Skip to main content
SpringerLink
Log in

Goniodysgenesis or Late Congenital Glaucoma. Pigmentary Glaucoma

  • Chapter
The Glaucomas

  • 984 Accesses

Abstract

The diagnosis of late congenital glaucoma is basically made through the chamber angle. These are children over 6 years of age, young people, or adults up to 45 years of age, presenting persistence of pathologic mesodermal tissue reaching at least to the spur, the trabecular meshwork, the height of the Schlemm canal, or even further up to the Schwalbe line. The most outstanding sign in gonioscopy is the absence of the ciliary body band, which may be total or partial. This is a fetal alteration of the development of the iridocorneal angle called goniodysgenesis and it is accompanied by a halt in the development of the superficial mesodermal layer of the iris that enables the radial vascular and avascular columns of the deep iris to be seen, and at the avascular columns, the black triangles can be seen corresponding to the posterior pigmentary epithelium of the iris. In general, peripheral atrophy of the superficial layer of the iris is visible.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 219.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 279.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 379.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Busacca A, Carvalho C (1968) La morphogenèse du sinus camérulaire par la gonioscopie. Ann Ocul 201:400–430

    Google Scholar 

  2. Gorin G (1964) Developmental glaucoma. Am J Ophthalmol 58:572–580

    PubMed  CAS  Google Scholar 

  3. Kniestedt C, Kammann M, Sturmer J, Gloor B (2000) Dysgenetische Kammerwinkelveränderungen bei Patienten mit Glaukom oder Verdacht auf Glaukom aufgetreten vor dem 40. Lebensjahr Klin Mbl Augenheilkd 216:377–387

    Article  CAS  Google Scholar 

  4. Jerndal T, Hansson H A, Bill A (1978) Goniodygenesis. A new perspective on glaucoma. Scriptor, Copenhagen

    Google Scholar 

  5. Boles Carenini B (1965) Contributo alla conoscenza del cosidetto glaucoma giovanile. Ann Ottal XCI:140–170

    Google Scholar 

  6. Kluyskens J (1950) Le glaucome congénital. Rapport présenté à la Société Belge d’Ophtalmologie 94:159–160

    Google Scholar 

  7. Sampaolesi R (1962) Nuevo signo gonioscópico en los glaucomas congénitos de aparición tardía. Arch Octal B Aires 37:161–165

    CAS  Google Scholar 

  8. Sampaolesi R (1968) New goniscopic signs in congenital glaucoma of late onset. First South American Symposium on Glaucoma, Bariloche 1966. Mod Probl Ophthalmol 6:106–123

    Google Scholar 

  9. Rieger H (1934) Verlagerung und Schlitzform der Pupille mit Hipoplasie des Irisvorderblattes. Z Augenheilk 84:98–99

    Google Scholar 

  10. Rieger H (1935) Beiträge zur Kenntnis seltener Missbildungen der Iris. II. Über Hypoplasie des Irisvorderblattes mit Verlagerung und Entrundung der Pupille. Albrecht von Graefes Arch Klin Exp Ophthalmol 133:602–635

    Article  Google Scholar 

  11. Arnold PH (1911) Veränderungen des Oberflächen-Relief der Iris an der Stelle des Augenblasensplates. Klin Mbl Augenheilk 47:441–560

    Google Scholar 

  12. Streiff J (1915) Über eine untere Irismulde und über Iristypen und Übergang zur Anomalie. Klin Mbl Augenheilk 54:33–48

    Google Scholar 

  13. Vogt A (1931) Slit lamp microscopy of the living eye, Vol III. Springer, Berlin Heidelberg New York

    Google Scholar 

  14. Sugar HS, Barbour FA (1949) Pigmentary glaucoma: a rare clinical entity. Am J Ophthalmol 32:90–92

    PubMed  CAS  Google Scholar 

  15. Malbran J (1957) Le glaucome pigmentaire, ses relations avec le glaucome congénital. Probl Act Ophtalmol I, pp 132–146

    Google Scholar 

  16. Etienne R (1960) L’atrophie essentielle des couches epithéliales de l’iris et du corps ciliaire. Ses relations avec le glaucome. Ann Oculist 192:224–244

    Google Scholar 

  17. Etienne R, Pommie. ML (1957) Contribution à l’étude du glaucome pigmentaire. Ann Oculist 190:491–499

    CAS  Google Scholar 

  18. Weekers R, Watillon M (1966) Hypertension oculaires attribuables à fraction conjuguée d’une anomalie mésodermique et d’une migration pigmentaire. Bull Soc Ophthalmol France 637–650

    Google Scholar 

  19. Di Tizio A, Le Pri L (1963) Contributto clinico allo studio del glaucoma pigmentario. Boll Oculist XLII:435–453

    Google Scholar 

  20. Nordmann J, Gerhard JP, Payeur G (1966) A propos du glaucome dit pigmentaire. Bull Soc Ophtal France 651–655

    Google Scholar 

  21. Heinzen H, Lueder R (1960) The value of gonioscopy for the diagnosis of pigmental glaucoma demonstrated by 9 personal cases. Ophthalmologica 139:244–2454

    PubMed  CAS  Google Scholar 

  22. Shaffer RN, Weiss DI (1970) Congenital and pediatric glaucoma. Mosby, St Louis, pp 107, 117, 119

    Google Scholar 

  23. Mauksch H (1925) Über idiopathischen Zerfall des retinalen Pigmentblattes der Iris bei zwei Brüdern. Z Augenheilk 57:262

    Google Scholar 

  24. Cavara W (1929) Contributto a la conocenza del fusi di Krukenberg. Boll Ocul 8:1161

    Google Scholar 

  25. Koby F (1932) Biomicroscopie du corps vitre. Masson, Paris

    Google Scholar 

  26. Focosy M (1933) Su i depositi pigmentati della cristalloide posteriori. Boll Ocul 12:873

    Google Scholar 

  27. Bietti G (1934) Weitere Beitrage zur Kenntniss des Retrolentikularen Pigment-Ringes und zu seiner Entstehnungsweise. Klin Mbl Augenheilk 93:54

    Google Scholar 

  28. Mann I (1937) Developmental abnormalities of the eye. Cambridge: Cambridge University Press

    Google Scholar 

  29. Zentmayer W (1938) Association of anular band of pigment on posterior capsule of the lens with a Krukenberg’s spindle. Arch Ophthalmol Chicago 20:52

    Google Scholar 

  30. Evans WH, Odom RE, Wenaas EJ (1941) Krukenberg’s spindles: a study of two hundred and two collected cases. Arch Ophthalmol Chicago 26:1023–1056

    Google Scholar 

  31. Cameron W (1941) Krukenberg’s spindle associated with megalocornea and posterior pigmentation of the lens. Am J Ophthalmol 24:687–689

    Google Scholar 

  32. Bellows JG (1944) Krukenberg’s spindle and its relation to anular pigment band on the periphery of the lens. Arch Ophthalmol Chicago 32:480–482

    Google Scholar 

  33. Becker B, Shin DH, Cooper DG, Kass M (1977) The pigment dispersion syndrome. Am J Ophthalmol 83:161–166

    PubMed  CAS  Google Scholar 

  34. Kaiser-Kupfer MI (1980) Clinical research methodology in ophthalmology. Trans. Am Ophthal Soc LXXCVIII:896–94

    Google Scholar 

  35. Farrar SM, Shields MB, Miller KN, Stoup CM (1989) Risk factors for the development and severity of glaucoma in the pigment dispersion syndrome. Am J Ophthalmol 108:223–229

    PubMed  CAS  Google Scholar 

  36. Campbelll, DG (1979) Pigmentary dispersion and glaucoma: a new theory. Arch Ophthalmol 97:1667–1672

    Google Scholar 

  37. Calixto N (1981) Contribuição para o estudo de alguns aspectos da sindrome de dispersao pigmentaria do segmento interior de olho. PhD thesis. Facultade de Medicina da Universidade Federal de Minas Gerais

    Google Scholar 

  38. Calixto N, Cronenberg S (1985) Glaucoma pigmentario sem pigmento. Rev Bras Oftalmol 44:30–40

    Google Scholar 

  39. Brachet A, Chermet M (1974) Association glaucome pigmentaire et décollement de rétine. Ann Oculist 207:451–457

    Google Scholar 

  40. Sampaolesi (1975) Desprendimiento de retina y glaucoma pigmentario. Arch Oftalmol Buenos Aires 50:370–375

    Google Scholar 

  41. Richter CU, Richardson TM, Grane WM (1986) Pigmentary dispersion syndrome and pigmentary glaucoma: a prospective study of the natural history. Arch Ophthalmol 104:211

    PubMed  CAS  Google Scholar 

  42. Richardson TM (1989) Pigmentary glaucoma. In: Ritch R., Shields MB, Krupin TS (eds) The glaucomas. Mosby, St. Louis, pp  981–995

    Google Scholar 

  43. Rosen E (1962) Microzonuloscopy. In Krukenberg spindles and annular pigment rings of the lens. Am J Ophthalmol 53:845–853

    PubMed  CAS  Google Scholar 

  44. Scheie HG, Fleischauer HW (1958) Idiopathic atrophy of the epithelial layers of the iris and ciliary body. Arch Ophthalmol 59:216

    CAS  Google Scholar 

  45. Mapstone R (1981) Pigment release. Br J Ophthalmol 65:258–263

    Article  PubMed  CAS  Google Scholar 

  46. Ritch R, Shields MB, Krupin T (1996) The glaucomas, Clinical Science, 2nd edn. Mosby, St Louis

    Google Scholar 

  47. Kaufman PL, Mittag TW (1991) In: Podos SM, Yanoff M (eds) Glaucoma textbook of ophthalmology, Vol. 7. Podos-Yanoff, Mosby, St. Louis

    Google Scholar 

  48. Sugar HS (1957) The glaucomas, 2nd edn. Hoeber-Harper, New York

    Google Scholar 

  49. Sugar HS (1966) Pigmentary glaucoma: 25 year review. Am J Ophthalmol 62:499–507

    PubMed  CAS  Google Scholar 

  50. Bick MW (1957) Pigmentary glaucoma in females. Arch Ophthalmol 58:483–494

    CAS  Google Scholar 

  51. Krukenberg F (1899) Beiderseitige angeborene Melanose der Hornhaut. Klin Mbl Augenheilk 37:254–258

    Google Scholar 

  52. Hansen NR (1923) Über Hornhautverfärbung. Klin Mbl Augenheilk 81:399

    Google Scholar 

  53. Francois J (1958) L’hérédité en ophtalmologie. Massos, Paris

    Google Scholar 

  54. Shaffer R (1967) Symposium on glaucoma. Transactions of the New Orleans Academy of Ophthalmology. Mosby, St. Louis

    Google Scholar 

  55. Anderson JS, Pralea AM, DelBono EA et al (1997) A gene responsible for the pigment dispersion syndrome maps to chromosome 7q35-q36. Arch Ophthalmol 115:384–388

    Google Scholar 

  56. Anderson JS, Delbono EA, Haines JL, Wiggs JL (1999) Identification and genetic analysis of pigmentary glaucoma Loci on 7q36 and 18q. Invest Ophthalmol Vis Sci 140:5596

    Google Scholar 

  57. Ritch R, Shields MB, Krupin TS (1989) The glaucomas, 1sr edn. Mosby, St Louis, pp. 981–995

    Google Scholar 

  58. Sampaolesi R (1995) Amotio retinae und Pigmentdispersionssyndrom. Klim Mbl Augenheilkd 206:29–32

    Article  CAS  Google Scholar 

  59. Sampaolesi R (1974) Glaucoma, 1st edn. Editorial Médica Panamericana, Buenos Aires pp 735–765

    Google Scholar 

  60. Sampaolesi R (1967) El llamado glaucoma pigmentario, su relación con el glaucoma congénito. Arch Oftalmol Argentinos 367–383

    Google Scholar 

  61. Sampaolesi R (1968) Le glaucome dit pigmentaire, son rapport avec le glaucome congénitale. Bull Mem Soc Fr Ophtalmol 434–463

    Google Scholar 

  62. Brachet A, Chermet M (1974) Association glaucome pigmentaire et décollement de retine. Ann Oculist 207: 451–457

    Google Scholar 

  63. Campbelll DG (1994) Pigmentary glaucoma: mechanism and role for laser iridotomy. J Glaucoma 3:173–174

    Article  Google Scholar 

  64. Jampel HD (1993) Lack of effect of peripheral laser iridotomy in pigment dispersion syndrome. Arch Ophthalmol 111:1606

    PubMed  CAS  Google Scholar 

  65. Lehto I (1997) Läßt sich vom Effekt der Iridotomie beim Pigmentglaukom auf den Pathomechanismus schieben? Universitäts-Augenklinik, Helsinki, Finland

    Google Scholar 

  66. Nordmann J (1968) Discusión al trabajo “Le glaucome dit pigmentarie,” Sampaolesi. BuIl Soc Fr Ophtalmol 434–463

    Google Scholar 

  67. Alper JC (1985) Congenital nevi. The controversy rages on. Arch Dermatol 121:734–735

    Article  PubMed  CAS  Google Scholar 

  68. Blanco MF; Mazzini MA (1956) Clínica Dermatológica e Sifilográfica.Hachette, Guanabara, RJ, Brazil, pp 104–1075

    Google Scholar 

  69. Bourquin J (1917) Angeborene melanose des auges. Zeitschr Augenheilk 37:129

    Google Scholar 

  70. Capeans Tomé C (1999) Actualización en tumores intraoculares. Tecnimedia Editorial, pp 127–140

    Google Scholar 

  71. Calixto N, Cronemberg S (1989) Glaucoma pigmentário. In: Almeida HG, Almeida GV, Calixto NE, Carvalho CA (eds) Roca, Sao Paulo, pp 277–290.

    Google Scholar 

  72. Cu-Unjieng AB, Shields JA, Eagle RC, Shields CL, Marmor M, De Potter P (1995) Iris melanoma in ocular melanocytosis. Córne 14:206–209

    CAS  Google Scholar 

  73. Elder DE, Murphy GF (1990) Melanocytic tumors of the skin. Atlas of tumor pathology, 3rd Series. Fasc. 2. Armed Forces Institute of Pathology, Washington, DC, p 64

    Google Scholar 

  74. Elshaw SR, Sisley K, Cross N, Murray AK, MacNeil SM, Wagner M, Nichols CE, Rennie IG (2001) A comparison of ocular melanocyte and uveal melanoma cell invasion and the implication of alpha 1beta1, alpha4beta1 and alpha6beta1 integrins. Br J Ophthalmol 85:732–738

    Article  PubMed  CAS  Google Scholar 

  75. Friedman SM, Margo CE (1998) Choroidal melanoma and neurofibromatosis type 1. Arch Opthalmol 116:694–695

    CAS  Google Scholar 

  76. Garrido CM, Arra A (1987) Valve of the S100 protein in the study of nevus and ocular melanomas. Ophthalmologica 194:201–203

    Article  PubMed  CAS  Google Scholar 

  77. Gunduz K, Shields J, Shields CL et al (1998) Choroidal melanoma in a 14- year-old patient with ocular melanocytosis. Arch Ophthalmol 116:1112–1114

    PubMed  CAS  Google Scholar 

  78. Gunduz K, Shields CL, Shields JA, Eagle RC Jr, Singh AD (2000) Iris mammillations as the only sign of ocular melanocytosis in a chi choroidal melanoma. Arch Ophthalmol 118:716–717

    PubMed  CAS  Google Scholar 

  79. Heegaard S, Jensen OA, Prause JU (2000) Immunohistochemical diagnosis of malignant melanoma of the conjunctiva and uvea: comparison of the novel antibody against m with S100 protein and HMB45. Melanoma Res 10:350–354

    Article  PubMed  CAS  Google Scholar 

  80. Heegaard S, Jensen OA, Prause JU (2000) Immunohistochemical diagnosis of malignant melanoma in Queensland, Australia. Ophthalmic Epidermiol 7:159–167

    Article  Google Scholar 

  81. Hogan MJ, Alvarado JA, Weddell JE (1971) Histology of the human eye. WB Saunders, Philadelphia

    Google Scholar 

  82. Honavar SG, Singh AD, Shields CL, Shields JA, Eagle RC Jr (2000) Iris melanoma in a patient with neurofibromatosis. Surv Ophthalmol 45:231–236

    Article  PubMed  CAS  Google Scholar 

  83. Honavar SG, Shields CL, Singh AD, Demirci H, Rutledge BK, Shields JA, Eagle RC Jr (2002) Two discrete choroidal melanomas in an eye with ocular melanoma. Surv Ophthalmol 47:36–41

    Article  PubMed  Google Scholar 

  84. Infante de German-Ribon R, Singh AD, Arevalo JF, Driebe W, Eskin T (1999) Choroidal melanoma with oculodermal melanocytosis in Hispanic patients. Am J Ophthalmol 128:251–253

    Article  PubMed  CAS  Google Scholar 

  85. Jakobiec FA (1982) Ocular anatomy, embryology and teratology. Harper and Son, Philadelphia, pp 97–119

    Google Scholar 

  86. Kadonga JN, Frieden IJ (1991) Neurocutaneus melanosis definition and review of the literature. J Am Acad Dermatol 24:747–755

    Article  Google Scholar 

  87. Kaufer G (1969) Nevus melanosis adquiridas y melanomas de conjuntiva. Arch Oftalmol Buenos Aires. TXLIV:177–184

    Google Scholar 

  88. Magnin PH (1969) Bases de la melanogénesis humana. Editorial Universitaria de Buenos Aires, Buenos Aires

    Google Scholar 

  89. Matthews JL, Martin JH (1971) Atlas of human histology and ultrastructure. Lea and Febiger, Philadelphia

    Google Scholar 

  90. McLean W, Burnier MN, Zimerman LE, Jakobiec FA (1994) Tumors of the eye and ocular adnexa. Atlas of tumor pathology, 3rd Series. Fasc. 12. Armed Forces Institute of Pathology, Washington, DC, pp 76–82

    Google Scholar 

  91. Meerhoff W, Sanabria D, Bonifaciiino R et al (1995) Melanoma de conjuntiva en melanosis primaria adquirida. Arch Cátedra Oftalmol 2:4–9

    Google Scholar 

  92. Nawa Y, Yoshiaki H, Mototuev S (1999) Conjunctival melanoma associated with extensive congenital nevus and split nevus of eyelid. Arch Ophthalmol 117:269–271

    PubMed  CAS  Google Scholar 

  93. Neale MH, Myatt NE, Khoury GG, Weaber P, Lamon A, Hungerford JL, Kurbacher CM, Hall P, Corrie PG, Cree IA (2001) Comparison of the ex vivo chosen sensitivity of uveal and cutaneous melanoma. Melanoma Res 11:601–609

    Article  PubMed  CAS  Google Scholar 

  94. Offret G, Dhermy P, Brini A, Bec P (1974) Anatomie pathologique de L’oeil et de ses annexes. Masson, Paris, pp 46–54

    Google Scholar 

  95. Ota M (1939) Nevus fusco-coeruleus ophthalmo-maxilaris. Tokyo Med. J 63:1243

    Google Scholar 

  96. Polak M, Azcoaga JE (1967) Neurohistología. Editorial Universitaría de Buenos Aires, pp 39–40

    Google Scholar 

  97. Rehany U, Rumelt S (1999) Iridocorneal melanoma associated with type 1 neurofibromatosis: a clinicopathologic study. Ophthalmology 106:614–618

    Article  PubMed  CAS  Google Scholar 

  98. Richardson TM, Hutchinson BT, Grant WM (1977) The outflow tract in pigmentary glaucoma: a light and electron microscopic study. Arch Ophthal 195:1015

    Google Scholar 

  99. Streiff J (1926) Pigmentsternchengrupen Auf der hinteren Linsenkapsel als Spur der Membrana Capsularis in einem Fall von einseitiger Aniridie und Retinalpigmentmangel und Vorderblattanomalie der Iris am anderem Auge. Klin Mbl Augenheilk 77:610–617

    Google Scholar 

  100. To KW, Rabinowitz SM, Friedman AH et al (1989) Neurofibromatosis and neural crest neoplasms: primary acquired melanosis and malignant melanoma of the conjunctiva. Clin Patholl Re 33:373–3799

    CAS  Google Scholar 

  101. Vajdic CM, Kricker A, Giblin M, McKenzie J, Aitken J, Giles GG, Armstrong BK (2001) Eye color and cutaneous nevi predict risk of ocular in Australia. Int J Cancer 92:906–912

    Article  PubMed  CAS  Google Scholar 

  102. Warmar RE, Bullock JD, Shields JA, Eagle RC (1998) Coexistence of 3 tumors of neural crest origin: neurofibroma meningioma and uveal malignant melanoma. Arch Ophthalmol 116:1241–1243

    Google Scholar 

Download references

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

(2009). Goniodysgenesis or Late Congenital Glaucoma. Pigmentary Glaucoma. In: The Glaucomas. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-69146-4_20

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-69146-4_20

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-69144-0

  • Online ISBN: 978-3-540-69146-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation