Abstract
Purpose of Review
Aniridia is a complex ocular disorder that causes limbal stem cell deficiency determining progressive aniridic keratopathy and the loss of corneal transparency. Allogeneic limbal stem cell transplantation followed by penetrating keratoplasty can stabilize the ocular surface but requires prolonged systemic immunosuppression. The type-1 Boston Keratoprosthesis can be a valid alternative allowing faster visual recovery without the need for systemic immunosuppression.
Recent Findings
Several studies have reported good visual outcomes and good retention rates for keratoprosthesis in aniridic patients. Nonetheless, concerns still exist regarding complications that might lead to irreversible loss of function, such as glaucoma progression or intraocular infection. Other complications include retroprosthetic membrane formation, hypotony, and device extrusion. The presence of macular pathology and prevalence of glaucoma are two factors that may affect final visual acuity outcomes in aniridic patients.
Summary
Implantation of the type-1 Boston Keratoprosthesis for aniridic keratopathy has shown promising results. However, further studies are needed to provide more evidence regarding long-term survival and complications.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Nelson LB, Spaeth GL, Nowinski TS, Margo CE, Jackson L. Aniridia: a review. Surv Ophthalmol. 1984;28(6):621–42.
Gupta SK, De Becker I, Tremblay F, Guernsey DL, Neumann PE. Genotype/phenotype correlations in aniridia. Am J Ophthalmol. 1998;126(2):203–10.
Kokotas H, Petersen MB. Clinical and molecular aspects of aniridia. Clin Genet. 2010;77(5):409–20.
Bakhtiari P, Chan C, et al. Surgical and visual outcomes of the type I Boston Keratoprosthesis for the management of aniridic fibrosis syndrome in congenital aniridia. Am J Ophthalmol. 2012;153(5):967–71.
Miller SJ, Lavker RM, Sun TT. Keratinocyte stem cells of cornea, skin, and hair follicle. In: Potten C, editor. stem cells. New York: Academic Press; 1997. p. 331–62.
Muller LJ, Marfurt CF et al. [Erratum to: Muller LJ, Marfurt CF et al. Corneal nerves: structure, contents and function. Exp Eye Res 2003;76:521–42.
Jastaneiah S, Al Rajhi AA. Association of Aniridia and dry eyes. Ophthalmology. 2005;112:1535–40.
Kruse FE. Classification of ocular surface disease. In: Holland EJ, Mannis MJ, editors. Ocular surface disease: medical and surgical management. Springer: New York; 2002. p. 16–36.
Lopez-Garcia JS, Rivas L, et al. Autologous serum eyedrops in the treatment of aniridic keratopathy. Ophthalmology. 2008;115:262–7.
Nakamura T, Inatomi T, et al. Phenotypic investigation of human eyes with transplanted autologous cultivated oral mucosal epithelial sheets for severe ocular surface diseases. Ophthalmology. 2007;114:1080–8.
Nelson LB, Spaeth GL, et al. Aniridia: a review. Surv Ophthalmol. 1984;28:621–42.
Nishida K, Kinoshita S, et al. Ocular surface abnormalities in Aniridia. Am J Ophthalmol. 1995;120:368–75.
Rama P, Vigano M, Knutsson KA. The Ocular surface in aniridia. Aniridia: recent developments in scientific and clinical research. Switzerland: Springer; 2015. p. 45–52.
Tseng SC. Concept and application of limbal stem cells. Eye. 1989;3:141–57.
Edén U, Fagerholm P, et al. Pathologic epithelial and anterior corneal nerve morphology in early-stage congenital aniridic keratopathy. Ophthalmology. 2012;119(9):1803–10.
Collinson JM, Chanas SA, et al. Corneal development, limbal stem cell function, and corneal epithelial cell migration in the Pax6 (±) mouse. Invest Ophthalmol Vis Sci. 2004;45:1101–8.
Holland EJ, Djalilian AR, Scwartz GS. Management of aniridic keratopathy with keratolimbal allograft: a limbal stem cell transplantation technique. Ophthalmology. 2003;110:125–30.
Brandt JD, Casuso LA, Budenz DL. Markedly increased central corneal thickness and unrecognized finding in congenital Aniridia. Am J Ophthalmol. 2004;137:348–50.
Tiller AM, Odenthal MT, et al. The influence of keratoplasty on visual prognosis in aniridia: a historical review of one large family. Cornea. 2003;22(2):105–10.
Holland EJ, Schwartz GS. The evolution of epithelial transplantation for severe ocular surface disease and proposed classification system. Cornea. 1996;15:549–56.
Daya SM, Watson A, et al. Outcomes and DNA analysis of ex vivo expanded stem cell allograft for ocular surface reconstruction. Ophthalmology. 2005;112(3):470–7.
Henderson TR, Coster DJ, William KA. The long term outcome of limbal allografts: the search for surviving cells. Br J Ophthalmol. 2001;85:604–9.
Sharpe JR, Daya SM, et al. Survival of cultured allogeneic limbal epithelial cells following corneal repair. Tissue Eng. 2007;13(1):123–32.
Khan B, Dudenhoefer EJ, Dohlman CH. Keratoprosthesis: an update. Curr Opin Ophthalmol. 2001;12:282–7.
• Williamson S, Hsu K, De La Cruz J. Boston Kpro Type I as a viable alternative to visual rehabilitation in aniridia patients: advances and limitations. Aniridia: Recent developments in scientific and clinical research. Switzerland: Springer, 2015. pp. 75–83. The chapter of this book is very well written and summarizes the outcomes of the Boston Kpro Type 1 in aniridic patients. The authors of this text performed an extensive review of literature regarding the specific subject.
Akpek EK, Harissi-Dagher M, Petrarca R, et al. Outcomes of Boston Keratoprosthesis in aniridia: a retrospective multicenter study. Am J Ophthalmol. 2007;144(2):270–6.
Rixen JJ, Cohen AW, Kitzmann AS, Wagoner MD, Goins KM. Treatment of aniridia with Boston type I keratoprosthesis. Cornea. 2013;32(7):947–50.
•• Hassanaly SI, Talajic JC, Harissi-Dagher M. Outcomes following Boston type 1 keratoprosthesis implantation in aniridia patients at the University of Montreal. Am J Ophthalmol. 2014;158(2): 270–6. This retrospective study describes the outcomes of KPro Type 1 for aniridic keratopathy in a single center with all procedures performed by the same surgeon. It contains the largest cohort of eyes present in literature(n = 26) with a mean follow up of 28.7 months.
Kang JJ, de la Cruz J, Cortina MS. Visual outcomes of Boston keratoprosthesis implantation as the primary penetrating corneal procedure. Cornea. 2012;31(12):1436–40.
Robert MC, Harissi-Dagher M. Boston type 1 keratoprosthesis: the CHUM experience. Can J Ophthalmol. 2011;46(2):164–8.
Patel AP, Wu EI, Ritterbrand DC, Seedor JA. Boston type 1 keratoprosthesis: the New York Eye and Ear experience. Eye. 2010;26(3):418–25.
Srikumaran D, Munoz B, Aldave AJ, et al. Long-term outcomes of boston type 1 keratoprosthesis implantation: a retrospective multicenter cohort. Ophthalmology. 2014;121(11):2159–64.
Greiner MA, Li JY, Mannis MJ. Longer-term vision outcomes and complications with thee Boston type 1 keratoprosthesis at the University of California Davis. Ophthalmology. 2011;118(8):1543–50.
• Rudnisky CJ, Belin MW et al. Visual acuity outcomes of the Boston keratoprosthesis type 1: Multicenter study results. Am. J. Ophthalmol. 2016;162:89–98. This recent publication analyzes the visual acuity outcomes of the Boston KPro type 1 for different indications. Visual rehabilitation in aniridia is lower compared to other subgroups most likely due to the presence of foveal hypoplasia.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
Karl Anders Knutsson and Paolo Rama declare no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Additional information
This article is part of the Topical Collection on Ocular Prosthesis.
Rights and permissions
About this article
Cite this article
Knutsson, K.A., Rama, P. Aniridia and Boston KPro: Is It a Long-Term Option?. Curr Ophthalmol Rep 4, 154–158 (2016). https://doi.org/10.1007/s40135-016-0105-0
Published:
Issue Date:
DOI: https://doi.org/10.1007/s40135-016-0105-0