, Dourmashkin R, Shah D, Watts RW. Glaucoma in a case of Hurler disease. Br J Ophthalmol. 1980;64(10):773–8.PubMedGoogle Scholar
  48. 48.
    Forrester JV, Lee WR, Kerr PR, Dua HS. The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4(Pt 2):354–65.Google Scholar
  49. 49.
    Walsh FB, Murray RG. Ocular manifestations of disturbances in calcium metabolism. The Ninth Sanford R, Gifford Lecture. Am J Ophthalmol. 1953;36:1657.PubMedGoogle Scholar
  50. 50.
    Cogan DG, Albright F, Bartter FC. Hypercalcemia and band keratopathy. Report of nineteen cases. Arch Ophthalmol. 1940;40:624.Google Scholar
  51. 51.
    Mornet E. Hypophosphatasia. Best Pract Res Clin Rheumatol. 2008;22(1):113–27.PubMedGoogle Scholar
  52. 52.
    Porter R, Crombie AL. Corneal calcification as a presenting and diagnostic sign in hyperparathyroidism. Br J Ophthalmol. 1973;57:665.PubMedGoogle Scholar
  53. 53.
    Shields JA, Shields CL. CME review: sclerochoroidal calcification: the 2001 Harold Gifford Lecture. Retina. 2002;22(3):251–6.PubMedGoogle Scholar
  54. 54.
    Jensen OA. Ocular calcifications in primary hyperparathyroidism. Acta Ophthalmol (Copenh). 1975;53:173.Google Scholar
  55. 55.
    Spencer WH. Sclera. In: Spencer WH, editor. Ophthalmic Pathology. 3rd ed. Philadelphia: WB Saunders Company; 1985. p. 389–422.Google Scholar
  56. 56.
    Gartner S, Rubner K. Calcified scleral nodules in hypervitaminosis D. Am J Ophthalmol. 1955;39:658.PubMedGoogle Scholar
  57. 57.
    Heat P. Calcinosis oculi. Trans Am Ophthalmol Soc. 1961;59:141.Google Scholar
  58. 58.
    Brenner RL, Smith JL, Cleveland WW, et al. Eye signs of hypophosphatasia. Arch Ophthalmol. 1969;81:614.PubMedGoogle Scholar
  59. 59.
    Fraser D. Hypophosphatasia. Am J Med. 1957;22:730.PubMedGoogle Scholar
  60. 60.
    Bethune JE, Dent CE. Hypophosphatasia in the adult. Am J Med. 1960;28:615.PubMedGoogle Scholar
  61. 61.
    Lessel S, Norton EWD. Band keratopathy and conjunctival calcification in hypophosphatasia. Arch Ophthalmol. 1964;71:497.Google Scholar
  62. 62.
    Roxburgh STD. Atypical retinitis pigmentosa with hypophosphatasia. Trans Ophthalmol Soc UK. 1983;103:513.PubMedGoogle Scholar
  63. 63.
    Pagenstecher A. Beitrage zur pathologischen Anatomie des Auges. Albrecht von Graefes Archiv für Ophthalmologie. 1860;7:92.Google Scholar
  64. 64.
    Watson PG, Hazleman BL. The sclera and systemic disorders. Philadelphia: W.B. Saunders Company; 1976. p. 347–81.Google Scholar
  65. 65.
    Duke-Elder S, Leigh AG. Diseases of the outer Eye. Cornea and sclera. In: Duke-Elder S, editor. System of ophthalmology, vol 8, pt 2. CV Mosby Co: St Louis; 1965.Google Scholar
  66. 66.
    Franceschetti A, Bischler V. La sclérite nodulaire nécrosante et ses rapports avec la scléromalacie. Ann d’Oc. 1950;183:737.Google Scholar
  67. 67.
    Kiss J. Fall von seniler Sklerverdunnung. Klin Monatsbl f Augenh. 1934;92:121.Google Scholar
  68. 68.
    Roper KL. Senile hyaline scleral plaques. Arch Ophthalmol. 1945;34:283.Google Scholar
  69. 69.
    Graves B. Bilateral mesial superficial deficiency of the sclera: scleral plaques. Br J Ophthalmol. 1941;25:35.PubMedGoogle Scholar
  70. 70.
    Katz D. A localized area of calcareous degeneration in the sclera. Arch Ophthalmol. 1929;2:30.Google Scholar
  71. 71.
    Culler AM. The pathology of scleral plaques; report of 5 cases of degenerative plaques in the sclera mesially, one studied histologically. Br J Ophthalmol. 1939;23:44.PubMedGoogle Scholar
  72. 72.
    Drescher EP, Henderson JW. Senile hyaline scleral plaques; report of 3 cases. Proc Staff Meet Mayo Clinic. 1949;24:334.Google Scholar
  73. 73.
    van der Hoeve J. Scleromalacia perforans. Arch Ophthalmol. 1934;11:111.Google Scholar
  74. 74.
    François J. Scleromalacia perforans, arthritis deformans and pemphigus. Trans Ophthalmol Soc UK. 1951;71:61.Google Scholar
  75. 75.
    Sorensen TB. Paralimbal scleromalacia. Acta Ophthalmol. 1975;53:901.Google Scholar
  76. 76.
    Tolentino FI, Brockhurst RJ. Unilateral scleral icterus due to choroidal hemorrhage. Arch Ophthalmol. 1963;70:358.PubMedGoogle Scholar
  77. 77.
    Beghetti M, Mermillod B, Halperin DS. Blue sclerae: a sign of iron deficiency anemia in children? Pediatrics. 1993;91(6):1195–6.PubMedGoogle Scholar
  78. 78.
    Kalra L, Treloar A, Price R, Jones BJ. Blue sclerae and iron deficiency in general practice. Lancet. 1987;1(8528):335.PubMedGoogle Scholar
  79. 79.
    Agnoleto A. Blue sclerotics in iron deficiency. Lancet. 1971;ii:1160.Google Scholar
  80. 80.
    Walter FB, Israel MS. General pathology. 5th ed. Edinburgh: Churchill Livingstone; 1979. p. 63–5.Google Scholar
  81. 81.
    Bennet RM. Blue sclerotics in iron deficiency. Lancet. 1971;ii:1100.Google Scholar
  82. 82.
    Pope FM. Blue sclerotics in iron deficiency. Lancet. 1971;ii:1160.Google Scholar
  83. 83.
    Wiernik PH. Blue sclerae. Lancet. 1972;ii:1199.Google Scholar
  84. 84.
    Allen RA, Straatsma BR, Apt L, et al. Ocular manifestations of the Marfan syndrome. Trans Am Acad Ophthalmol Otolaryngol. 1967;71:18.PubMedGoogle Scholar
  85. 85.
    Cross HE, Jensen AD. Ocular manifestations in the Marfan syndrome and homocystinuria. Am J Ophthalmol. 1973;75:405.PubMedGoogle Scholar
  86. 86.
    Nahum Y, Spierer A. Ocular features of Marfan syndrome: diagnosis and management. Isr Med Assoc J. 2008;10(3):179–81.PubMedGoogle Scholar
  87. 87.
    Dean JC. Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet. 2007;15(7):724–33.PubMedGoogle Scholar
  88. 88.
    Gerritsen T, Waisman H. Homocystinuria. In: Stanbury J, Wyngaarden J, Fredrickson D, editors. The metabolic basis of inherited disease. 3rd ed. New York: McGraw-Hill; 1972. p. 404.Google Scholar
  89. 89.
    Spurway J. Hereditary tendency to fracture. Br Med J. 1896;2:844.PubMedGoogle Scholar
  90. 90.
    Eddowes A. Dark sclerotics and fragilitas ossium. Br Med J. 1900;2:222.Google Scholar
  91. 91.
    van der Hoeve J, de Kleyn A. Blaue Sclera, Knochenbruchigkeit und Schwerhorigkeit Albrecht Von Graefes. Arch Klin Exp Ophthalmol. 1918;95:81.Google Scholar
  92. 92.
    Martin E, Shapiro JR. Osteogenesis imperfecta: epidemiology and pathophysiology. Curr Osteoporos Rep. 2007;5(3):91–7.PubMedGoogle Scholar
  93. 93.
    Cheung MS, Glorieux FH. Osteogenesis Imperfecta: update on presentation and management. Rev Endocr Metab Disord. 2008;9(2):153–60.PubMedGoogle Scholar
  94. 94.
    McKusick VA. Heritable disorders of connective tissue. 4th ed. CV Mosby Company: St Louis; 1972.Google Scholar
  95. 95.
    Shoenfeld T, Fried A, Ehrenfeld NE. Osteogenesis imperfecta: Review of the literature and presentation of 29 cases. Am J Dis Child. 1975;129:679.PubMedGoogle Scholar
  96. 96.
    Wilcox RA, McDonald FS. Gray-blue sclerae and osteopenia secondary to osteogenesis imperfecta. Mayo Clin Proc. 2007;82(3):265.PubMedGoogle Scholar
  97. 97.
    Evereklioglu C, Madenci E, Bayazit YA, Yilmaz K, Balat A, Bekir NA. Central corneal thickness is lower in osteogenesis imperfecta and negatively correlates with the presence of blue sclera. Ophthalmic Physiol Opt. 2002;22(6):511–5.PubMedGoogle Scholar
  98. 98.
    Kaiser-Kupfer MI, McCain L, Shapiro JR, et al. Low ocular rigidity in patients with osteogenesis imperfecta. Invest Ophthalmol Vis Sci. 1981;20:807.PubMedGoogle Scholar
  99. 99.
    Ruedemann AD. Osteogenesis imperfecta congenita and blue sclerotics. Arch Ophthalmol. 1953;49:6.Google Scholar
  100. 100.
    Leonibus F, Gemolotto G. Rilievi istologici su di un caso di sclera blu. Bolletino di Oculistica. 1954;33:789.Google Scholar
  101. 101.
    Buchanan L. Case of congenital maldevelopment of the cornea and sclerotic. Trans Ophthalmol Soc UK. 1903;23:267.Google Scholar
  102. 102.
    Casanovas J. Blue scleras and fragilitas osseum. Am J Dis Children. 1935;50:1298.Google Scholar
  103. 103.
    Follis Jr RJ. Osteogenesis imperfecta congenita: a connective tissue diathesis. J Ped. 1953;44:713.Google Scholar
  104. 104.
    Haebara H, Yamasaki Y, Kyogoku M. An autopsy case of osteogenesis imperfecta. Acta Path Jap. 1969;19:377.PubMedGoogle Scholar
  105. 105.
    Blumcke S, Niedorf HR, Theil HJ, et al. Histochemical and fine structural studies on the cornea in osteogenesis imperfecta congenita. Virchows Arch Abt B Zellpath. 1972;11:124.Google Scholar
  106. 106.
    Chan CC, Green WR, Zenaida C, et al. Ocular findings in osteogenesis imperfecta congenita. Arch Ophthalmol. 1982;100:1459.Google Scholar
  107. 107.
    Eichholtz W, Mueller D. Electron microscopy findings on the cornea and sclera in osteogenesis imperfecta. Klin Monatsbl für Augenheilk. 1972;161:646.Google Scholar
  108. 108.
    Chan CC, Green WR, de la Cruz ZC, Hillis A. Ocular findings in osteogenesis imperfecta congenita. Arch Ophthalmol. 1982;100(9):1458–63.PubMedGoogle Scholar
  109. 109.
    Callewaert B, Malfait F, Loeys B, De Paepe A. Ehlers-Danlos syndromes and Marfan syndrome. Best Pract Res Clin Rheumatol. 2008;22(1):165–89.PubMedGoogle Scholar
  110. 110.
    Maumenee IH. The eye in the Marfan syndrome. Trans Am Ophthalmol Soc. 1981;79:684–733.PubMedGoogle Scholar
  111. 111.
    Lebwoh MG, Distefano D, Prioleau PG, et al. Pseudoxanthoma elasticum andmitral valve prolapse. N Engl J Med. 1982;307:228.Google Scholar
  112. 112.
    Huang S, Steele H, Kumar G, et al. Ultrastructural changes of elastic fibers in pseudoxanthoma elasticum: a study of histogenesis. Arch Pathol. 1967;83:108.PubMedGoogle Scholar
  113. 113.
    Goodman R, Smith E, Paton D, et al. Pseudoxanthoma elasticum: a clinical and histopathological study. Medicine. 1963;42:297.PubMedGoogle Scholar
  114. 114.
    Paton D. The relation of angioid streaks to systemic disease. Springfield IL: Thomas; 1972. p. 13–31.Google Scholar
  115. 115.
    Lorincz A. Ehlers-Danlos syndrome. In: Demis D, editor. Clinical dermatology. Hagerstown, MD: Harper & Row; 1979.Google Scholar
  116. 116.
    Beighton P. Serious ophthalmological complications in the Ehlers-Danlos syndrome. Br J Ophthalmol. 1970;54:263.PubMedGoogle Scholar
  117. 117.
    Green W, Friedman-Kien A, Banfield W. Angioid streaks in Ehlers-Danlos syndrome. Arch Ophthalmol. 1966;76:197.PubMedGoogle Scholar
  118. 118.
    Cameron JA. Corneal abnormalities in Ehlers-Danlos syndrome type VI. Cornea. 1993;12(1):54.PubMedGoogle Scholar
  119. 119.
    Biglan AW, Brown SI, Johnson BC. Keratoglobus and blue sclera. Am J Ophthalmol. 1977;83:225.PubMedGoogle Scholar
  120. 120.
    Babel J, Houber J. Keratocone et sclerotiques bleues dans one anomalie congenitale de tissue conjuncti. J Genet Hum. 1969;17:241.PubMedGoogle Scholar
  121. 121.
    Gregoratos N, Bartoscocas C, Papas K. Blue sclera with keratoglobus and brittle cornea. Br J Ophthalmol. 1971;55:424.PubMedGoogle Scholar
  122. 122.
    Hymas SW, Dar H, Newman E. Blue sclera keratoglobus. Br J Ophthalmol. 1969;53:53.Google Scholar
  123. 123.
    Stein R, Lazar M, Adam A. Brittle cornea. A familial trait associated with blue sclera. Am J Ophthalmol. 1968;66:67.PubMedGoogle Scholar
  124. 124.
    Greenfield G, Romano A, Stein R, et al. Blue sclera and keratoconus Key features of a distinct heritable disorder of connective tissue. Clin Genet. 1973;4:8.PubMedGoogle Scholar
  125. 125.
    McKusick VA. Multiple forms of the Ehlers-Danlos syndrome. Arch Surg. 1974;109:475.PubMedGoogle Scholar
  126. 126.
    Watson PG, Hazleman BL. The sclera and systemic disorders. Philadelphia: W.B. Saunders Company; 1976. p. 306–46.Google Scholar
  127. 127.
    Macsai MS, Lemley HL, Schwartz T. Management of oculus fragilis in Ehlers-Danlos type VI. Cornea. 2000;19(1):104–7.PubMedGoogle Scholar
  128. 128.
    Pinnell SR, Krane SM, Kenzora JE, et al. Heritable disorder with hydroxylisine-deficient collagen Hydroxylisine-deficient collagen disease. N Engl J Med. 1972;286:1013.PubMedGoogle Scholar
  129. 129.
    Maumenee IH. Hereditary connective tissue diseases involving the eye. Trans Ophthalmol Soc UK. 1974;94:753.PubMedGoogle Scholar
  130. 130.
    Judisch G, Waziri M, Krachmer J. Ocular Ehlers-Danlos syndrome with normal lysyl hydroxylase activity. Arch Ophthalmol. 1976;94:1489.PubMedGoogle Scholar
  131. 131.
    Falls HF, Allen AW. Dominantly inherited keratoconus. Report of a family. J Genet Hum. 1969;17:317.PubMedGoogle Scholar
  132. 132.
    Hammerstein W. Zur Genetik des Keratoconus. Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1974;190:293.PubMedGoogle Scholar
  133. 133.
    Hallermann W, Wilson EJ. Genetishe Betrachtungen uber den Keratokonus. Klin Monatsbl Augenheilkd. 1977;170:906.PubMedGoogle Scholar
  134. 134.
    Redmond KB. The role of heredity in keratoconus. Trans Ophthalmol Soc NZ. 1968;27:52.Google Scholar
  135. 135.
    Woillez M, Razemon PH, Constantinides G. A propos d’un nouveau cas de keratocone chez des juneaux univitellins. Bull Soc Ophthalmol Fr. 1976;76:279.Google Scholar
  136. 136.
    Austin MG, Shaefer RF. Marfan syndrome with unusual blood vessel manifestations; primary medionecrosis, dissection of the right inominate. Arch Pathol Lab Med. 1957;64:205.Google Scholar
  137. 137.
    Storch H. Ein Fall von Arachnodaktylie (Dystrophia mesodermalis congenita), Typus Marfan. Dermatologica. 1952;104:322.Google Scholar
  138. 138.
    Blandau RJ. Morphogenesis and malformations of the skin. Birth Defects Orig Artic Ser. 1981;17:155.Google Scholar
  139. 139.
    Robertson I. Keratoconus and the Ehlers-Danlos syndrome. Med J Aust. 1975;1:571.PubMedGoogle Scholar
  140. 140.
    Shaffer RN, Weiss DI. Congenital and pediatric glaucomas. CV Mosby: St Louis; 1970.Google Scholar
  141. 141.
    Sampaolesi R. Corneal diameter and axial length in congenital glaucoma. Can J Ophthalmol. 1988;23:42.PubMedGoogle Scholar
  142. 142.
    Donaldson DD, Bennett N, Anderson DR, et al. Peripapillary staphyloma. Arch Ophthalmol. 1969;82:704.PubMedGoogle Scholar
  143. 143.
    Wallman J, Turkel J. Extreme myopia produced by modest change in early visual experience. Science. 1978;201:1249.PubMedGoogle Scholar
  144. 144.
    Raviola E, Weisel TN. An animal model of myopia. N Engl J Med. 1985;312:1609.PubMedGoogle Scholar
  145. 145.
    Wiesel TN, Raviola E. Increase in axial lenght of the macaque monkey eye after corneal opacification. Invest Ophthalmol Vis Sci. 1979;18:1232.PubMedGoogle Scholar
  146. 146.
    Judge SJ. Does the eye grow into focus. Nature. 1990;345:477.PubMedGoogle Scholar
  147. 147.
    Wallman J, Adams JI. Developmental aspects of experimental myopia in chicks: susceptibility, recovery, and relation to emmetropization. Vision Res. 1987;27:1139.PubMedGoogle Scholar
  148. 148.
    Curtin BJ, Teng CC. Scleral changes in pathological myopia. Trans Am Acad Ophthalmol. 1958;62:777.Google Scholar
  149. 149.
    Heine L. Beitrage zur Anatomie des myopischen Auges. Archiv für Augenheilkunde. 1899;38:277.Google Scholar
  150. 150.
    Christensen AM, Wallman J. Increased DNA and protein synthesis in scleras of eyes with visual-deprivation myopia. Invest Ophthalmol Vis Sci Supp. 1989;30:402.Google Scholar
  151. 151.
    Rada JA, Thoft RA, Hassell JR. Extracellular matrix changes in the sclera of chickens with experimental myopia. Invest Ophthalmol Vis Sci Supp. 1990;31:253.Google Scholar
  152. 152.
    Wu YR. DNA, collagen, and uronic acid in form deprivation myopia. Invest Ophthalmol Vis Sci. 1990;31:254.Google Scholar
  153. 153.
    Anderson B, Margolis C. Scleromalacia: Clinical and pathologic study of a case with consideration of differential diagnosis, relationship of collagen disease, and effect of ACTH and cortisone therapy. Am J Ophthalmol. 1952;35:917.PubMedGoogle Scholar
  154. 154.
    Arkle JS, Ingram HV. Scleromalacia perforans. Trans Ophthalmol Soc UK. 1935;55:552.Google Scholar
  155. 155.
    Mader TH, Stulting RD, Crosswell HH. Bilateral paralimbal scleromalacia perforans. Am J Ophthalmol. 1990;109:233.PubMedGoogle Scholar
  156. 156.
    Schaffer RN. The management of glaucoma in ­nanophthalmos. Trans Am Ophthalmol Soc. 1975;73:119.Google Scholar
  157. 157.
    Brockhurst RJ. Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol. 1980;98:1987.PubMedGoogle Scholar
  158. 158.
    Gass JDM. Uveal effusion syndrome; a new hypothesis concerning pathogenesis and technique of surgical treatment. Retina. 1983;3:159.PubMedGoogle Scholar
  159. 159.
    Yue BYJT, Duvall J, Goldberg MF. Nanophthalmic sclera. Morphologic and tissue culture studies. Ophthalmology. 1986;93:534.PubMedGoogle Scholar
  160. 160.
    Trelstad RL, Silbermann NN, Brockhurst RJ. Nanophthalmic sclera. Arch Ophthalmol. 1982;100:1935.PubMedGoogle Scholar
  161. 161.
    Stewart DH, Streeten BW, Brockhurst RJ. Abnormal scleral collagen in nanophthalmos. An ultrastructural study. Arch Ophthalmol. 1991;109:1017.PubMedGoogle Scholar
  162. 162.
    Yue BYJT, Kurosawa A, Duvall J, et al. Nanophthalmic sclera: fibronectin studies. Ophthalmology. 1988;95:56.PubMedGoogle Scholar
  163. 163.
    Boniuk M, Zimmerman LE. Episcleral osseous choristoma. Am J Ophthalmol. 1961;53:290.Google Scholar
  164. 164.
    Pittke EC, Marquardt R, Mohr W. Cartilage ­choristoma of the eye. Arch Ophthalmol. 1983;101:1569.PubMedGoogle Scholar
  165. 165.
    Bengisu U, Tahsinoglu M, Toker G. Neurofibromatosis associated with cartilaginous choristoma of the episclera. Ann Ocul. 1973;206:401.Google Scholar
  166. 166.
    Ferry AP, Hein HF. Epibulbar osseous choristoma within an epibulbar dermoid. Am J Ophthalmol. 1970;70:764.PubMedGoogle Scholar
  167. 167.
    Kim RY, Seiff SR, Howes EL, et al. Necrotizing scleritis secondary to conjunctival squamous cell carcinoma in acquired immunodeficiency syndrome. Am J Ophthalmol. 1990;109:231.PubMedGoogle Scholar
  168. 168.
    Stokes JJ. Intraocular extension of epibulbar squamous cell carcinoma of the limbus. Trans Am Acad Ophthalmol Otolaryngol. 1955;59:143.PubMedGoogle Scholar
  169. 169.
    Lindenmuth KA, Sugar A, Kincaid KC, et al. Invasive squamous cell carcinoma of the conjunctiva presenting as necrotizing scleritis with scleral perforation and uveal prolapse. Surv Ophthalmol. 1988;33:50.PubMedGoogle Scholar
  170. 170.
    Overly WL, Jakubek DJ. Multiple squamous cell carcinomas and human immunodeficiency virus infection. Ann Intern Med. 1987;106:334.PubMedGoogle Scholar
  171. 171.
    Konwaler BE, Keasby L, Kaplan L. Subcutaneous pseudosarcomatous fibromatosis (fasciitis). Am J Clin Pathol. 1955;25:241.PubMedGoogle Scholar
  172. 172.
    Font RL, Zimmerman LE. Nodular fasciitis of the eye and adnexa. A report of ten cases. Arch Ophthalmol. 1966;75:475.PubMedGoogle Scholar
  173. 173.
    Tolls RE, Mohr S, Spencer WH. Benign nodular fasciitis originating in Tenon’s capsule. Arch Ophthalmol. 1966;75:482.PubMedGoogle Scholar
  174. 174.
    Ferry AP, Sherman SE. Nodular fasciitis of the conjunctiva apparently originating in the fascia bulbi (Tenon’s capsule). Am J Ophthalmol. 1974;78:514.PubMedGoogle Scholar
  175. 175.
    Hirschberg J. Casuistiche Mittheilungen uber Geschwulste der orbita und des bulbus. Klinische Monatsbläter für Augenheilkunde. 1868;6:153.Google Scholar
  176. 176.
    Betteto G, Amidei B. Fibrous sarcoma of the sclera. Annali di Ottalmologia. 1954;80:495.Google Scholar
  177. 177.
    Ninane J. The eye as a sanctuary in acute lymphoblastic leukemia. Lancet. 1980;1:452.PubMedGoogle Scholar
  178. 178.
    Allen R, Straatsma B. Ocular involvement in leukemia and allied disorders. Arch Ophthalmol. 1961;66:490.PubMedGoogle Scholar
  179. 179.
    Kincaid M, Green W, Kelley J. Acute ocular leukemia. Am J Ophthalmol. 1979;87:698.PubMedGoogle Scholar
  180. 180.
    Murray K, Paolino F, Goldman J, et al. Ocular involvement in leukaemia. Lancet. 1977;1:829.PubMedGoogle Scholar
  181. 181.
    Nitsch M. Neurofibromatose des Auges. Zeitschrift für Augenheilkunde. 1929;69:117.Google Scholar
  182. 182.
    Dabezies OH, Penner R. Neurofibroma and neurilemmoma of the bulbar conjunctiva. Arch Ophthalmol. 1961;66:73.Google Scholar
  183. 183.
    Quintana M, Lee WR. Intrascleral schwannoma. Ophthalmologica. 1976;173:64.PubMedGoogle Scholar
  184. 184.
    Kyrieleis W. Ein Neurinom am Limbus corneae. Graefes Arch Clin Exp Ophthalmol. 1927;119:119.Google Scholar
  185. 185.
    Szabo G, Cseh E. Sklera-Neurinom in der Nähe des Limbus. Ophthalmologica. 1943;106:14.Google Scholar
  186. 186.
    Graham CM, McCartney ACE, Buckley RJ. Intrascleral neurilemmoma. Br J Ophthalmol. 1989;73:378.PubMedGoogle Scholar
  187. 187.
    Reese AB. Precancerous and cancerous melanosis. Am J Ophthalmol. 1966;61:1272.PubMedGoogle Scholar
  188. 188.
    Crandall AS, Yanoff M, Schaffer DB. Intrascleral nerve loop mistakenly identified as a foreign body. Arch Ophthalmol. 1977;95:497.PubMedGoogle Scholar
  189. 189.
    Jay B. Naevi and melanomata of the conjunctiva. Br J Ophthalmol. 1965;49:169.PubMedGoogle Scholar
  190. 190.
    Lee JS, Smith RE, Minckler DS. Scleral melanocytoma. Ophthalmology. 1982;89:178.PubMedGoogle Scholar
  191. 191.
    Epstein E, Bragg K, Linden G. Biopsy and prognosis of malignant melanoma. JAMA. 1969;208:1369.PubMedGoogle Scholar
  192. 192.
    Garret M. Ocular metastasis from seminoma. Br J Ophthalmol. 1959;43:759.Google Scholar
  193. 193.
    Sacks I. Kaposi’s disease manifesting in the eye. Br J Ophthalmol. 1956;40:574.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Maite Sainz de la Maza
    • 1
  • Joseph Tauber
    • 2
  • C. Stephen Foster
    • 3
  1. 1.Clinical Institute of OphthalmologyHospital Clinic of BarcelonaBarcelonaSpain
  2. 2.Tauber Eye CenterKansas CityUSA
  3. 3.Massachusetts Eye Research and Surgery InstitutionCambridgeUSA

Personalised recommendations