Skip to main content

Boston Kpro Type I as a Viable Alternative to Visual Rehabilitation in Aniridia Patients: Advances and Limitations

  • Chapter
Aniridia

Abstract

The purpose of this chapter is to describe the experience of Boston Type 1 Keratoprosthesis in Aniridia. Aniridia-associated keratopathy (AAK) affects up to 90 % of aniridic patients and may significantly decrease best-corrected acuity. Poor outcomes of penetrating keratoplasty in aniridia have been reported for over 20 years. Limbal stem cell transplantation can help restore a healthy ocular surface, but requires long term systemic immunosuppression. Boston type 1 Keratoprosthesis implantation represents a promising alternative for visual rehabilitation in AAK patients. The central PMMA optic is unaffected by corneal graft vascularization or conjunctivalization, and may be customized for aphakia or pseudophakia. Multiple studies have reported improved visual outcomes after Kpro implantation with device retention rates of 70 % or greater. Complications include retroprosthetic membranes, corneal melt, device extrusion, and glaucoma.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
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

References

  1. Lee H, Khan R, O’Keefe M (2008) Aniridia: current pathology and management. Acta Ophthalmol 86:708–715

    Article  CAS  PubMed  Google Scholar 

  2. Ramaesh K, Ramaesh T, Dutton GN, Dhillon B (2005) Evolving concepts on the pathogenic mechanisms of aniridia related keratopathy. Int J Biochem Cell Biol 37:547–557

    Article  CAS  PubMed  Google Scholar 

  3. Gomes J, Eagle R, Gomes A, Rapuano C, Cohen E, Laibson P (1996) Recurrent keratopathy after penetrating keratoplasty for aniridia. Cornea 15:457–462

    Article  CAS  PubMed  Google Scholar 

  4. Holland E, Djalilian A, Schwartz G (2003) Management of aniridic keratopathy with keratolimbal allograft: a limbal stem cell transplantation technique. Ophthalmology 110:125–130

    Article  PubMed  Google Scholar 

  5. Edén U, Fagerholm P, Danyali R, Lagali N (2012) Pathologic epithelial and anterior corneal nerve morphology in early-stage congenital aniridic keratopathy. Ophthalmology 119:1803–1810

    Article  PubMed  Google Scholar 

  6. Lopez-Garcia J, Garcia-Lozano I, Rivas L, Martinez-Garchitorena J (2006) Congenital aniridia keratopathy treatment. Arch Soc Esp Oftalmol 81:435–444

    CAS  PubMed  Google Scholar 

  7. Kremer I, Rajpal RK, Rapuano CJ, Cohen EJ, Laibson PR (1993) Results of penetrating keratoplaty in aniridia. Am J Ophthalmol 115:317–320

    Article  CAS  PubMed  Google Scholar 

  8. Tiller AM, Odenthal MT, Verbraak FD, Gortzak-Moorstein N (2003) The influence of keratoplasty on visual prognosis in aniridia: a historical review of one large family. Cornea 22:105–110

    Article  PubMed  Google Scholar 

  9. Hou JH, de la Cruz J, Djalilian AR (2012) Outcomes of Boston keratoprosthesis implantation for failed keratoplasty after keratolimbal allograft. Cornea 31(12):1432–1435

    Article  PubMed  Google Scholar 

  10. Solomon A, Ellies P, Anderson DF, Touhami A, Grueterich M, Espana EM, Ti SE, Goto E, Feuer WJ, Tseng SC (2002) Long-term outcome of keratolimbal allograft with or without penetrating keratoplasty for total limbal stem cell deficiency. Ophthalmology 109:1159–1166

    Article  PubMed  Google Scholar 

  11. Tseng S, Prabhasawat P, Barton K, Gray T, Meller D (1998) Amniotic membrane transplantation with or without limbal allografts for corneal surface reconstruction in patients with limbal stem cell deficiency. Arch Ophthalmol 16(4):431–441

    Article  Google Scholar 

  12. Shimazaki J, Maruyama F, Shimmura S, Fujishima H, Tsubota K (2001) Immunologic rejection of the central graft after limbal allograft transplantation combined with penetrating keratoplasty. Cornea 20:149–152

    Article  CAS  PubMed  Google Scholar 

  13. Ilari L, Daya SM (2002) Long-term outcomes of keratolimbal allograft for the treatment of severe ocular surface disorders. Ophthalmology 109:1278–1284

    Article  PubMed  Google Scholar 

  14. Dohlman CH, Harissi-Dagher M, Khan BF, Sippel K, Aquavella JV, Graney JM (2006) Introduction to the use of the Boston keratoprosthesis. Expert Rev Ophthalmol 1:41–48

    Article  Google Scholar 

  15. Akpek EK, Harissi-Dagher M, Petrarca R, Butrus SI, Pineda R, Aquavella JV, Dohlman CH (2007) Outcomes of Boston keratoprosthesis in aniridia: a retrospective multicenter study. Am J Ophthalmol 144(2):227–231

    Article  PubMed  Google Scholar 

  16. Rixen JJ, Cohen AW, Kitzmann AS, Wagoner MD, Goins KM (2013) Treatment of aniridia with Boston type I keratoprosthesis. Cornea 32(7):947–950

    Article  PubMed  Google Scholar 

  17. Hassanaly SI, Talajic JC, Harissi-Dagher M (2014) Outcomes following Boston type 1 keratoprosthesis implantation in aniridia patients at the University of Montreal. Am J Ophthalmol 158(2):270–276

    Article  PubMed  Google Scholar 

  18. Greiner MA, Li JY, Mannis MJ (2011) Longer-term vision outcomes and complications with the Boston type 1 keratoprosthesis at the University of California, Davis. Ophthalmology 118(8):1543–1550

    Article  PubMed  Google Scholar 

  19. Kang JJ, de la Cruz J, Cortina MS (2012) Visual outcomes of Boston keratoprosthesis implantation as the primary penetrating corneal procedure. Cornea 31(12):1436–1440

    Article  PubMed  Google Scholar 

  20. Patel AP, Wu EI, Ritterband DC, Seedor JA (2012) Boston type 1 keratoprosthesis: the New York eye and ear experience. Eye 26(3):418–425

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  21. Robert MC, Harissi-Dagher M (2011) Boston type 1 keratoprosthesis: the CHUM experience. Can J Ophthalmol 46(2):164–168

    Article  PubMed  Google Scholar 

  22. Srikumaran D, Munoz B, Aldave AJ, Aquavella JV, Hannush SB, Schultze R, Belin M, Akpek EK (2014) Long-term outcomes of Boston type 1 keratoprosthesis implantation: a retrospective multicenter cohort. Ophthalmology. doi:10.1016/j.ophtha.2014.05.030

    PubMed  Google Scholar 

  23. Sivaraman KR, Hou JH, Allemann N, de la Cruz J, Cortina MS (2013) Retroprosthetic membrane and risk of sterile keratolysis in patients with type I Boston keratoprosthesis. Am J Ophthalmol 155(5):814–822

    Article  PubMed  Google Scholar 

  24. Sonsino J, Ewald MD (2010) Corrective options for irregular corneas. CL Spectr 180:26

    Google Scholar 

  25. Sayegh RR, Avena Diaz L, Vargas-Martín F, Webb RH, Dohlman CH, Peli E (2010) Optical functional properties of the Boston keratoprosthesis. Invest Ophthalmol Vis Sci 51(2):857–863

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Samantha Williamson M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Williamson, S., Hsu, K., de la Cruz, J. (2015). Boston Kpro Type I as a Viable Alternative to Visual Rehabilitation in Aniridia Patients: Advances and Limitations. In: Parekh, M., Poli, B., Ferrari, S., Teofili, C., Ponzin, D. (eds) Aniridia. Springer, Cham. https://doi.org/10.1007/978-3-319-19779-1_9

Download citation

Publish with us

Policies and ethics