Infantile, Childhood, and Juvenile Glaucomas

  • David S. Walton


The childhood glaucomas are a clinically diverse group of ocular disorders. On examination many different potential primary and secondary etiologies of the glaucoma must be considered. The systemic conditions associated with the pediatric glaucomas also need to be recognized. The profound and common anterior segment abnormalities secondary to the increased intraocular pressure (IOP) seen in these children makes their conditions seem more similar than is necessarily true. The purpose of this chapter is to provide information to assist with the examination of children with glaucomas to enable physicians to recognize them more accurately, so that more appropriate and optimum glaucoma treatment decisions will be made.


Anterior Chamber Trabecular Meshwork Scleral Flap Ahmed Glaucoma Valve Glaucoma Drainage Device 
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.
    Hoskins DH, Shaffer RN, Hetherington J. Anatomical classification of the developmental glaucomas. Arch Ophthalmol. 1984;102:1331–1336.PubMedGoogle Scholar
  2. 2.
    Anderson DR. Pathology of the glaucomas. Br J Ophthalmol. 1972;56:146–157.CrossRefPubMedGoogle Scholar
  3. 3.
    Tawara A, Inomata H. Developmental immaturity of the trabecular meshwork in congenital glaucoma. Am J Ophthalmol. 1981;92:508–525.PubMedGoogle Scholar
  4. 4.
    Barkan O. Pathogenesis of congenital glaucoma: gonioscopic and anatomic observation of the angle of the anterior chamber in the normal eye and in congenital glaucoma. Am J Ophthalmol. 1955;40:1–11.PubMedGoogle Scholar
  5. 5.
    Worst JGF. Congenital glaucoma: remarks on the aspect of chamber angle, ontogenetic and pathogenetic background, and mode of action of goniotomy. Invest Ophthalmol. 1968;7:127–134.PubMedGoogle Scholar
  6. 6.
    Anderson DR. The development of the trabecular meshwork and its abnormality in primary infantile glaucoma. Trans Am Ophthalmol Soc. 1981;79:458–485.PubMedGoogle Scholar
  7. 7.
    Tawara A, Inomata H, Tsukamoto S. Ciliary body band width as an indicator of goniodysgenesis. Am J Ophthalmol. 1996;122:790–800.PubMedGoogle Scholar
  8. 8.
    Maul E, Strozzi L, Munoz C, et al. The outflow pathway in congenital glaucoma. Am J Ophthalmol. 1980;89:667–675.PubMedGoogle Scholar
  9. 9.
    Tawara A, Inomata H. Congenital abnormalities of the trabecular meshwork in primary glaucoma with open angle. Glaucoma. 1987;9:28–34.Google Scholar
  10. 10.
    Maumenee AE. The pathogenesis of congenital glaucoma: a new theory. Trans Am Ophthalmol Soc. 1958;56:507–570.PubMedGoogle Scholar
  11. 11.
    Wright JD, Robb RM, Deuker DK, et al. Congenital glaucoma unresponsive to conventional therapy: a clinicopathological case presentation. J Pediatr Ophthalmol Strabismus. 1983;20:172–179.PubMedGoogle Scholar
  12. 12.
    Kolker AE, Hetherington J. Congenital glaucoma. In: Becker-Shaffer’s Diagnosis and Therapy of the Glaucomas. 5th ed. St Louis: CV Mosby; 1983: 317 [ Chapter 18].
  13. 13.
    Sarfarazi M, Arkarsu AN, Hossain A, et al. Assignment of a locus(GLC3A) for primary congenital glaucoma(buphthalmos) to 2p21 and evidence for genetic heterogeneity. Genomics. 1995;30: 171–178.CrossRefPubMedGoogle Scholar
  14. 14.
    Stoilov I, Nurten A, Sarfarazi M. Identification of three different truncating mutations in cytochrome P4501B1 (CYP1B1) as the principal cause of primary congenital glaucoma in families linked to the GLC3A on chromosome 2p21. Hum Mol Genet. 1997;6: 641–647.CrossRefPubMedGoogle Scholar
  15. 15.
    Miller SJH. Genetic aspects of glaucoma. Trans Ophthalmol Soc UK. 1966;86:425–434.PubMedGoogle Scholar
  16. 16.
    Taylor RH, Ainsworth JR, Evans AR, et al. The epidemiology of pediatric glaucoma: The Toronto experience. J AAPOS. 1999;3: 308–315.CrossRefPubMedGoogle Scholar
  17. 17.
    Sarfarazi M, Stoilov I. Molecular genetics of primary congenital glaucoma. Eye. 2000;14:422–428.PubMedGoogle Scholar
  18. 18.
    Akarsu AN, Turacli ME, Aktan SG, et al. A second locus (GLC3B) for primary glaucoma (buphthalmos) maps to the 1p36 region. Hum Mol Genet. 1996;5:1199–1203.CrossRefPubMedGoogle Scholar
  19. 19.
    Stoilov IR, Sarfarazzi M. The Third Genetic Locus(GLC3C) for Primary Congenital Glaucoma (PCG) Maps to Chromosome 14q24.3. Fort Lauderdale (FL): Association for Research in Vision and Ophthalmology; 2002.Google Scholar
  20. 20.
    Panicker SG, Mandal AN, Reddy ABM, et al. Correlations of genotype with phenotype in Indian patients with primary congenital glaucoma. Invest Ophthalmol Vis Sci. 2004;45:1149–1156.CrossRefPubMedGoogle Scholar
  21. 21.
    Bejjani BA, Stockton DW, Lewis RA, et al. Multiple CYP1B1 mutations and incomplete penetrance in an inbred population segregating primary congenital glaucoma suggest frequent de novo events and a dominant modifier locus. Hum Mol Genet. 2000;9:367–374.CrossRefPubMedGoogle Scholar
  22. 22.
    Alward WLM, Semina EV, Kalenak JW, et al. Autosomal dominant iris hypoplasia is caused by a mutation in Rieger syndrome (RIEG/PITX2) gene. Am J Ophthalmol. 1998;125:98–100.CrossRefPubMedGoogle Scholar
  23. 23.
    Nishimura DY, Swiderski RE, Alward WLM, et al. The forkhead transcription factor gene FKHL7 is responsible for glaucoma phenotypes which map to 6p25. Nat Genet. 1998;19:140–147.CrossRefPubMedGoogle Scholar
  24. 24.
    Alward WLM, Fingert JH, Coote MA, et al. Clinical features associated with mutations in the chromosome 1 open angle glaucoma gene (GLC1A). N Engl J Med. 1998;338:1022–1023.CrossRefPubMedGoogle Scholar
  25. 25.
    Freedman SF. Primary congenital glaucoma. In: Albert DM, Jacobiec FA (aus): Principles and Practice of Ophthalmology. WB Saunders Company; 2000.Google Scholar
  26. 26.
    Zimmerman TJ, Kooner KS, Morgan KS. Safety and efficacy of timolol in pediatric glaucoma. Surv Ophthalmol. 1983;28:262.CrossRefPubMedGoogle Scholar
  27. 27.
    Hoskins HDJ, Hetherington JJ, Magee SD, et al. Clinical experience with timolol in childhood glaucoma. Arch Ophthalmol. 1985;103:1163.PubMedGoogle Scholar
  28. 28.
    McMahon CD, Hetherington JJ, Hoskins HDJ, et al. Timolol and pediatric glaucomas. Ophthalmology. 1981;88:249.PubMedGoogle Scholar
  29. 29.
    Williams T, Ginther WH. Hazard of ophthalmic timolol. N Eng J Med. 1982;306:1485.CrossRefGoogle Scholar
  30. 30.
    Olson RJ, Bromberg BB, Zimmerman TJ. Apneic spells associated with timolol therapy in a neonate. Am J Ophthalmol. 1979;88:120–121.PubMedGoogle Scholar
  31. 31.
    Toris CB, Gleason ML, Camras CB, et al. Effects of brimonidine on aqueous humor dynamics in human eyes. Arch Ophthalmol. 1995;113:1514–1517.PubMedGoogle Scholar
  32. 32.
    Juzych M, Robin A, Novack G. Alpha-2 agonists in glaucoma therapy. In: Zimmerman T, Kooner K, Sharir M, Fechtner R, eds. Textbook Of Ocular Pharmacology. Philadelphia: Lippincott-Raven; 1997: 247–54.Google Scholar
  33. 33.
    Carlsen JO, Zabriskie NA, Kwon YH, et al. Apparent central nervous system depression in infants after the use of topical brimonidine. Am J Ophthalmol. 1999;128:255–256.CrossRefPubMedGoogle Scholar
  34. 34.
    Korsch E, Grote A, Seybold M, et al. Systemic adverse effects of topical treatment with brimonidine in an infant with secondary glaucoma. Eur J Pediatr. 1999;158:685.CrossRefPubMedGoogle Scholar
  35. 35.
    Mungan NK, Wilson TW, Nischal KK, et al. Hypotension and bradycardia in infants after the use of topical brimonidine and beta-blockers. J AAPOS. 2003;7:69–70.CrossRefPubMedGoogle Scholar
  36. 36.
    Berlin RJ, Lee UT, Samples JR, et al. Ophthalmic drops causing coma in an infant. J Pediatr. 2001;138:441–443.CrossRefPubMedGoogle Scholar
  37. 37.
    Enyedi LB, Freedman SF. Safety and efficacy of brimonidine in children with glaucoma. J AAPOS. 2001;5:281–284.CrossRefPubMedGoogle Scholar
  38. 38.
    Parrish RK, Palmberg P. Sheu WP; XLT Study Group. A comparison of latanoprost, bimatoprost, and travoprost in patients with elevated intraocular pressure: a 12-week, randomized, masked-evaluator multicenter study. Am J Ophthalmol. 2003;135:688–703.CrossRefPubMedGoogle Scholar
  39. 39.
    Enyedi LB, Freedman SF. Latanoprost for the treatment of pediatric glaucoma. Surv Ophthalmol. 2002;47(Suppl 1):S129-S132.CrossRefPubMedGoogle Scholar
  40. 40.
    Enyedi LB, Freedman SF, Buckley EG. The effectiveness of latanoprost for the treatment of pediatric glaucoma. J AAPOS. 1999;3:33–39.CrossRefPubMedGoogle Scholar
  41. 41.
    Barkan O. Technic of goniotomy. Arch Ophthalmol. 1938;19:217–223.Google Scholar
  42. 42.
    Barkan O. Operation for congenital glaucoma. Am J Ophthalmol. 1942;25:552–568.Google Scholar
  43. 43.
    Ho CL, Walton DS. Goniosurgery for glaucoma secondary to chronic anterior uveitis. prognostic factors and surgical technique. J Glaucoma. 2004;13:445–449.CrossRefPubMedGoogle Scholar
  44. 44.
    Walton DS. Goniotomy. In: JT, ed. Glaucoma Surgery. St Louis, MO: Mosby-Year-Book, Inc.; 1992:107–121.Google Scholar
  45. 45.
    de Luise VP, Anderson DR. Primary infantile glaucoma (congenital glaucoma). Surv Ophthalmol. 1983;28:1–19.CrossRefGoogle Scholar
  46. 46.
    Boughton Wl, Parks MM. An analysis of treatment of congenital glaucoma by gonitomy. Am J Ophthalmol. 1981;91:566–572.Google Scholar
  47. 47.
    Mandal AK, Gothwal VK, Bagga H, et al. Outcome of surgery on infants younger than 1 month with congenital glaucoma. Ophthalmology. 2003;110:1909–1915.CrossRefPubMedGoogle Scholar
  48. 48.
    Shaffer RN. Prognosis of goniotomy in primary infantile glaucoma (trabeculodysgenesis). Tr Am Oph Soc.. 1982;80:321–325.Google Scholar
  49. 49.
    Russell-Eggitt IM, Rice NSC, Barrie J, et al. Relapse following goniotomy for congenital glaucoma due to trabecular dysgenesis. Eye. 1992;6:197–200.PubMedGoogle Scholar
  50. 50.
    McPherson SD Jr. Results of external trabeculotomy. Trans Am Ophthalmol Soc. 1973;71:163–170.PubMedGoogle Scholar
  51. 51.
    McPherson SD Jr, MacFarland D. External trabeculotomy for developmental glaucoma. Ophthalmol. 1980;87:302–305.Google Scholar
  52. 52.
    McPherson SD Jr, Berry DP. Goniotomy vs external trabeculotomy for developmental glaucoma. Ophthalmol. 1980;87:302–305.Google Scholar
  53. 53.
    Mandal A, Naduvilath TJ, Jayagandan DO. Surgical results of combined trabeculotomy-trabeculectomy for developmental glaucoma. Ophthalmology. 1998;105:974–982.CrossRefPubMedGoogle Scholar
  54. 54.
    Harms H, Dannheim R. Epicritical consideration of 300 cases of trabeculotomy ab externo. Trans Ophthalmol Soc UK. 1969;89:491–499.Google Scholar
  55. 55.
    Shrader CE, Cibis GW. Trabeculotomy. In: J T, ed. Glaucoma Surgery. St Louis, MO: Mosby-Year Book, Inc.; 1992:123–131.Google Scholar
  56. 56.
    Mendicino ME, Lynch MG, Drack A, et al. Long-term surgical and visual outcomes in primary congenital glaucoma: 360 degrees trabeculotomy versus goniotomy. J AAPOS. 2000;4:205–210.CrossRefPubMedGoogle Scholar
  57. 57.
    Akimoto M, Tamihara H, Negi A, et al. Surgical results of trabeculotomy ab externo for developmental glaucoma. Arch Ophthalmol. 1994;112:1540–1544.PubMedGoogle Scholar
  58. 58.
    Filous A, Brunova B. Results of the modified trabeculotomy in the treatment of primary congenital glaucoma. J AAPOS. 2002;6:182–186.CrossRefPubMedGoogle Scholar
  59. 59.
    Beauchamp GR, Parks MM. Filtering surgery in children: barriers to success. Ophthalmol. 1979;86:170–180.Google Scholar
  60. 60.
    Beck AD, Wilson WR, Lynch MG, et al. Trabeculectomy with adjunctive mitomycin-C in pediatric glaucoma. Am J Ophthalmol. 1998;126:648–657.CrossRefPubMedGoogle Scholar
  61. 61.
    Freedman S, McCormick K, Cox T. Mitomycin-C augmented trabeculectomy with postoperative wound modulation in pediatric glaucoma. J AAPOS. 1999;3:117–124.CrossRefPubMedGoogle Scholar
  62. 62.
    Wells AP, Cordeiro MF, Bunce C, et al. Cystic bleb formation and related complications in limbus- versus fornix-based conjunctival flaps in pediatric and young adult trabeculectomy with mitomycin C. Ophthalmology. 2003;110:2192–2197.CrossRefPubMedGoogle Scholar
  63. 63.
    Susanna R, Oltrogge EW, Carani JE, et al. Mitomycin as adjunct chemotherapy with trabeculectomy in congenital and developmental glaucomas. J Glaucoma. 1995;4:151–188.PubMedGoogle Scholar
  64. 64.
    Sidoti PA, Belmonte SJ, Liebmann JM, et al. Trabeculectomy with mitomycin-C in the treatment of pediatric glaucoma. Ophthalmology. 2000;107:422–429.CrossRefPubMedGoogle Scholar
  65. 65.
    Agarwal HC, Sood NN, Sihota R, et al. Mitomycin-C in congenital glaucoma. Ophthalmol Surg Lasers. 1997;28:979–985.Google Scholar
  66. 66.
    Tannenbaum DP, Hoffman D, Greaney MJ, et al. Outcomes of bleb excision and conjunctival advancement for leaking or hypotonous eyes after glaucoma filtering surgery. Br J Ophthalmol. 2004;88:99–103.CrossRefPubMedGoogle Scholar
  67. 67.
    Hill R, Heur D, Baerveldt G, et al. Molteno implantation for glaucoma in young patients. Ophthalmology. 1991;98:1042–1046.PubMedGoogle Scholar
  68. 68.
    Nesher R, Sherwood M, Kass M, et al. Molteno implants in children. J Glaucoma 1992;1:228–232.CrossRefPubMedGoogle Scholar
  69. 69.
    Walton DS, Katsavounidou G. Newborn primary congenital glaucoma:2005 update. J Pediatr Ophthalmol Strabismus. 2005;42:333–341.PubMedGoogle Scholar
  70. 70.
    Beck AD, Freedman S, Kammer J, Jin J. Aqueous shunt devices compared with trabeculectomy with mitomycin-C for children in the first two years of life. Am J Ophthalmol. 2003;136:994–1000.CrossRefPubMedGoogle Scholar
  71. 71.
    Coleman A, Smyth R, Wilson R, Tam M. Initial clinical experience with the ahmed glaucoma valve implant in pediatric patients. Arch Ophthalmol. 1997;115:186–191.PubMedGoogle Scholar
  72. 72.
    Englert J, Freedman S, Cox T. The ahmed valve in refractory pediatric glaucoma. Am J Ophthalmol. 1999;127:34–42.CrossRefPubMedGoogle Scholar
  73. 73.
    Morod Y, Donaldson C, Kim Y, et al. The Ahmed drainage implant in the treatment of pediatric glaucoma. Am J Ophthalmol. 2003;135: 821–829.CrossRefGoogle Scholar
  74. 74.
    Al-Torbaq AA, Edward DP. Delayed endopthalmitis in a child following an Ahmed glaucoma valve implant. J AAPOS. 2002;6:123–125.CrossRefPubMedGoogle Scholar
  75. 75.
    Al Faran MF, Tomey KF, Mutlaq FA. Cyclocryotherapy in selected cases of congenital glaucoma. Ophthalmic Surg. 1990;21:794–798.Google Scholar
  76. 76.
    Wagle NS, Freedman SF, Buckley EG, et al. Long-term outcome of cyclocryotherapy for refractory pediatric glaucoma. Ophthalmology. 1998;105:1921-6; discussion 1926–1927.CrossRefPubMedGoogle Scholar
  77. 77.
    Kirwan JF, Shah P, Khaw PT. Diode laser cyclophotocoagulation: role in the management of refractory pediatric glaucomas. Ophthalmology. 2002;109:316–323.CrossRefPubMedGoogle Scholar
  78. 78.
    Plager DA, Neely DE. Intermediate-term results of endoscopic diode laser cyclophotocoagulation for pediatric glaucoma. J AAPOS. 1999;3:131–137.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • David S. Walton
    • 1
  1. 1.Department of OphthalmologyHarvard Medical SchoolBostonUSA

Personalised recommendations