Japanese Journal of Ophthalmology

, Volume 63, Issue 1, pp 34–39 | Cite as

Clinical features of superior segmental optic hypoplasia: hospital-based study

  • Ayaka YagasakiEmail author
  • Akira Sawada
  • Yusuke Manabe
  • Tetsuya Yamamoto
Clinical Investigation



To determine the clinical features of patients diagnosed with superior segmental optic hypoplasia (SSOH) and to quantitatively compare retinal nerve fiber layer (RNFL) thickness in SSOH eyes, to that in normal subjects.

Study design

Retrospective comparative case series.


We examined the medical charts of 106 eyes of 59 patients with SSOH and 35 eyes of 35 normal subjects as controls. Forty-four of 59 patients had been examined by spectral-domain optical coherence tomography (SD-OCT). Eyes with SSOH were classified into a definite and a suspect type determined by standard automated perimetry. The definite type had inferior visual field (VF) defects, while the suspect type did not have inferior VF defects. The findings of the SD-OCT images of 35 eyes with SSOH were compared to those of the 35 normal eyes.


Of the 106 eyes with SSOH, 56 (52.8%) were classified as the definite type and 50 (47.2%) as the suspect type. OCT showed that the average of the total RNFL thickness was significantly thinner in the SSOH group than in the normal group (P < 0.001; Mann-Whitney U test). Sectorial analysis demonstrated that the RNFL was thinner than controls in all quadrants (all P < 0.001; Mann-Whitney U test). The comparison of the hourly sectors showed that the RNFL was thinner at 10, 11, 12, 1, 2, 3, 5, and 6 o’clock sectors in the SSOH group than controls.


Approximately one-half of eyes with SSOH had a detectable VF defect. OCT showed that eyes with SSOH have a thinner RNFL than controls except in 4 o`clock and from 7 o’clock to 9 o’clock.


Superior segmental optic hypoplasia Optical coherence tomography Retinal nerve fiber layer Visual field defect 


Conflicts of interest

A. Yagasaki, None; A. Sawada, None; Y. Manabe, None; T. Yamamoto, None. The authors have no proprietary or financial interest in any products used in this study.


  1. 1.
    Kim RY, Hoyt WF, Lessell S, Narahara MH. Superior segmental optic hypoplasia. A sign of maternal diabetes mellitus. Am J Ophthalmol. 1989;107:1312–5.Google Scholar
  2. 2.
    Landau K, Djahanshahi-Bajka J, Kirchschläger BM. Topless optic disks in children of mothers with type I diabetes mellitus. Am J Ophthalmol. 1998;125:605–11.CrossRefGoogle Scholar
  3. 3.
    Petersen RA, Walton DS. Optic nerve hypoplasia with good visual acuity and visual field defects. Arch Ophthalmol. 1997;95:254–8.CrossRefGoogle Scholar
  4. 4.
    Bjork A, Laurell CG, Laurell U. Bilateral optic nerve hypoplasia with good visual acuity. Am J Ophthalmol. 1978;86:524–9.CrossRefGoogle Scholar
  5. 5.
    Nelson M, Lessell S, Sadun AA. Optic nerve hypoplasia and maternal diabetes mellitus. Arch Neurol. 1986;43:20–5.CrossRefGoogle Scholar
  6. 6.
    Yamamoto T, Sato M, Iwase A. Superior segmental optic hypoplasia found in Tajimi Eye Health Care Project participants. Jpn J Ophthalmol. 2004;48:578–83.CrossRefGoogle Scholar
  7. 7.
    Han SB, Park KH, Kim DM, Kim TW. Prevalence of superior segmental optic nerve hypoplasia in Korea. Jpn J Ophthalmol. 2009;53:225–8.CrossRefGoogle Scholar
  8. 8.
    Seo S, Lee CE, Kim DW, Kim YK, Jeoung JW, Kim CY, et al. Prevalence and risk factors of superior segmental optic hypoplasia in a Korean population: the Korea National Health and Nutrition Examination Survey. BMC Ophthalmol. 2014;14:157.CrossRefGoogle Scholar
  9. 9.
    Iwase A, Suzuki Y, Araie M, Yamamoto T, Abe H, Shirato S, et al. The prevalence of primary open-angle glaucoma in Japanese. The Tajimi study. Ophthalmology. 2004;111:1641–8.Google Scholar
  10. 10.
    Shiose Y, Kitazawa Y, Tsukahara S, Akamatsu T, Mizokami K, Katsushima H, et al. Epidemiology of glaucoma in Japan. A nationwide glaucoma survey. Jpn J Ophthalmol. 1991;35:133–55.Google Scholar
  11. 11.
    Unoki K, Ohba N, Hoyt WF. Optical coherence tomography of superior segmental optic hypoplasia. Br J Ophthalmol. 2002;86:910–4.CrossRefGoogle Scholar
  12. 12.
    Lee HJ, Kee C. Optical coherence tomography and Heidelberg retina tomography for superior segmental optic hypoplasia. Br J Ophthalmol. 2009;93:1468–73.CrossRefGoogle Scholar
  13. 13.
    Fuse N, Aizawa N, Yokoyama Y, Nakamura M, Omodaka K, Sado K, et al. Analysis of retinal fiber layer thickness in superior segmental optic hypoplasia (SSOH). Nippon Ganka Gakkai Zasshi. 2012;116:575–80 (in Japanese).Google Scholar
  14. 14.
    Yamada M, Ohkubo S, Higashide T, Nitta K, Takeda H, Sugiyama K. Differentiation by imaging superior segmental optic hypoplasia and normal-tension glaucoma with inferior visual field defects only. Jpn J Ophthalmol. 2013;57:25–33.CrossRefGoogle Scholar
  15. 15.
    Wojtkowski M, Bajraszewski T, Targowski P, Kowalczyk A. Real-time in vivo imaging by high-speed spectral optical coherence tomography. Opt Lett. 2003;28:1745–7.CrossRefGoogle Scholar
  16. 16.
    Nassif N, Cense B, Park BH, Yun SH, Chen TC, Bouma BE, et al. In vivo human retinal imaging by ultrahigh-speed spectral domain optical coherence tomography. Opt Lett. 2004;29:480–2.CrossRefGoogle Scholar
  17. 17.
    Hayashi K, Tomidokoro A, Konno S, Mayama C, Aihara M, Araie M. Evaluation of optic nerve head configurations of super segmental optic hypoplasia by spectral-domain optical coherence tomography. Br J Ophthalmol. 2010;94:768–72.CrossRefGoogle Scholar
  18. 18.
    Han JC, Choi DY, Kee C. The different characteristics of cirrus optical coherence tomography between superior segmental optic hypoplasia and normal tension glaucoma with superior retinal nerve fiber defect. J Ophthalmol. 2015;2015:641204.CrossRefGoogle Scholar
  19. 19.
    Frisen L, Holmegaard L. Spectrum of optic nerve hypoplasia. Br J Ophthalmol. 1987;62:7–15.CrossRefGoogle Scholar
  20. 20.
    Lee HJ, Ozaki M, Okano M, Kee C. Coexistence and development of an open-angle glaucoma in eyes with superior segmental optic hypoplasia. J Glaucoma. 2015;24:207–13.CrossRefGoogle Scholar
  21. 21.
    Hayashi K, Tomidokoro A, Aihara M, Tsuji H, Shirato S, Araie M. Long-term follow-up of superior segmental optic hypoplasia. Jpn J Ophthalmol. 2008;52:412–4.CrossRefGoogle Scholar
  22. 22.
    Takagi M, Abe H, Hatase T, Yaoeda K, Miki A, Shirakashi M. Superior segmental optic nerve hypoplasia in youth. Jpn J Ophthalmol. 2008;52:468–74.CrossRefGoogle Scholar
  23. 23.
    Fujimoto N. Differentiation and combination of optic nerve hypoplasia and glaucoma. Neuroophthalmol Jpn. 2007;24:426–32.Google Scholar

Copyright information

© Japanese Ophthalmological Society 2018

Authors and Affiliations

  • Ayaka Yagasaki
    • 1
    Email author
  • Akira Sawada
    • 1
  • Yusuke Manabe
    • 1
  • Tetsuya Yamamoto
    • 1
  1. 1.Department of OphthalmologyGifu University Graduate School of MedicineGifu-shiJapan

Personalised recommendations