Cataract in Childhood Glaucoma and Anterior Segment Dysgenesis

  • Sudarshan Kumar Khokhar
  • Yogita Gupta
  • Abhidnya Surve
  • Chirakshi Dhull


Cataracts can occur in association with ocular or systemic comorbidities. Presence of these comorbidities may alter the presentation and management of lens conditions. In this chapter we will be discussing two such comorbidities: childhood glaucomas and anterior segment dysgenesis. Although childhood glaucoma and anterior segment dysgenesis are separate entities, they have been clubbed together since anterior segment dysgenesis is associated with glaucoma in significant number of cases and few cases of presumed primary congenital glaucoma may actually be glaucomas secondary to anterior segment dysgenesis.


  1. 1.
    Wiggs JL, Damji KF, Haines JL, Pericak-Vance MA, Allingham RR. The distinction between juvenile and adult-onset primary open-angle glaucoma. Am J Hum Genet. Jan 1996;58(1):243–4.PubMedPubMedCentralGoogle Scholar
  2. 2.
    Feroze KB, Patel BC. Buphthalmos. In: StatPearls [internet]. Treasure Island, FL: StatPearls Publishing; 2018.. Scholar
  3. 3.
    Yanoff M, Duker JS. Ophthalmology. 3rd ed. Philadelphia: Mosby Elsevier; 2009.Google Scholar
  4. 4.
    Basic and clinical science course (2011–2012). Glaucoma. American Academy of Ophthalmology.Google Scholar
  5. 5.
    Edmunds B, Thompson JR, Salmon J, Wornald RP. The national survey of trabeculectomy III. Early and late complications. Eye. 2002;16:297–303.CrossRefGoogle Scholar
  6. 6.
    Adelman RA, Brauner SC, Afshari NA, Grosskreutz CL. Cataract formation after initial trabeculectomy in young patients. Ophthalmology. 2003;110:625–9.CrossRefGoogle Scholar
  7. 7.
    Daugeliene L, Yamamoto T, Kitazawa Y. Cataract development after trabeculectomy with mitomycin C: a 1-year study. Jpn J Ophthalmol. 2000;44:52–7.CrossRefGoogle Scholar
  8. 8.
    Khokhar SK, Pillay G, Agarwal E, Mahabir M. Innovations in pediatric cataract surgery. Indian J Ophthalmol. 2017;65(3):210–6.CrossRefGoogle Scholar
  9. 9.
    Hugkulstone CE. Changes in keratometry following trabeculectomy. Br J Ophthalmol. 1991;75(4):217–8.CrossRefGoogle Scholar
  10. 10.
    Chan HHL, Kong YXG. Glaucoma surgery and induced astigmatism: a systematic review. Eye Vis [Internet]. 2017;4.
  11. 11.
    Vaz FM, Osher RH. Cataract surgery and anterior megalophthalmos: custom intraocular lens and special considerations. J Cataract Refract Surg. 2007;33(12):2147–50.CrossRefGoogle Scholar
  12. 12.
    Sukhija J, Kaur S, Pandav SS, Kaushik S, Raj S, Ram J. Cataract surgery in children with buphthalmos. Clin Exp Ophthalmol. 2016;44(8):739–40.CrossRefGoogle Scholar
  13. 13.
    Dietlein TS, Jacobi PC, Krieglstein GK. Eyeball growth after successful glaucoma surgery in the 1st year of life--follow-up values for primary congenital glaucoma. Klin Monatsbl Augenheilkd. 1998;213(2):67–70.CrossRefGoogle Scholar
  14. 14.
    Khokhar S, Yadav D, Gupta S, Chaurasia AK, Gupta A, Gupta V. Refractive outcomes of cataract surgery in primary congenital glaucoma. Eye (Lond). 2018; [Epub ahead of print]CrossRefGoogle Scholar
  15. 15.
    Waring GO, Rodrigues MM, Laibson PR. Anterior chamber cleavage syndrome. A stepladder classification. Surv Ophthalmol. 1975;20:3–27.CrossRefGoogle Scholar
  16. 16.
    Shields MB, Buckley E, Klintworth GK, Thresher R. Axenfeld-Rieger syndrome. A spectrum of developmental disorders. Surv Ophthalmol. 1985;29:387–409.CrossRefGoogle Scholar
  17. 17.
    Townsend WM, Font RL, Zimmerman LE. Congenital corneal leukomas. 2. Histopathologic findings in 19 eyes with central defect in Descemet’s membrane. Am J Ophthalmol. 1974;77:192–206.CrossRefGoogle Scholar
  18. 18.
    Beauchamp G, Knepper P. Role of the neural crest in anterior segment development and disease. J Pediatr Ophthalmol Strabismus. 1984;21:209–14.PubMedGoogle Scholar
  19. 19.
    Glaser T, Walton DS, Maas RL. Genomic structure, evolutionary conservation and aniridia mutations in the human PAX6 gene. Nat Genet. 1992;2:232–9.CrossRefGoogle Scholar
  20. 20.
    Phillips JC, et al. A second locus for Rieger syndrome maps to chromosome 13q14. Am J Hum Genet. 1996;59:613–9.PubMedPubMedCentralGoogle Scholar
  21. 21.
    Dahl E, Koseki H, Balling R. Pax genes and organogenesis. BioEssays. 1997;19:755–65.CrossRefGoogle Scholar
  22. 22.
    Doward W, et al. A mutation in the RIEG1 gene associated with Peters’ anomaly. 4.Google Scholar
  23. 23.
    Iseri SU, et al. Seeing clearly: the dominant and recessive nature of FOXE3 in eye developmental anomalies. Hum Mutat. 2009;30:1378–86.CrossRefGoogle Scholar
  24. 24.
    Fitch N, Kaback M. The Axenfeld syndrome and the Rieger syndrome. J Med Genet. 1978;15:30–4.CrossRefGoogle Scholar
  25. 25.
    Chang TC, Summers CG, Schimmenti LA, Grajewski AL. Axenfeld-Rieger syndrome: new perspectives. Br J Ophthalmol. 2012;96:318–22.CrossRefGoogle Scholar
  26. 26.
    Bhandari R, Ferri S, Whittaker B, Liu M, Lazzaro DR. Peters anomaly: review of the literature. Cornea. 2011;30:939–44.CrossRefGoogle Scholar
  27. 27.
    Harissi-Dagher M, Colby K. Anterior segment dysgenesis: Peters anomaly and sclerocornea. Int Ophthalmol Clin. 2008;48:35–42.CrossRefGoogle Scholar
  28. 28.
    Nelson LB, Spaeth GL, Nowinski TS, Margo CE, Jackson L. Aniridia. A review. Surv Ophthalmol. 1984;28:621–42.CrossRefGoogle Scholar
  29. 29.
    Eden U, Riise R, Tornqvist K. Corneal involvement in congenital aniridia. Cornea. 2010;29:1096–102.CrossRefGoogle Scholar
  30. 30.
    Chang JW, Kim JH, Kim SJ, Yu YS. Congenital aniridia: long-term clinical course, visual outcome, and prognostic factors. Korean J Ophthalmol. 2010;28:479–85.CrossRefGoogle Scholar
  31. 31.
    Singh B, Mohamed A, Chaurasia S, Ramappa M, Mandal AK, Jalali S, Sangwan VS. Clinical manifestations of congenital aniridia. J Pediatr Ophthalmol Strabismus. 2014;51:59–62.CrossRefGoogle Scholar
  32. 32.
    Khokhar S, Sinha G, Sharma R, Patil B, Mahabir M, Nayak B. Anterior pyramidal Cataract. A rare association. JAMA Ophthalmol. 2015;133(4):e144626. Scholar
  33. 33.
    Farjo AA, Meyer RF, Farjo QA. Phacoemulsification in eyes with corneal opacification. J Cataract Refract Surg. 2003;29:242–5.CrossRefGoogle Scholar
  34. 34.
    Moore JE, Herath GD, Sharma A. Continuous curvilinear capsulorhexis with use of an endoscope. J Cataract Refract Surg. 2004;30:960–3.CrossRefGoogle Scholar
  35. 35.
    Al Sabti K, Raizada S, Al Abduljalil T. Cataract surgery assisted by anterior endoscopy. Br J Ophthalmol. 2009;93:531–4.CrossRefGoogle Scholar
  36. 36.
    Srinivasan S, Ting DS, Snyder ME, Prasad S, Koch HR. Prosthetic iris devices. Can J Ophthalmol. 2014;49(1):6–17.CrossRefGoogle Scholar
  37. 37.
    Mostafa YS, Osman AA, Hassanein DH, Zeid AM, Sherif AM. Iris reconstruction using artificial iris prosthesis for management of aniridia. Eur J Ophthalmol. 2018;28(1):103–7.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Sudarshan Kumar Khokhar
    • 1
  • Yogita Gupta
    • 1
  • Abhidnya Surve
    • 1
  • Chirakshi Dhull
    • 1
  1. 1.Dr. Rajendra Prasad Centre for Ophthalmic SciencesAll India Institute of Medical SciencesNew DelhiIndia

Personalised recommendations