Skip to main content

Advertisement

SpringerLink
Log in

Macular structural characteristics in children with Down syndrome

  • Pediatrics
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

This prospective study aimed to investigate macular structural characteristics in children with Down syndrome compared to those in healthy children.

Methods

Two groups of children (aged 6–16 years) were enrolled: children with Down syndrome (Down syndrome group, N = 17) and age-matched healthy children who were full-term at birth (control group, N = 18). Eligible patients had visual acuity of 20/100 or better and gestational age at birth of ≥ 36 weeks. Fourier domain optical coherence tomography was used for imaging of the macular retinal structure, and retinal volume scans centered on the macula were obtained. Central subfield thickness (CST) and the thickness of the inner and outer retinal layer regions were analyzed using the instrument’s segmentation software. The analysis of data is provided for the right eye only, since there was no significant difference between right and left eyes for either the Down syndrome or control groups.

Results

Children in the Down syndrome group generally had identifiable retinal structure. The CST for the full retina and inner and outer retinal layers were all significantly greater in the Down syndrome group than the control group (independent t test, all p < 0.05). Despite the significantly thicker macula, only about 29 % (5 of 17) of the right eyes of patients with Down syndrome had macular thickness outside the normal range. Visual acuity in the Down syndrome group was not directly correlated with increased CST (t = 1.288, r = 0.326, p = 0.202).

Conclusions

On average, CST in the Down syndrome group was greater than that in the control group, suggesting abnormal macular development in children with Down syndrome.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Cregg M, Woodhouse JM, Stewart RE, Pakeman VH, Bromham NR, Gunter HL, Trojanowska L, Parker M, Fraser WI (2003) Development of refractive error and strabismus in children with Down syndrome. Invest Ophthalmol Vis Sci 44(3):1023–1030

    Article  PubMed  Google Scholar 

  2. Little JA, Woodhouse JM, Lauritzen JS, Saunders KJ (2007) The impact of optical factors on resolution acuity in children with Down syndrome. Invest Ophthalmol Vis Sci 48(9):3995–4001. doi:10.1167/iovs.06-1387

    Article  PubMed  Google Scholar 

  3. Ljubic A, Trajkovski V, Stankovic B (2011) Strabismus, refractive errors and nystagmus in children and young adults with Down syndrome. Ophthalmic Genet 32(4):204–211. doi:10.3109/13816810.2011.592175

    Article  PubMed  Google Scholar 

  4. McCullough SJ, Little JA, Saunders KJ (2013) Higher order aberrations in children with Down syndrome. Invest Ophthalmol Vis Sci 54(2):1527–1535. doi:10.1167/iovs.12-10597

    Article  PubMed  Google Scholar 

  5. Woodhouse JM, Pakeman VH, Cregg M, Saunders KJ, Parker M, Fraser WI, Sastry P, Lobo S (1997) Refractive errors in young children with Down syndrome. Optom Vis Sci 74(10):844–851

    Article  CAS  PubMed  Google Scholar 

  6. Cregg M, Woodhouse JM, Pakeman VH, Saunders KJ, Gunter HL, Parker M, Fraser WI, Sastry P (2001) Accommodation and refractive error in children with Down syndrome: cross-sectional and longitudinal studies. Invest Ophthalmol Vis Sci 42(1):55–63

    CAS  PubMed  Google Scholar 

  7. Felius J, Beauchamp CL, Stager DR Sr (2014) Visual acuity deficits in children with nystagmus and Down syndrome. Am J Ophthalmol 157(2):458–463. doi:10.1016/j.ajo.2013.09.023

    Article  PubMed  Google Scholar 

  8. John FM, Bromham NR, Woodhouse JM, Candy TR (2004) Spatial vision deficits in infants and children with Down syndrome. Invest Ophthalmol Vis Sci 45(5):1566–1572

    Article  PubMed  Google Scholar 

  9. Little JA, McCullough S, McClelland J, Jackson AJ, Saunders KJ (2013) Low-contrast acuity measurement: does it add value in the visual assessment of down syndrome and cerebral palsy populations? Invest Ophthalmol Vis Sci 54(1):251–257. doi:10.1167/iovs.12-10506

    Article  PubMed  Google Scholar 

  10. Little JA, Woodhouse JM, Lauritzen JS, Saunders KJ (2009) Vernier acuity in Down syndrome. Invest Ophthalmol Vis Sci 50(2):567–572. doi:10.1167/iovs.08-2250

    Article  PubMed  Google Scholar 

  11. Paudel N, Leat SJ, Adhikari P, Woodhouse JM, Shrestha JB (2010) Visual defects in Nepalese children with Down syndrome. Clin Exp Optom 93(2):83–90. doi:10.1111/j.1444-0938.2010.00458.x

    Article  PubMed  Google Scholar 

  12. Woodhouse JM, Meades JS, Leat SJ, Saunders KJ (1993) Reduced accommodation in children with Down syndrome. Invest Ophthalmol Vis Sci 34(7):2382–2387

    CAS  PubMed  Google Scholar 

  13. Provis JM, Hendrickson AE (2008) The foveal avascular region of developing human retina. Arch Ophthalmol 126(4):507–511. doi:10.1001/archopht.126.4.507

    Article  CAS  PubMed  Google Scholar 

  14. Springer AD, Hendrickson AE (2004) Development of the primate area of high acuity. 2. Quantitative morphological changes associated with retinal and pars plana growth. Vis Neurosci 21(5):775–790. doi:10.1017/S0952523804215115

    Article  CAS  PubMed  Google Scholar 

  15. Wang J, Spencer R, Leffler JN, Birch EE (2012) Critical period for foveal fine structure in children with regressed retinopathy of prematurity. Retina 32(2):330–339. doi:10.1097/IAE.0b013e318219e685

    Article  CAS  PubMed  Google Scholar 

  16. Yuodelis C, Hendrickson A (1986) A qualitative and quantitative analysis of the human fovea during development. Vis Res 26(6):847–855

    Article  CAS  PubMed  Google Scholar 

  17. Candy TR, Crowell JA, Banks MS (1998) Optical, receptoral, and retinal constraints on foveal and peripheral vision in the human neonate. Vis Res 38(24):3857–3870

    Article  CAS  PubMed  Google Scholar 

  18. Wallman J, Winawer J (2004) Homeostasis of eye growth and the question of myopia. Neuron 43(4):447–468

    Article  CAS  PubMed  Google Scholar 

  19. Huynh SC, Samarawickrama C, Wang XY, Rochtchina E, Wong TY, Gole GA, Rose KA, Mitchell P (2009) Macular and nerve fiber layer thickness in Amblyopia: the Sydney childhood Eye study. Ophthalmology 116(9):1604–1609. doi:10.1016/j.ophtha.2009.03.013

    Article  PubMed  Google Scholar 

  20. Altintas O, Yuksel N, Ozkan B, Caglar Y (2005) Thickness of the retinal nerve fiber layer, macular thickness, and macular volume in patients with strabismic amblyopia. J Pediatr Ophthalmol Strabismus 42(4):216–221

    PubMed  Google Scholar 

  21. Al-Haddad CE, El Mollayess GM, Mahfoud ZR, Jaafar DF, Bashshur ZF (2013) Macular ultrastructural features in amblyopia using high-definition optical coherence tomography. Br J Ophthalmol 97(3):318–322. doi:10.1136/bjophthalmol-2012-302434

    Article  PubMed  Google Scholar 

  22. Wang BZ, Taranath D (2012) A comparison between the amblyopic eye and normal fellow eye ocular architecture in children with hyperopic anisometropic amblyopia. J AAPOS 16(5):428–430. doi:10.1016/j.jaapos.2012.06.006

    Article  PubMed  Google Scholar 

  23. Pang Y, Goodfellow GW, Allison C, Block S, Frantz KA (2011) A prospective study of macular thickness in amblyopic children with unilateral high myopia. Invest Ophthalmol Vis Sci 52(5):2444–2449. doi:10.1167/iovs.10-5550

    Article  PubMed  Google Scholar 

  24. Yoon SW, Park WH, Baek SH, Kong SM (2005) Thicknesses of macular retinal layer and peripapillary retinal nerve fiber layer in patients with hyperopic anisometropic amblyopia. Korean J Ophthalmol 19(1):62–67

    Article  PubMed  Google Scholar 

  25. Aziz HA, Ruggeri M, Berrocal AM (2011) Intraoperative OCT of bilateral macular coloboma in a child with Down syndrome. J Pediatr Ophthalmol Strabismus 48:e37–39. doi:10.3928/01913913-20110712-03

    PubMed  Google Scholar 

  26. Al-Hemidan AI, Al-Hazzaa SA, Chavis P, Al-Hussein H (1999) Optic disc elevation in Down syndrome. Ophthalmic Genet 20(1):45–51

    Article  CAS  PubMed  Google Scholar 

  27. Altun A, Altun G, Kurna SA, Olcaysu OO, Aki SF (2014) Unilateral morning glory optic disc anomaly in a case with Down syndrome. BMC Ophthalmol 14:48. doi:10.1186/1471-2415-14-48

    Article  PubMed Central  PubMed  Google Scholar 

  28. Parsa CF, Almer Z (2008) Supranumerary optic disc vessels may indicate reduced systemic angiogenesis in Down syndrome. Br J Ophthalmol 92(3):432–433. doi:10.1136/bjo.2007.124248

    Article  CAS  PubMed  Google Scholar 

  29. Stirn Kranjc B (2012) Ocular abnormalities and systemic disease in Down syndrome. Strabismus 20(2):74–77. doi:10.3109/09273972.2012.680234

    Article  PubMed  Google Scholar 

  30. Fimiani F, Iovine A, Carelli R, Pansini M, Sebastio G, Magli A (2007) Incidence of ocular pathologies in Italian children with Down syndrome. Eur J Ophthalmol 17(5):817–822

    CAS  PubMed  Google Scholar 

  31. Bambo MP, Garcia-Martin E, Otin S, Sancho E, Fuertes I, Herrero R, Satue M, Pablo L (2014) Influence of cataract surgery on repeatability and measurements of spectral domain optical coherence tomography. Br J Ophthalmol 98 (1):52–58. doi:10.1136/bjophthalmol-2013-303752

  32. Comyn O, Heng LZ, Ikeji F, Bibi K, Hykin PG, Bainbridge JW, Patel PJ (2012) Repeatability of Spectralis OCT measurements of macular thickness and volume in diabetic macular edema. Invest Ophthalmol Vis Sci 53 (12):7754–7759. doi:10.1167/iovs.12-10895

  33. Wu WC, Lin RI, Shih CP, Wang NK, Chen YP, Chao AN, Chen KJ, Chen TL, Hwang YS, Lai CC, Huang CY, Tsai S (2012) Visual acuity, optical components, and macular abnormalities in patients with a history of retinopathy of prematurity. Ophthalmology 119(9):1907–1916. doi:10.1016/j.ophtha.2012.02.040

    Article  PubMed  Google Scholar 

  34. Marmor MF, Choi SS, Zawadzki RJ, Werner JS (2008) Visual insignificance of the foveal pit: reassessment of foveal hypoplasia as fovea plana. Arch Ophthalmol 126(7):907–913. doi:10.1001/archopht.126.7.907

    Article  PubMed Central  PubMed  Google Scholar 

  35. Recchia FM, Recchia CC (2007) Foveal dysplasia evident by optical coherence tomography in patients with a history of retinopathy of prematurity. Retina 27(9):1221–1226. doi:10.1097/IAE.0b013e318068de2e

    Article  PubMed  Google Scholar 

  36. Meyer CH, Lapolice DJ, Freedman SF (2002) Foveal hypoplasia in oculocutaneous albinism demonstrated by optical coherence tomography. Am J Ophthalmol 133(3):409–410

    Article  PubMed  Google Scholar 

  37. Wang J, Smith HA, Donaldson DL, Haider KM, Roberts GJ, Sprunger DT, Neely DE, Plager DA (2014) Macular structural characteristics in children with congenital and developmental cataracts. J AAPOS. doi:10.1016/j.jaapos.2014.05.008

    Google Scholar 

  38. Laguna A, Barallobre MJ, Marchena MA, Mateus C, Ramirez E, Martinez-Cue C, Delabar JM, Castelo-Branco M, de la Villa P, Arbones ML (2013) Triplication of DYRK1A causes retinal structural and functional alterations in Down syndrome. Hum Mol Genet 22(14):2775–2784. doi:10.1093/hmg/ddt125

    Article  CAS  PubMed  Google Scholar 

  39. Odell D, Dubis AM, Lever JF, Stepien KE, Carroll J (2011) Assessing Errors Inherent in OCT-Derived Macular Thickness Maps. J Ophthalmol 2011:692574. doi:10.1155/2011/692574

    PubMed Central  PubMed  Google Scholar 

  40. Doyle SJ, Bullock J, Gray C, Spencer A, Cunningham C (1998) Emmetropisation, axial length, and corneal topography in teenagers with Down’s syndrome. Br J Ophthalmol 82(7):793–796

    Article  PubMed Central  CAS  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Afiah Hasnie, Alexander Walls, Jessica Felker and Paxton Ott for helping obtain patient data.

Conflict of interest

All authors certify that they have NO affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Financial support

This study is supported by an Indiana University–Purdue University Indianapolis (IUPUI) EMPOWER [Enhanced Mentoring Program with Opportunities for Ways to Excel in Research] award to Dr. Wang and a Research to Prevent Blindness (RPB) unrestricted grant to the Glick Eye Institute.

Author information

Authors and Affiliations

  1. Eugene and Marilyn Glick Eye Institute, Department of Ophthalmology, Indiana University School of Medicine, 1160 W. Michigan St., Indianapolis, IN, 46202, USA

    Scott O’Brien, Jingyun Wang, Heather A. Smith, Dana L. Donaldson, Kathryn M. Haider, Gavin J. Roberts, Derek T. Sprunger, Daniel E. Neely & David A. Plager

Authors
  1. Scott O’Brien
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jingyun Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Heather A. Smith
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Dana L. Donaldson
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kathryn M. Haider
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Gavin J. Roberts
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Derek T. Sprunger
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Daniel E. Neely
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. David A. Plager
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jingyun Wang.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

O’Brien, S., Wang, J., Smith, H.A. et al. Macular structural characteristics in children with Down syndrome. Graefes Arch Clin Exp Ophthalmol 253, 2317–2323 (2015). https://doi.org/10.1007/s00417-015-3088-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00417-015-3088-x

Keywords

Search

Navigation