Does thalassemia truly cause microvascular changes without us noticing? An optical coherence tomography angiography study of the children with beta-thalassemia.

Purpose: It was aimed to evaluate the retinochoroidal microvascular alterations of pediatric beta-thalassemia patients and investigate the effect of blood transfusion on perfusion among transfusion-dependent thalassemia (TDT), by means of optical coherence tomography angiography (OCTA).

Methods: In this multicentered, prospective, cross-sectional study, 56 TDT, 14 non-TDT (NTDT), and 63 healthy children were evaluated. The vessel density (VD) in superficial capillary plexus (SCP), deep capillary plexus, radial peripapillary capillary network, choriocapillaris, and the foveal avascular zone area (FAZ) were evaluated by OCTA. Before and after transfusion values ​​of the TDT group were compared, and correlations were made with blood values ​​and iron accumulation.

Results: Foveal and parafoveal zones were significantly thinner among TDT patients, with larger FAZ area. Macula VD of SCP and ppVD was lowest in NTDT group. In the TDT group, a decrease in retinal nerve fiber thickness and ppVD values was detected ​​after transfusion. A negative significant relationship was found between both hemoglobin (Hb), hematocrit (Htc), and ppVD.

Conclusions: OCTA provides a better insight into retinal and choriocapillaris vascular impairment influenced by tissue hypoxia and oxidative stress in different clinical phenotypes of beta-thalassemia.