Aim: The aim of this study is to evaluate retinal vascular changes in patients with sickle cell disease (SCD) and beta-thalassemia with optical coherence tomography angiography (OCT-A).

Methods: For this purpose, 98 patients with SCD, 75 patients with beta-thalassemia, and 100 healthy controls in Mersin University Hospital between January 1, 2020, and November 1, 2021, were included in this study. OCT-A imaging was performed with ZEISS AngioPlex OCT angiography (Carl Zeiss Meditec, Dublin, CA, USA).

Results: All OCT-A parameters (FAZ area, perimeter, circularity, vessel, and perfusion density) were found to be statistically significantly different in both patients with thalassemia and patients with sickle cell disease when compared to the controls.

Conclusions: In conclusion, retinopathy related to both hemoglobinopathy subgroups can be diagnosed and followed up with OCT-A. It was also found that OCT-A parameters are affected before the development of clinically detectable retinopathy.

Keywords: opptical coherence tomography angiography, retinopathy, sickle cell disease, thalassemia

Copyright and license

How to cite

Özer Ö, Güçlü E. Optical coherence tomography angiography changes in patients with hemoglobinopathy. Northwestern Med J. 2024;4(2):81-6. https://doi.org/10.54307/2024.NWMJ.110


  1. Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008; 86(6): 480-7. https://doi.org/10.2471/blt.06.036673
  2. Hassan T, Badr M, Hanna D, et al. Retinopathy in Egyptian patients with sickle cell disease: A cross-sectional study. Medicine (Baltimore). 2021; 100(51): e28355. https://doi.org/10.1097/MD.0000000000028355
  3. Moriarty BJ, Acheson RW, Condon PI, Serjeant GR. Patterns of visual loss in untreated sickle cell retinopathy. Eye (Lond). 1988; 2(Pt 3): 330-5. https://doi.org/10.1038/eye.1988.62
  4. Goldberg MF. Classification and pathogenesis of proliferative sickle retinopathy. Am J Ophthalmol. 1971; 71(3): 649-65. https://doi.org/10.1016/0002-9394(71)90429-6
  5. Abi Saad M, Haddad AG, Alam ES, et al. Preventing thalassemia in Lebanon: successes and challenges in a developing country. Hemoglobin. 2014; 38(5): 308-11. https://doi.org/10.3109/03630269.2014.939279
  6. Feroze KB, Azevedo AM. Retinopathy Hemoglobinopathies. In: StatPearls. Treasure Island (FL): StatPearls Publishing; July 17, 2023.
  7. Liaska A, Petrou P, Georgakopoulos CD, et al. β-Thalassemia and ocular implications: a systematic review. BMC Ophthalmol. 2016; 16: 102. https://doi.org/10.1186/s12886-016-0285-2
  8. Bhoiwala DL, Dunaief JL. Retinal abnormalities in β-thalassemia major. Surv Ophthalmol. 2016; 61(1): 33-50. https://doi.org/10.1016/j.survophthal.2015.08.005
  9. Lynch G, Scott AW, Linz MO, et al. Foveal avascular zone morphology and parafoveal capillary perfusion in sickle cell retinopathy. Br J Ophthalmol. 2020; 104(4): 473-9. https://doi.org/10.1136/bjophthalmol-2019-314567
  10. Zhou DB, Scott AW, Linz MO, et al. Interocular asymmetry of foveal avascular zone morphology and parafoveal capillary density in sickle cell retinopathy. PLoS One. 2020; 15(6): e0234151. https://doi.org/10.1371/journal.pone.0234151
  11. Han IC, Tadarati M, Pacheco KD, Scott AW. Evaluation of Macular Vascular Abnormalities Identified by Optical Coherence Tomography Angiography in Sickle Cell Disease. Am J Ophthalmol. 2017; 177: 90-9. https://doi.org/10.1016/j.ajo.2017.02.007
  12. Cennamo G, Montorio D, Mazzella G, et al. Retinal and Choriocapillaris Vascular Changes in Patients Affected by Different Clinical Phenotypes of β-Thalassemia: An Optical Coherence Tomography Angiography Study. Biology (Basel). 2021; 10(4): 276. https://doi.org/10.3390/biology10040276
  13. Mokrane A, Gazeau G, Lévy V, Fajnkuchen F, Giocanti-Aurégan A. Analysis of the foveal microvasculature in sickle cell disease using swept-source optical coherence tomography angiography. Sci Rep. 2020; 10(1): 11795. https://doi.org/10.1038/s41598-020-68625-8