Skip to main content

Advertisement

Log in

Keratoconus detection by novel indices in patients with Down syndrome: a cohort population-based study

  • Clinical Investigation
  • Published:
Japanese Journal of Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To use novel indices to determine the prevalence of KC and its progression in patients aged 10–30 years with Down syndrome.

Study design

Cohort population-based study.

Methods

Two hundred twenty-six of 250 invited Down syndrome patients were enrolled. The diagnostic criteria were confirmed by two independent expert examiners using slit-lamp examinations and topographic indices measured by Pentacam HR (Oculus Optikgeräte): maximum keratometry centered on the steepest point (zonal Kmax-3 mm), Ambrósio's relational thickness (ART), inferior-superior asymmetry (IS-value), Belin/Ambrósio deviation value (BAD-D), the Tomographic and Biomechanical Index (TBI), and a posterior elevation map. In the KC cases, Corvis ST (Oculus Optikgeräte) was done. All the KC cases completed the second phase in 2017.

Results

KC was identified in 28 patients (12.39%; 95% confidence interval: 8.2–17.9%): 20 bilateral and eight unilateral cases. Of these, 24 were in the ≤ 20-years age group, and four, in the > 20-years age group. The frequency of KC was not significantly correlated with age (P = 0.804) or gender (P = 0.322). In the KC cases, the mean zonal Kmax-3 mm, ART-max, IS-value, BAD-D, CBI, and TBI were 50.40 ± 5.88 D, 321.63 ± 111.94 μm, 1.99 ± 2.51, 3.73 ± 3.12, 0.54 ± 0.61, and 0.86 ± 0.20, respectively, and the minimum corneal thickness was 492.17 ± 42.67 μm. Of the 28 patients, 39.6% showed progression, and all were in the ≤ 20-years age group.

Conclusion

The prevalence of KC in Down syndrome patients is significantly higher than that reported in non-Down syndrome individuals of the same age groups. The progression rate is approximately similar to that of the non-Down syndrome population. Screening programs should be applied to prohibit serious visual impairment in these populations.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Hedayatfar A, Hashemi H, Aghaei H, Ashraf N, Asgari S. Subclinical inflammatory response: accelerated versus standard corneal cross-linking. Ocul Immunol Inflamm. 2019;27:513–6. https://doi.org/10.1080/09273948.2017.1420201.

    Article  PubMed  Google Scholar 

  2. Rabinowitz YS. Keratoconus. Surv Ophthalmol. 1998;42:297–319.

    Article  CAS  Google Scholar 

  3. Torres Netto EA, Al-Otaibi WM, Hafezi NL, Kling S, Al-Farhan HM, Randleman JB, et al. Prevalence of keratoconus in paediatric patients in Riyadh, Saudi Arabia. Br J Ophthalmol. 2018;102:1436–41.

    Article  Google Scholar 

  4. Hashemi H, Khabazkhoob M, Fotouhi A. Topographic keratoconus is not rare in an Iranian population: the Tehran Eye Study. Ophthalmic Epidemiol. 2013;20:385–91.

    Article  Google Scholar 

  5. Wollensak G. Crosslinking treatment of progressive keratoconus: new hope. Curr Opin Ophthalmol. 2006;17:356–60.

    Article  Google Scholar 

  6. Naderan M, Rajabi MT, Zarrinbakhsh P, Farjadnia M. Is keratoconus more severe in pediatric population? Int Ophthalmol. 2017;37:1169–73.

    Article  Google Scholar 

  7. Léoni-Mesplié S, Mortemousque B, Mesplié N, Touboul D, Praud D, Malet F, et al. Epidemiological aspects of keratoconus in children [in French]. J Fr Ophtalmol. 2012;35:776–85.

    Article  Google Scholar 

  8. Saee-Rad S, Raoofian R, Mahbod M, Miraftab M, Mojarrad M, Asgari S, et al. Analysis of superoxide dismutase 1, dual-specificity phosphatase 1, and transforming growth factor, beta 1 genes expression in keratoconic and non-keratoconic corneas. Mol Vis. 2013;19:2501–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  9. Udar N, Atilano SR, Brown DJ, Holguin B, Small K, Nesburn AB, et al. SOD1: a candidate gene for keratoconus. Invest Ophthalmol Vis Sci. 2006;47:3345–51.

    Article  Google Scholar 

  10. Aslan L, Aslankurt M, Yüksel E, Özdemir M, Aksakal E, Gümüşalan Y, et al. Corneal thickness measured by Scheimpflug imaging in children with Down syndrome. J AAPOS. 2013;17:149–52.

    Article  Google Scholar 

  11. Gore DM, Watson MP, Tuft SJ. Permanent visual loss in eyes with keratoconus. Acta Ophthalmol. 2014;92:e244–e245245.

    Article  Google Scholar 

  12. Shajari M, Jaffary I, Herrmann K, Grunwald C, Steinwender G, Mayer WJ, et al. Early tomographic changes in the eyes of patients with keratoconus. J Refract Surg. 2018;34:254–9.

    Article  Google Scholar 

  13. Ambrósio R Jr, Caiado AL, Guerra FP, Louzada R, Sinha RA, Luz A, et al. Novel pachymetric parameters based on corneal tomography for diagnosing keratoconus. J Refract Surg. 2011;27:753–8.

    Article  Google Scholar 

  14. Vinciguerra R, Elsheikh A, Roberts CJ, Ambrósio R Jr, Kang DS, Lopes BT, et al. Influence of pachymetry and intraocular pressure on dynamic corneal response parameters in healthy patients. J Refract Surg. 2016;32:550–61.

    Article  Google Scholar 

  15. Roberts CJ, Mahmoud AM, Bons JP, Hossain A, Elsheikh A, Vinciguerra R, et al. Introduction of two novel stiffness parameters and interpretation of air puff-induced biomechanical deformation parameters with a dynamic Scheimpflug analyzer. J Refract Surg. 2017;33:266–73.

    Article  Google Scholar 

  16. Rabinowitz YS, Rasheed K. KISA% index: a quantitative videokeratography algorithm embodying minimal keratometric criteria for diagnosing keratoconus. J Cataract Refract Surg. 1999;25:1327–35.

    Article  CAS  Google Scholar 

  17. Burns DM, Johnston FM, Frazer DG, Patterson C, Jackson AJ. Keratoconus: an analysis of corneal asymmetry. Br J Ophthalmol. 2004;88:1252–5.

    Article  CAS  Google Scholar 

  18. Villavicencio OF, Gilani F, Henriquez MA, Izquierdo L Jr, Ambrósio RR Jr, Belin MW. Independent population validation of the Belin/Ambrosio enhanced ectasia display: implications for keratoconus studies and screening. Int J Kerato Ectatic Corneal Dis. 2014;3:1–8.

    Article  Google Scholar 

  19. Vinciguerra R, Ambrósio R Jr, Elsheikh A, Roberts CJ, Lopes B, Morenghi E, et al. Detection of keratoconus with a new biomechanical index. J Refract Surg. 2016;32:803–10.

    Article  Google Scholar 

  20. Ambrósio R Jr, Lopes BT, Faria-Correia F, Salomão MQ, Bühren J, Roberts CJ, et al. Integration of Scheimpflug-based corneal tomography and biomechanical assessments for enhancing ectasia detection. J Refract Surg. 2017;33:434–43.

    Article  Google Scholar 

  21. Kosekahya P, Caglayan M, Koc M, Kiziltoprak H, Tekin K, Atilgan CU. Longitudinal evaluation of the progression of keratoconus using a novel progression display. Eye Contact Lens. 2019;45:324–30. https://doi.org/10.1097/ICL.0000000000000582.

    Article  PubMed  Google Scholar 

  22. Haugen OH, Hovding G, Eide GE. Biometric measurements of the eyes in teenagers and young adults with Down syndrome. Acta Ophthalmol Scand. 2001;79:616–25.

    Article  CAS  Google Scholar 

  23. Shapiro MB, France TD. The ocular features of Down's syndrome. Am J Ophthalmol. 1985;99:659–63.

    Article  CAS  Google Scholar 

  24. Liza-Sharmini AT, Azlan ZN, Zilfalil BA. Ocular findings in Malaysian children with Down syndrome. Singap Med J. 2006;47:14–9.

    CAS  Google Scholar 

  25. Fimiani F, Iovine A, Carelli R, Pansini M, Sebastio G, Magli A. Incidence of ocular pathologies in Italian children with Down syndrome. Eur J Ophthalmol. 2007;17:817–22.

    Article  CAS  Google Scholar 

  26. Shehadeh MM, Diakonis VF, Jalil SA, Younis R, Qadoumi J, Al-Labadi L. Prevalence of keratoconus among a Palestinian tertiary student population. Open Ophthalmol J. 2015;9:172–6.

    Article  CAS  Google Scholar 

  27. Godefrooij DA, de Wit GA, Uiterwaal CS, Imhof SM, Wisse RP. Age-specific incidence and prevalence of keratoconus: a nationwide registration study. Am J Ophthalmol. 2017;175:169–72.

    Article  Google Scholar 

  28. Pearson AR, Soneji B, Sarvananthan N, Sandford-Smith JH. Does ethnic origin influence the incidence or severity of keratoconus? Eye (Lond). 2000;14:625–8.

    Article  Google Scholar 

  29. Baghdasaryan E, Huang X, Marion KM, Tepelus TC, Bagherinia H, Sadda SR, et al. Reproducibility of central corneal thickness measurements in normal eyes using the Zeiss Cirrus 5000 HD-OCT and Pentacam HR. Open Ophthalmol J. 2018;12:72–83.

    Article  Google Scholar 

  30. Kosekahya P, Koc M, Caglayan M, Kiziltoprak H, Atilgan CU, Yilmazbas P. Repeatability and reliability of ectasia display and topometric indices with the Scheimpflug system in normal and keratoconic eyes. J Cataract Refract Surg. 2018;44:63–70.

    Article  Google Scholar 

  31. de Luis EB, Escudero Argaluza J, Pijoan Zubizarreta JI, Santamaria Carro A, Etxebarria EJ. Evaluation of the reliability and repeatability of Scheimpflug system measurement in keratoconus. Cornea. 2018;37:177–81.

    Article  Google Scholar 

  32. Alio JL, Vega-Estrada A, Sanz P, Osman AA, Kamal AM, Mamoon A, et al. Corneal morphologic characteristics in patients with Down Syndrome. JAMA Ophthalmol. 2018;136:971–8.

    Article  Google Scholar 

  33. Choi JA, Kim MS. Progression of keratoconus by longitudinal assessment with corneal topography. Invest Ophthalmol Vis Sci. 2012;53:927–35.

    Article  Google Scholar 

  34. Ferdi AC, Nguyen V, Gore DM, Allan BD, Rozema JJ, Watson SL. Keratoconus natural progression: a systematic review and meta-analysis of 11529 eyes. Ophthalmology. 2019;126:935–45. https://doi.org/10.1016/j.ophtha.2019.02.029.

    Article  PubMed  Google Scholar 

  35. Asgari S, Mehravaran S, Fotouhi A, Makateb A, Hashemi H. Total corneal refractive power and shape in Down syndrome. Eur J Ophthalmol. 2019. https://doi.org/10.1177/1120672119883594.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Shiva Fayyaz who extracted the data and Dr Shiva Mehravaran who assisted us in the preparation of the manuscript.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Soheila Asgari.

Ethics declarations

Conflicts of interest

H. Hashemi, None; M. Miraftab, None, K. Amanzadeh, None; M. A. Seyedian, None; R. Vinciguerra, Consultant fee (Oculus); R. Ambrósio Jr, Consultant fee (Oculus); C. Roberts, Consultant fee (Oculus, Ziemer); A. Makateb, None; P. Vinciguerra, Consultant fee (Oculus, Schwind); S. Asgari, None.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Corresponding Author: Soheila Asgari

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hashemi, H., Miraftab, M., Amanzadeh, K. et al. Keratoconus detection by novel indices in patients with Down syndrome: a cohort population-based study. Jpn J Ophthalmol 64, 285–291 (2020). https://doi.org/10.1007/s10384-020-00725-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10384-020-00725-4

Keywords

Navigation