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Unmeasurable small size superficial and deep foveal avascular zone in nanophthalmos: the Collaborative Nanophthalmos OCTA Study
  1. Ahmad M Mansour1,2,
  2. Michael W Stewart3,
  3. Salma W Yassine1,
  4. Carl-Joe Z Mehanna1,
  5. Antonio Marcelo B Casella4,
  6. Rola N Hamam1,
  7. Jay Chhablani5,
  8. Luiz H Lima6,
  9. Ari Shinojima7,
  10. Hiroyuki Kaneko7,
  11. Muhammad H Younis1,
  12. Hasan K Shahin1,
  13. Subhadra Jalali5,
  14. Cem Kucukerdonmez8,
  15. Ali O Saatci9,
  16. Renuka Chakurkar5,
  17. Carol L Shields10
  1. 1 Department of Ophthalmology, American University of Beirut, Beirut, Lebanon
  2. 2 Department of Ophthalmology, Rafic Hariri University Hospital, Beirut, Lebanon
  3. 3 Department of Ophthalmology, Mayo Clinic, Jacksonville, Florida, USA
  4. 4 Department of Ophthalmology, Universidade Estadual de Londrina, Londrina, Brazil
  5. 5 Vitreoretinal Service, LV Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India
  6. 6 Department of Ophthalmology and Visual Sciences, Federal University of Sao Paulo, Sao Paulo, Brazil
  7. 7 Division of Ophthalmology, Department of Visual Science, Nihon University School of Medicine, Tokyo, Japan
  8. 8 Department of Ophthalmology, Ekol Eye Hospital, Izmir, Turkey
  9. 9 Department of Ophthalmology, Dokuz Eylul University, School of Medicine, Izmir, Turkey
  10. 10 Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Ahmad M Mansour, American University of Beirut, Beirut 1136044, Lebanon; ammansourmd{at}


Aim To study the macular structure and vasculature in consecutive nanophthalmic eyes using optical coherence tomography angiography.

Methods This is a prospective, multicentre, cross-sectional study of patients with nanophthalmos (one or both eyes). The superficial and deep foveal avascular zones (FAZ) were measured both manually and with the machine’s built-in automated measurement tool. Correlations between best corrected visual acuity (BCVA), central macular thickness (CMT) and subfoveolar choroidal thickness (SFCT) were calculated.

Results Sixty-five eyes of 35 subjects (16 men and 19 women) with a mean age of 37.4 years were analysed. The mean±SD of refractive error was 14.3±3.2 dioptres, axial length was 16.4±1.6 mm, CMT was 410.2±128.3 µm and SFCT was 450.1±108.3 µm. FAZ was unmeasurable small size in both the superficial and deep capillary plexus in all eyes, along with tortuosity of the superficial foveal capillaries and large vessels. Foveal folds were present in 29 eyes. Disc drusen was detected in 27 eyes and was absent in 31 eyes, while fundus autofluorescence was positive in 17 and negative in 24 eyes. BCVA varied from 20/20 to 20/800, with a mean of 20/76. Using Spearman’s correlation, logarithm of the minimum angle of resolution BCVA correlated negatively with axial length (r=−0.30; p=0.015).

Conclusions FAZ attenuation, capillary tortuosity, foveal folds and thickened subfoveal choroid characterise the nanophthalmic macula. These findings may result from a redundant retina and the absence of apoptotic foveolar retraction because of developmental arrest of the optic vesicle after closure of the embryonic fissure.

  • angiogenesis
  • embryology and development
  • imaging
  • macula
  • sclera and episclera

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  • Contributors Design: AMM, MWS, C-JZM, AMBC, RNH, JC, LHL, AS, HK, MHY, HKS, SJ, CK, AOS, RC. Conduct of the study: AMM. Collection: AMM. Management: AMM, MWS, C-JZM, AMBC, RNH, JC, LHL, AS, HK, MHY, HKS, SJ, CK, AOS, RC. Analysis: AMM, C-JZM. Interpretation of data: AMM, MWS, CLS. Preparation: AMM, MWS, JC, CLS. Review: AMM, MWS, JC, CLS. Approval of the manuscript: AMM, MWS, C-JZM, AMBC, RNH, JC, LHL, AS, HK, MHY, HKS, SJ, CK, AOS, RC, CLS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval The study protocol was approved by the Institutional Review Board of Rafic Hariri University Hospital.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement AMM can provide all the data by email:

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